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Giant cell arteritis (Clinical)

Giant cell arteritis Giant Cell Arteritis Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis ( GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis), also known as temporal arteritis Temporal arteritis Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis, is a type of large-vessel vasculitis Vasculitis Inflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body. Systemic Lupus Erythematosus that predominantly affects the aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis Giant Cell Arteritis Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis is defined by inflammatory leukocytes Leukocytes White blood cells. These include granular leukocytes (basophils; eosinophils; and neutrophils) as well as non-granular leukocytes (lymphocytes and monocytes). White Myeloid Cells: Histology in the vessel walls leading to reactive damage, ischemia Ischemia A hypoperfusion of the blood through an organ or tissue caused by a pathologic constriction or obstruction of its blood vessels, or an absence of blood circulation. Ischemic Cell Damage, and necrosis Necrosis The death of cells in an organ or tissue due to disease, injury or failure of the blood supply. Ischemic Cell Damage. Giant cell arteritis Giant Cell Arteritis Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis causes headaches, scalp tenderness, jaw Jaw The jaw is made up of the mandible, which comprises the lower jaw, and the maxilla, which comprises the upper jaw. The mandible articulates with the temporal bone via the temporomandibular joint (TMJ). The 4 muscles of mastication produce the movements of the TMJ to ensure the efficient chewing of food. Jaw and Temporomandibular Joint: Anatomy pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways, vision Vision Ophthalmic Exam problems, and potentially blindness Blindness The inability to see or the loss or absence of perception of visual stimuli. This condition may be the result of eye diseases; optic nerve diseases; optic chiasm diseases; or brain diseases affecting the visual pathways or occipital lobe. Retinopathy of Prematurity. The diagnosis is made with temporal artery biopsy Temporal Artery Biopsy Giant Cell Arteritis. Prompt treatment with glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids can relieve symptoms and prevent vision Vision Ophthalmic Exam loss.

Last updated: Apr 21, 2023

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Overview

Epidemiology[1,6,7]

  • Most common idiopathic Idiopathic Dermatomyositis systemic vasculitis Vasculitis Inflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body. Systemic Lupus Erythematosus
  • Age is the greatest risk factor:
    • Generally affects individuals older than 50 years
    • Increased incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency in the 8th and 9th decades of life
  • 3 times more common in women
  • Northern European and Scandinavian ancestry
  • Lower body mass index Body mass index An indicator of body density as determined by the relationship of body weight to body height. Bmi=weight (kg)/height squared (m2). Bmi correlates with body fat (adipose tissue). Their relationship varies with age and gender. For adults, bmi falls into these categories: below 18. 5 (underweight); 18. 5-24. 9 (normal); 25. 0-29. 9 (overweight); 30. 0 and above (obese). Obesity ( BMI BMI An indicator of body density as determined by the relationship of body weight to body height. Bmi=weight (kg)/height squared (m2). Bmi correlates with body fat (adipose tissue). Their relationship varies with age and gender. For adults, bmi falls into these categories: below 18. 5 (underweight); 18. 5-24. 9 (normal); 25. 0-29. 9 (overweight); 30. 0 and above (obese). Obesity) is associated with a higher risk.

Etiology[1,6]

  • Exact cause is unknown.
  • Genetic factors may increase susceptibility:
    • Genetic variants in the major histocompatibility complex Major histocompatibility complex The genetic region which contains the loci of genes which determine the structure of the serologically defined (sd) and lymphocyte-defined (ld) transplantation antigens, genes which control the structure of the immune response-associated antigens, human; the immune response genes which control the ability of an animal to respond immunologically to antigenic stimuli, and genes which determine the structure and/or level of the first four components of complement. Innate Immunity: Phagocytes and Antigen Presentation (MHC)
    • Human leukocyte antigen Antigen Substances that are recognized by the immune system and induce an immune reaction. Vaccination (HLA) subtypes
    • Genes Genes A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms. DNA Types and Structure involved in T cells T cells Lymphocytes responsible for cell-mediated immunity. Two types have been identified – cytotoxic (t-lymphocytes, cytotoxic) and helper T-lymphocytes (t-lymphocytes, helper-inducer). They are formed when lymphocytes circulate through the thymus gland and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen. T cells: Types and Functions
  • 45%–50% have concurrent polymyalgia rheumatica Polymyalgia rheumatica A syndrome in the elderly characterized by proximal joint and muscle pain, high erythrocyte sedimentation rate, and a self-limiting course. Pain is usually accompanied by evidence of an inflammatory reaction. Women are affected twice as commonly as men and caucasians more frequently than other groups. The condition is frequently associated with giant cell arteritis and some theories pose the possibility that the two diseases arise from a single etiology or even that they are the same entity. Giant Cell Arteritis.

Pathophysiology[1,6,7]

Giant cell arteritis Giant Cell Arteritis Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis ( GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis) is caused by a complicated cascade of vascular inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation, damage, and dysfunctional repair:

  • Triggering event has not been identified.
  • Involves large- and medium-sized arteries Medium-Sized Arteries Kawasaki Disease:
    • Usually affecting the aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy and its major branches
    • Common involvement of branches off the carotid and vertebral arteries Arteries Arteries are tubular collections of cells that transport oxygenated blood and nutrients from the heart to the tissues of the body. The blood passes through the arteries in order of decreasing luminal diameter, starting in the largest artery (the aorta) and ending in the small arterioles. Arteries are classified into 3 types: large elastic arteries, medium muscular arteries, and small arteries and arterioles. Arteries: Histology
  • Most notably involves the superficial temporal artery Superficial Temporal Artery Giant Cell Arteritis ( STA STA Giant Cell Arteritis; branch off the external carotid artery External carotid artery Branch of the common carotid artery which supplies the exterior of the head, the face, and the greater part of the neck. Carotid Arterial System: Anatomy)
  • Mechanism:
    1. An antigen Antigen Substances that are recognized by the immune system and induce an immune reaction. Vaccination is sensed by dendritic cells Dendritic cells Specialized cells of the hematopoietic system that have branch-like extensions. They are found throughout the lymphatic system, and in non-lymphoid tissues such as skin and the epithelia of the intestinal, respiratory, and reproductive tracts. They trap and process antigens, and present them to T-cells, thereby stimulating cell-mediated immunity. They are different from the non-hematopoietic follicular dendritic cells, which have a similar morphology and immune system function, but with respect to humoral immunity (antibody production). Skin: Structure and Functions in the adventitia → recruits more dendritic cells Dendritic cells Specialized cells of the hematopoietic system that have branch-like extensions. They are found throughout the lymphatic system, and in non-lymphoid tissues such as skin and the epithelia of the intestinal, respiratory, and reproductive tracts. They trap and process antigens, and present them to T-cells, thereby stimulating cell-mediated immunity. They are different from the non-hematopoietic follicular dendritic cells, which have a similar morphology and immune system function, but with respect to humoral immunity (antibody production). Skin: Structure and Functions, lymphocytes Lymphocytes Lymphocytes are heterogeneous WBCs involved in immune response. Lymphocytes develop from the bone marrow, starting from hematopoietic stem cells (HSCs) and progressing to common lymphoid progenitors (CLPs). B and T lymphocytes and natural killer (NK) cells arise from the lineage. Lymphocytes: Histology, and macrophages Macrophages The relatively long-lived phagocytic cell of mammalian tissues that are derived from blood monocytes. Main types are peritoneal macrophages; alveolar macrophages; histiocytes; kupffer cells of the liver; and osteoclasts. They may further differentiate within chronic inflammatory lesions to epithelioid cells or may fuse to form foreign body giant cells or langhans giant cells. Innate Immunity: Phagocytes and Antigen Presentation.
    2. Dendritic cells Dendritic cells Specialized cells of the hematopoietic system that have branch-like extensions. They are found throughout the lymphatic system, and in non-lymphoid tissues such as skin and the epithelia of the intestinal, respiratory, and reproductive tracts. They trap and process antigens, and present them to T-cells, thereby stimulating cell-mediated immunity. They are different from the non-hematopoietic follicular dendritic cells, which have a similar morphology and immune system function, but with respect to humoral immunity (antibody production). Skin: Structure and Functions process and present the antigens → cluster of differentiation 4 (CD4+) T cell activation T cell activation Adaptive Cell-mediated Immunity.
    3. CD4+ T cells T cells Lymphocytes responsible for cell-mediated immunity. Two types have been identified – cytotoxic (t-lymphocytes, cytotoxic) and helper T-lymphocytes (t-lymphocytes, helper-inducer). They are formed when lymphocytes circulate through the thymus gland and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen. T cells: Types and Functions proliferate → vascular inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation via activation of macrophages Macrophages The relatively long-lived phagocytic cell of mammalian tissues that are derived from blood monocytes. Main types are peritoneal macrophages; alveolar macrophages; histiocytes; kupffer cells of the liver; and osteoclasts. They may further differentiate within chronic inflammatory lesions to epithelioid cells or may fuse to form foreign body giant cells or langhans giant cells. Innate Immunity: Phagocytes and Antigen Presentation.
    4. Medial layer is invaded by inflammatory cells → macrophages Macrophages The relatively long-lived phagocytic cell of mammalian tissues that are derived from blood monocytes. Main types are peritoneal macrophages; alveolar macrophages; histiocytes; kupffer cells of the liver; and osteoclasts. They may further differentiate within chronic inflammatory lesions to epithelioid cells or may fuse to form foreign body giant cells or langhans giant cells. Innate Immunity: Phagocytes and Antigen Presentation undergo granulomatous organization ( giant cells Giant cells Multinucleated masses produced by the fusion of many cells; often associated with viral infections. In aids, they are induced when the envelope glycoprotein of the HIV virus binds to the CD4 antigen of uninfected neighboring T4 cells. The resulting syncytium leads to cell death and thus may account for the cytopathic effect of the virus. Giant Cell Arteritis) → growth factors and metalloproteinases produced → loss of vascular smooth muscle cells and destruction of the internal elastic Elastic Connective Tissue: Histology lamina.
    5. Injured smooth muscle cells respond to this damage → myofibroblasts Myofibroblasts Spindle-shaped cells with characteristic contractile proteins and structures that contribute to the wound healing process. They occur in granulation tissue and also in pathological processes such as fibrosis. Hypertrophic and Keloid Scars differentiate, migrate toward the intimal layer, and produce extracellular matrix Extracellular matrix A meshwork-like substance found within the extracellular space and in association with the basement membrane of the cell surface. It promotes cellular proliferation and provides a supporting structure to which cells or cell lysates in culture dishes adhere. Hypertrophic and Keloid Scars proteins Proteins Linear polypeptides that are synthesized on ribosomes and may be further modified, crosslinked, cleaved, or assembled into complex proteins with several subunits. The specific sequence of amino acids determines the shape the polypeptide will take, during protein folding, and the function of the protein. Energy Homeostasis → intimal hyperplasia Hyperplasia An increase in the number of cells in a tissue or organ without tumor formation. It differs from hypertrophy, which is an increase in bulk without an increase in the number of cells. Cellular Adaptation and arterial occlusion → ischemic complications of the disease.
Superficial temporal artery

Branches of the STA, which may be involved in GCA

Image by BioDigital, edited by Lecturio.

Clinical Presentation

Clinical manifestations[6,8,10,13]

Symptom onset is typically subacute, developing over weeks to months. More abrupt presentations, with symptoms arising over a few days; occurs in a minority of cases.

  • Constitutional:
    • Fever Fever Fever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin. Fever (typically low-grade)
    • Fatigue Fatigue The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli. Fibromyalgia 
    • Malaise Malaise Tick-borne Encephalitis Virus 
    • Weight loss Weight loss Decrease in existing body weight. Bariatric Surgery 
    • Anorexia Anorexia The lack or loss of appetite accompanied by an aversion to food and the inability to eat. It is the defining characteristic of the disorder anorexia nervosa. Anorexia Nervosa
  • Head and neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess:
    • Headache Headache The symptom of pain in the cranial region. It may be an isolated benign occurrence or manifestation of a wide variety of headache disorders. Brain Abscess:
      • Occurs in ⅔ of patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship
      • Varying severity and quality Quality Activities and programs intended to assure or improve the quality of care in either a defined medical setting or a program. The concept includes the assessment or evaluation of the quality of care; identification of problems or shortcomings in the delivery of care; designing activities to overcome these deficiencies; and follow-up monitoring to ensure effectiveness of corrective steps. Quality Measurement and Improvement
      • Localized to the temples and can be tender to the touch
      • Associated scalp tenderness
    • Jaw claudication Jaw claudication Mandibular pain or fatigue during mastication that is relieved with rest Giant Cell Arteritis:
      • Occurs in about ½ of patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship
      • Mandibular pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways or fatigue Fatigue The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli. Fibromyalgia during mastication Mastication The act and process of chewing and grinding food in the mouth. Jaw and Temporomandibular Joint: Anatomy that is relieved with rest
  • Ophthalmic:
    • Transient vision Vision Ophthalmic Exam loss ( amaurosis fugax Amaurosis fugax Transient complete or partial monocular blindness due to retinal ischemia. This may be caused by emboli from the carotid artery (usually in association with carotid stenosis) and other locations that enter the central retinal artery. Carotid Artery Stenosis):
      • Results from loss of blood flow Blood flow Blood flow refers to the movement of a certain volume of blood through the vasculature over a given unit of time (e.g., mL per minute). Vascular Resistance, Flow, and Mean Arterial Pressure to the eye
      • Typically monocular  
      • Painless, transient, and intermittent
      • Blurring, diplopia Diplopia A visual symptom in which a single object is perceived by the visual cortex as two objects rather than one. Disorders associated with this condition include refractive errors; strabismus; oculomotor nerve diseases; trochlear nerve diseases; abducens nerve diseases; and diseases of the brain stem and occipital lobe. Myasthenia Gravis, or vision Vision Ophthalmic Exam loss
    • Permanent vision Vision Ophthalmic Exam loss:
  • Musculoskeletal:
    • Polymyalgia rheumatica Polymyalgia rheumatica A syndrome in the elderly characterized by proximal joint and muscle pain, high erythrocyte sedimentation rate, and a self-limiting course. Pain is usually accompanied by evidence of an inflammatory reaction. Women are affected twice as commonly as men and caucasians more frequently than other groups. The condition is frequently associated with giant cell arteritis and some theories pose the possibility that the two diseases arise from a single etiology or even that they are the same entity. Giant Cell Arteritis:
      • Aching and morning stiffness in the proximal muscles and joints
      • Shoulders, hip girdles, neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess, and torso affected

Physical exam[6,8,10,13]

  • Cardiovascular:
  • Fundoscopy Fundoscopy Cranial Nerve Palsies:
    • Can be entirely normal with transient symptoms
    • Retinal ischemia Ischemia A hypoperfusion of the blood through an organ or tissue caused by a pathologic constriction or obstruction of its blood vessels, or an absence of blood circulation. Ischemic Cell Damage → cotton-wool spots in the retina Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outermost layer is pigmented, whereas the inner nine layers are transparent. Eye: Anatomy
    • Anterior ischemic optic neuropathy Neuropathy Leprosy (loss of blood flow Blood flow Blood flow refers to the movement of a certain volume of blood through the vasculature over a given unit of time (e.g., mL per minute). Vascular Resistance, Flow, and Mean Arterial Pressure to the anterior portion of the optic nerve Optic nerve The 2nd cranial nerve which conveys visual information from the retina to the brain. The nerve carries the axons of the retinal ganglion cells which sort at the optic chiasm and continue via the optic tracts to the brain. The largest projection is to the lateral geniculate nuclei; other targets include the superior colliculi and the suprachiasmatic nuclei. Though known as the second cranial nerve, it is considered part of the central nervous system. The 12 Cranial Nerves: Overview and Functions) → swollen, pale disc and blurred margins of the retina Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outermost layer is pigmented, whereas the inner nine layers are transparent. Eye: Anatomy
  • Musculoskeletal:
    • Limited shoulder, neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess, and hip range of motion Range of motion The distance and direction to which a bone joint can be extended. Range of motion is a function of the condition of the joints, muscles, and connective tissues involved. Joint flexibility can be improved through appropriate muscle strength exercises. Examination of the Upper Limbs
    • Wrist and metacarpophalangeal synovitis Synovitis Inflammation of the synovial membrane. Rheumatoid Arthritis
Acute central retinal artery occlusion

Fundoscopy of the right eye, showing cotton-wool spots along the superotemporal and inferotemporal arterioles (arrows). The cotton-wool spots denote obstruction of the retinal arterioles, leading to retinal ischemia.

Image: “Cotton-wool spots” by James Paget University Hospital NHS Foundation Trust, Lowestoft Road, Gorleston, Great Yarmouth NR31 6LA, Norfolk, UK. License: CC BY 2.0.

Diagnosis

Diagnostic algorithm

Temporal arteritis diagnostic algorithm

The above is a simple algorithm to help aid in the diagnosis of GCA. This outlines when this disease should be considered, and the basic steps in diagnosis.

Image by Lecturio.

Diagnostic modalities

1st, check laboratory studies:[6,8]

  • Erythrocyte sedimentation rate Erythrocyte Sedimentation Rate Soft Tissue Abscess ( ESR ESR Soft Tissue Abscess) and C-reactive protein (CRP):
    • Almost always elevated
    • ESR ESR Soft Tissue Abscess ≥ 50 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma/hr owing to rouleau formation of RBCs RBCs Erythrocytes, or red blood cells (RBCs), are the most abundant cells in the blood. While erythrocytes in the fetus are initially produced in the yolk sac then the liver, the bone marrow eventually becomes the main site of production. Erythrocytes: Histology
    • Non-specific
  • Complete blood count:
    • Normochromic anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types
    • Reactive thrombocytosis
    • Normal or minimally elevated leukocytes Leukocytes White blood cells. These include granular leukocytes (basophils; eosinophils; and neutrophils) as well as non-granular leukocytes (lymphocytes and monocytes). White Myeloid Cells: Histology
  • Liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver: Anatomy function panel:
    • Elevated alkaline phosphatase Alkaline Phosphatase An enzyme that catalyzes the conversion of an orthophosphoric monoester and water to an alcohol and orthophosphate. Osteosarcoma
    • Decreased albumin Albumin Serum albumin from humans. It is an essential carrier of both endogenous substances, such as fatty acids and bilirubin, and of xenobiotics in the blood. Liver Function Tests

Next steps:[6,8,10,13]

  • Temporal artery biopsy Temporal Artery Biopsy Giant Cell Arteritis (gold standard): 
    • Should be performed in all patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship with suspected GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis
    • Lesions are segmental, so diagnosis requires a biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma of a long vessel segment (1–2 cm in vivo length).
    • Histopathology findings:
      • Panarteritis Panarteritis Giant Cell Arteritis
      • Giant cells Giant cells Multinucleated masses produced by the fusion of many cells; often associated with viral infections. In aids, they are induced when the envelope glycoprotein of the HIV virus binds to the CD4 antigen of uninfected neighboring T4 cells. The resulting syncytium leads to cell death and thus may account for the cytopathic effect of the virus. Giant Cell Arteritis
      • Fragmented internal elastic Elastic Connective Tissue: Histology lamina
    • If negative, a contralateral temporal artery biopsy Temporal Artery Biopsy Giant Cell Arteritis should be considered.
    • False negatives may occur owing to:
    • Ordering:
      • Schedule temporal artery biopsy Temporal Artery Biopsy Giant Cell Arteritis within 2–4 weeks after starting steroids Steroids A group of polycyclic compounds closely related biochemically to terpenes. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (sterols), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus. Benign Liver Tumors if treatment is initiated prior to histopathologic diagnosis.
      • Biopsies can be obtained on an outpatient basis under local anesthesia Anesthesia A state characterized by loss of feeling or sensation. This depression of nerve function is usually the result of pharmacologic action and is induced to allow performance of surgery or other painful procedures. Anesthesiology: History and Basic Concepts.
      • Can be performed by an ophthalmologist, neurosurgeon, vascular surgeon, or other surgical specialist
  • Color Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) ultrasound of temporal arteries Temporal Arteries Arteries arising from the external carotid or the maxillary artery and distributing to the temporal region. Jaw and Temporomandibular Joint: Anatomy:
    • Possible non-invasive alternative for temporal artery biopsy Temporal Artery Biopsy Giant Cell Arteritis, but is operator dependent
    • Should evaluate the vessels of the head, neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess, and upper extremities
    • Halo sign Halo sign Aspergillus/Aspergillosis” (darkened area surrounding the vascular lumen from mural edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema) and a non-compressible artery are highly specific.
    • Ideally performed before steroid therapy is commenced, or as soon as possible afterward, to improve diagnostic sensitivity.
    • If ultrasound results are equivocal or negative and suspicion remains, proceed with biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma.
Temporal arteritis

Biopsy of temporal artery showing transmural mixed inflammatory cell infiltrates with intimal thickening, fragmentation, and distortion of the internal elastic lamina

Image: “Biopsy of temporal artery” by the Department of Pathology and Laboratory Medicine, University of Florida, College of Medicine, Jacksonville, FL, USA. License: CC BY 2.0.

If the above workup is not diagnostic, consider evaluation for large-vessel involvement:[6,8,10,13]

  • Options:
    • Color Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) ultrasound of axillary and subclavian arteries Arteries Arteries are tubular collections of cells that transport oxygenated blood and nutrients from the heart to the tissues of the body. The blood passes through the arteries in order of decreasing luminal diameter, starting in the largest artery (the aorta) and ending in the small arterioles. Arteries are classified into 3 types: large elastic arteries, medium muscular arteries, and small arteries and arterioles. Arteries: Histology
    • Computed tomography with angiography Angiography Radiography of blood vessels after injection of a contrast medium. Cardiac Surgery ( CTA CTA A non-invasive method that uses a ct scanner for capturing images of blood vessels and tissues. A contrast material is injected, which helps produce detailed images that aid in diagnosing vascular diseases. Pulmonary Function Tests)
    • Magnetic resonance imaging (MRI) or magnetic resonance angiography Angiography Radiography of blood vessels after injection of a contrast medium. Cardiac Surgery ( MRA MRA Imaging of the Heart and Great Vessels)
    • Positron emission tomography ( PET PET An imaging technique that combines a positron-emission tomography (PET) scanner and a ct X ray scanner. This establishes a precise anatomic localization in the same session. Nuclear Imaging)
  • ​​Choice of CTA CTA A non-invasive method that uses a ct scanner for capturing images of blood vessels and tissues. A contrast material is injected, which helps produce detailed images that aid in diagnosing vascular diseases. Pulmonary Function Tests versus MRA MRA Imaging of the Heart and Great Vessels is based on clinician Clinician A physician, nurse practitioner, physician assistant, or another health professional who is directly involved in patient care and has a professional relationship with patients. Clinician–Patient Relationship preference and availability of local resources.
  • MRA MRA Imaging of the Heart and Great Vessels is superior to CTA CTA A non-invasive method that uses a ct scanner for capturing images of blood vessels and tissues. A contrast material is injected, which helps produce detailed images that aid in diagnosing vascular diseases. Pulmonary Function Tests/ PET PET An imaging technique that combines a positron-emission tomography (PET) scanner and a ct X ray scanner. This establishes a precise anatomic localization in the same session. Nuclear Imaging for cranial involvement.

2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria[11]

  • Considerations:
    • Used when there is a diagnosis of medium- or large-vessel vasculitis Vasculitis Inflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body. Systemic Lupus Erythematosus
    • Alternative diagnoses must be excluded.
  • Requirement: age ≥ 50 years at time of diagnosis
  • Scored on clinical and diagnostic findings (10 items)
  • ≥ 6 points needed for GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis classification
Table: ACR/EULAR classification criteria for GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis
Category criteria Points
Clinical Morning stiffness affecting shoulders and/or neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess 2
Sudden vision Vision Ophthalmic Exam loss 3
Claudication of jaw Jaw The jaw is made up of the mandible, which comprises the lower jaw, and the maxilla, which comprises the upper jaw. The mandible articulates with the temporal bone via the temporomandibular joint (TMJ). The 4 muscles of mastication produce the movements of the TMJ to ensure the efficient chewing of food. Jaw and Temporomandibular Joint: Anatomy or tongue Tongue The tongue, on the other hand, is a complex muscular structure that permits tasting and facilitates the process of mastication and communication. The blood supply of the tongue originates from the external carotid artery, and the innervation is through cranial nerves. Lips and Tongue: Anatomy 2
New temporal headache Headache The symptom of pain in the cranial region. It may be an isolated benign occurrence or manifestation of a wide variety of headache disorders. Brain Abscess 2
Scalp tenderness 2
Abnormal findings of the temporal artery, such as:
  • Tenderness
  • Diminished pulses
  • Cord-like appearance
2
Diagnostics ESR ESR Soft Tissue Abscess ≥ 50 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma/hr or CRP ≥ 10 mg/L prior to initiation of treatment 3
Either: 5
Bilateral axillary involvement on imaging 2
FDG-PET activity throughout the aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy 2
ACR: American College of Rheumatology
EULAR: European Alliance of Associations for Rheumatology
GCA: giant cell arteritis
ESR:erythrocyte sedimentation rate
FDG-PET: fluorodeoxyglucose positron emission tomography

Management

It is very important not to delay treatment while awaiting biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma results in order to minimize the risk of vision Vision Ophthalmic Exam loss. The following information is based on US and UK literature and guidelines. Management may vary based on practice location. 

Consultants[8,13]

  • Rheumatology: all suspected/confirmed disease
  • Ophthalmology: presence/risk of visual symptoms
  • Vascular surgery Vascular surgery Vascular surgery is the specialized field of medicine that focuses on the surgical management of the pathologies of the peripheral circulation. The main goal of most vascular procedures is to restore circulatory function to the affected vessels by relieving occlusions or by redirecting blood flow (e.g., bypass). Vascular Surgery: large-vessel involvement

High-dose systemic glucocorticoids Systemic Glucocorticoids Glucocorticoids

Systemic glucocorticoids Systemic Glucocorticoids Glucocorticoids are the mainstay of treatment, and treatment should be initiated promptly if there is a strong suspicion of GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis.

Initial dosing:[8‒10,13]

  • Without vision Vision Ophthalmic Exam loss:
    • Prednisone Prednisone A synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver. Immunosuppressants/ prednisolone Prednisolone A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states. Immunosuppressants
    • Dose:
      • 1 mg/kg daily (typically, 40–60 mg daily)
      • Maximum: 60–80 mg daily
  • With vision Vision Ophthalmic Exam loss:
    • Methylprednisolone Methylprednisolone A prednisolone derivative with similar anti-inflammatory action. Immunosuppressants
    • Dose:
      • 500–1000 mg/day IV for 3 days 
      • Followed by oral prednisone Prednisone A synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver. Immunosuppressants/ prednisolone Prednisolone A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states. Immunosuppressants, as above
  • Continue initial dose until symptoms and inflammatory markers improve.

Tapering:[8,9,13]

  • Should occur slowly over 612 months (total length of treatment is generally ≥ 2 years)
  • Optimal tapering regimen is unknown, but the following is based on consensus guidelines:
    • Consider a reduction of 10 mg every 2 weeks after 2–4 weeks of initial therapy if:
    • Once at 20 mg/day, reduce by 2.5 mg every 2 weeks to a dose of 10 mg/day if there are no flares.
    • Finally, tapering should be slowed (e.g., 1 mg every 1–2 months) to eventually wean off steroids Steroids A group of polycyclic compounds closely related biochemically to terpenes. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (sterols), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus. Benign Liver Tumors within 6–12 months.
  • Note: Disease relapse Relapse Relapsing Fever is more common at doses < 20 mg/day.

Side effects:[8,9,13]

  • The full list of glucocorticoid therapy side effects can be found here.
  • Gastric ulceration Ulceration Corneal Abrasions, Erosion, and Ulcers, osteoporosis Osteoporosis Osteoporosis refers to a decrease in bone mass and density leading to an increased number of fractures. There are 2 forms of osteoporosis: primary, which is commonly postmenopausal or senile; and secondary, which is a manifestation of immobilization, underlying medical disorders, or long-term use of certain medications. Osteoporosis, and immunosuppression can be mitigated by implementing the following at the start of treatment:
    • Screening Screening Preoperative Care tests for tuberculosis Tuberculosis Tuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis complex bacteria. The bacteria usually attack the lungs but can also damage other parts of the body. Approximately 30% of people around the world are infected with this pathogen, with the majority harboring a latent infection. Tuberculosis spreads through the air when a person with active pulmonary infection coughs or sneezes. Tuberculosis
    • Immunization against influenza Influenza Influenza viruses are members of the Orthomyxoviridae family and the causative organisms of influenza, a highly contagious febrile respiratory disease. There are 3 primary influenza viruses (A, B, and C) and various subtypes, which are classified based on their virulent surface antigens, hemagglutinin (HA) and neuraminidase (NA). Influenza typically presents with a fever, myalgia, headache, and symptoms of an upper respiratory infection. Influenza Viruses/Influenza and pneumococcal pneumonia Pneumonia Pneumonia or pulmonary inflammation is an acute or chronic inflammation of lung tissue. Causes include infection with bacteria, viruses, or fungi. In more rare cases, pneumonia can also be caused through toxic triggers through inhalation of toxic substances, immunological processes, or in the course of radiotherapy. Pneumonia
    • Bone Bone Bone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy. Bones: Structure and Types mineral density measurements, adequate dietary calcium Calcium A basic element found in nearly all tissues. It is a member of the alkaline earth family of metals with the atomic symbol ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes. Electrolytes, and vitamin D Vitamin D A vitamin that includes both cholecalciferols and ergocalciferols, which have the common effect of preventing or curing rickets in animals. It can also be viewed as a hormone since it can be formed in skin by action of ultraviolet rays upon the precursors, 7-dehydrocholesterol and ergosterol, and acts on vitamin D receptors to regulate calcium in opposition to parathyroid hormone. Fat-soluble Vitamins and their Deficiencies intake/supplementation
    • Proton pump Pump ACES and RUSH: Resuscitation Ultrasound Protocols inhibitor medication

Monitor with monthly follow-up visits (typically with a rheumatologist):[8,10,13]

  • Review relevant signs and symptoms.
  • Monitor blood pressure in all extremities.
  • Can also monitor ESR ESR Soft Tissue Abscess and CRP trends
  • Other labs to monitor:
    • CBC
    • Electrolytes Electrolytes Electrolytes are mineral salts that dissolve in water and dissociate into charged particles called ions, which can be either be positively (cations) or negatively (anions) charged. Electrolytes are distributed in the extracellular and intracellular compartments in different concentrations. Electrolytes are essential for various basic life-sustaining functions. Electrolytes
    • Renal function
    • Glucose Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Lactose Intolerance

Adjunctive treatment[8–10]

  • May be used when glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids are not tolerated or in individuals at high risk for adverse effects of steroids Steroids A group of polycyclic compounds closely related biochemically to terpenes. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (sterols), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus. Benign Liver Tumors, including those with:
    • Significant premorbid disease (e.g., diabetes Diabetes Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus, osteoporosis Osteoporosis Osteoporosis refers to a decrease in bone mass and density leading to an increased number of fractures. There are 2 forms of osteoporosis: primary, which is commonly postmenopausal or senile; and secondary, which is a manifestation of immobilization, underlying medical disorders, or long-term use of certain medications. Osteoporosis, obesity Obesity Obesity is a condition associated with excess body weight, specifically with the deposition of excessive adipose tissue. Obesity is considered a global epidemic. Major influences come from the western diet and sedentary lifestyles, but the exact mechanisms likely include a mixture of genetic and environmental factors. Obesity)
    • Emergence of significant steroid-related side effects during treatment
    • Relapsing disease course requiring protracted steroid regimen
  • Tocilizumab Tocilizumab Immunosuppressants
    • ​​IL-6 receptor Receptor Receptors are proteins located either on the surface of or within a cell that can bind to signaling molecules known as ligands (e.g., hormones) and cause some type of response within the cell. Receptors antagonist
    • Use recommended by ACR in addition to oral glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids over steroid monotherapy.[10]
    • Typical dose:
      • 162 mg SC weekly or every other week 
      • Typically, given alongside steroid therapy
    • Keep in mind:
      • Increases risk of opportunistic infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease
      • Contraindicated in individuals with concomitant severe active infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease (excluding COVID-19 COVID-19 Coronavirus disease 2019 (COVID-19) is an infectious disease caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) that mainly affects the respiratory system but can also cause damage to other body systems (cardiovascular, gastrointestinal, renal, and central nervous systems). )
      • Do not initiate if hepatic enzymes Enzymes Enzymes are complex protein biocatalysts that accelerate chemical reactions without being consumed by them. Due to the body’s constant metabolic needs, the absence of enzymes would make life unsustainable, as reactions would occur too slowly without these molecules. Basics of Enzymes are > 5x upper limit Limit A value (e.g., pressure or time) that should not be exceeded and which is specified by the operator to protect the lung Invasive Mechanical Ventilation of normal or if neutrophils Neutrophils Granular leukocytes having a nucleus with three to five lobes connected by slender threads of chromatin, and cytoplasm containing fine inconspicuous granules and stainable by neutral dyes. Innate Immunity: Phagocytes and Antigen Presentation < 2 x 109/L.
  • Methotrexate Methotrexate An antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA. Antimetabolite Chemotherapy
    • Typical dose: ​​7.5–15 mg/wk orally
    • The evidence to support use of methotrexate Methotrexate An antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA. Antimetabolite Chemotherapy is weaker than for tocilizumab Tocilizumab Immunosuppressants, but it is cheaper and more widely available.

Additional pharmacologic therapy[8,10,13]

  • Statins Statins Statins are competitive inhibitors of HMG-CoA reductase in the liver. HMG-CoA reductase is the rate-limiting step in cholesterol synthesis. Inhibition results in lowered intrahepatocytic cholesterol formation, resulting in up-regulation of LDL receptors and, ultimately, lowering levels of serum LDL and triglycerides. Statins are not recommended for the treatment of GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis.
  • Aspirin Aspirin The prototypical analgesic used in the treatment of mild to moderate pain. It has anti-inflammatory and antipyretic properties and acts as an inhibitor of cyclooxygenase which results in the inhibition of the biosynthesis of prostaglandins. Aspirin also inhibits platelet aggregation and is used in the prevention of arterial and venous thrombosis. Nonsteroidal Antiinflammatory Drugs (NSAIDs):
    • ACR conditionally recommends for patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship with flow-limiting lesions of the vertebral or carotid arteries Carotid Arteries Either of the two principal arteries on both sides of the neck that supply blood to the head and neck; each divides into two branches, the internal carotid artery and the external carotid artery. Carotid Arterial System: Anatomy.[10]
    • Royal College of Physicians Physicians Individuals licensed to practice medicine. Clinician–Patient Relationship recommends for all patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship with GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis (if no contraindications Contraindications A condition or factor associated with a recipient that makes the use of a drug, procedure, or physical agent improper or inadvisable. Contraindications may be absolute (life threatening) or relative (higher risk of complications in which benefits may outweigh risks). Noninvasive Ventilation).[13]

Additional counseling[8]

In addition to receiving education on their diagnosis, patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship should also receive counseling related to:

  • Smoking Smoking Willful or deliberate act of inhaling and exhaling smoke from burning substances or agents held by hand. Interstitial Lung Diseases cessation
  • Diet (e.g., mitigate risk of hyperglycemia Hyperglycemia Abnormally high blood glucose level. Diabetes Mellitus on glucocorticoid therapy)
  • Physical activity:
    • Activity may benefit patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship with individuals with GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis.
    • Prevents deconditioning Deconditioning Psychotherapy 

Differential Diagnosis

  • Stroke: disease in which poor blood flow Blood flow Blood flow refers to the movement of a certain volume of blood through the vasculature over a given unit of time (e.g., mL per minute). Vascular Resistance, Flow, and Mean Arterial Pressure to the brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification results in cell death Cell death Injurious stimuli trigger the process of cellular adaptation, whereby cells respond to withstand the harmful changes in their environment. Overwhelmed adaptive mechanisms lead to cell injury. Mild stimuli produce reversible injury. If the stimulus is severe or persistent, injury becomes irreversible. Apoptosis is programmed cell death, a mechanism with both physiologic and pathologic effects. Cell Injury and Death. There are 2 types: ischemic stroke Ischemic Stroke An ischemic stroke (also known as cerebrovascular accident) is an acute neurologic injury that occurs as a result of brain ischemia; this condition may be due to cerebral blood vessel occlusion by thrombosis or embolism, or rarely due to systemic hypoperfusion. Ischemic Stroke due to lack of blood flow Blood flow Blood flow refers to the movement of a certain volume of blood through the vasculature over a given unit of time (e.g., mL per minute). Vascular Resistance, Flow, and Mean Arterial Pressure, and hemorrhagic stroke Hemorrhagic stroke Stroke due to rupture of a weakened blood vessel in the brain (e.g., cerebral hemispheres; cerebellum; subarachnoid space). Subarachnoid Hemorrhage due to bleeding. Symptoms include focal weakness, numbness, loss of vision Vision Ophthalmic Exam, dysarthria Dysarthria Disorders of speech articulation caused by imperfect coordination of pharynx, larynx, tongue, or face muscles. This may result from cranial nerve diseases; neuromuscular diseases; cerebellar diseases; basal ganglia diseases; brain stem diseases; or diseases of the corticobulbar tracts. The cortical language centers are intact in this condition. Wilson Disease, and headache Headache The symptom of pain in the cranial region. It may be an isolated benign occurrence or manifestation of a wide variety of headache disorders. Brain Abscess. Computed tomography and MRI of the brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification are used for the diagnosis, and will differentiate stroke from GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis.
  • Migraine Migraine Migraine headache is a primary headache disorder and is among the most prevalent disorders in the world. Migraine is characterized by episodic, moderate to severe headaches that may be associated with increased sensitivity to light and sound, as well as nausea and/or vomiting. Migraine Headache: characterized by recurrent headaches that are moderate to severe, typically affecting ½ of the head, and pulsating in nature. Associated symptoms include nausea Nausea An unpleasant sensation in the stomach usually accompanied by the urge to vomit. Common causes are early pregnancy, sea and motion sickness, emotional stress, intense pain, food poisoning, and various enteroviruses. Antiemetics, vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia, and sensitivity to light, sound, or smell Smell The sense of smell, or olfaction, begins in a small area on the roof of the nasal cavity, which is covered in specialized mucosa. From there, the olfactory nerve transmits the sensory perception of smell via the olfactory pathway. This pathway is composed of the olfactory cells and bulb, the tractus and striae olfactoriae, and the primary olfactory cortex and amygdala. Olfaction: Anatomy. Diagnosis is clinical, based on the history and physical exam. Laboratory and imaging studies will be unremarkable. The history, physical exam, and low inflammatory markers will help differentiate migraine Migraine Migraine headache is a primary headache disorder and is among the most prevalent disorders in the world. Migraine is characterized by episodic, moderate to severe headaches that may be associated with increased sensitivity to light and sound, as well as nausea and/or vomiting. Migraine Headache from GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis.
  • Glaucoma Glaucoma Glaucoma is an optic neuropathy characterized by typical visual field defects and optic nerve atrophy seen as optic disc cupping on examination. The acute form of glaucoma is a medical emergency. Glaucoma is often, but not always, caused by increased intraocular pressure (IOP). Glaucoma: An increase in intraocular pressure Intraocular Pressure The pressure of the fluids in the eye. Ophthalmic Exam damages the optic nerve Optic nerve The 2nd cranial nerve which conveys visual information from the retina to the brain. The nerve carries the axons of the retinal ganglion cells which sort at the optic chiasm and continue via the optic tracts to the brain. The largest projection is to the lateral geniculate nuclei; other targets include the superior colliculi and the suprachiasmatic nuclei. Though known as the second cranial nerve, it is considered part of the central nervous system. The 12 Cranial Nerves: Overview and Functions and causes blindness Blindness The inability to see or the loss or absence of perception of visual stimuli. This condition may be the result of eye diseases; optic nerve diseases; optic chiasm diseases; or brain diseases affecting the visual pathways or occipital lobe. Retinopathy of Prematurity. Angle-closure glaucoma Angle-Closure Glaucoma Glaucoma symptoms include blurred vision Blurred Vision Retinal Detachment, halos around light, severe eye pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways, headache Headache The symptom of pain in the cranial region. It may be an isolated benign occurrence or manifestation of a wide variety of headache disorders. Brain Abscess, nausea Nausea An unpleasant sensation in the stomach usually accompanied by the urge to vomit. Common causes are early pregnancy, sea and motion sickness, emotional stress, intense pain, food poisoning, and various enteroviruses. Antiemetics, and vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia. Diagnosis is made by an ophthalmology exam and intraocular pressure Intraocular Pressure The pressure of the fluids in the eye. Ophthalmic Exam measurements, which will differentiate glaucoma Glaucoma Glaucoma is an optic neuropathy characterized by typical visual field defects and optic nerve atrophy seen as optic disc cupping on examination. The acute form of glaucoma is a medical emergency. Glaucoma is often, but not always, caused by increased intraocular pressure (IOP). Glaucoma from GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis.
  • Takayasu’s arteritis: a large-vessel, granulomatous vasculitis Vasculitis Inflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body. Systemic Lupus Erythematosus that primarily affects the aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy and its primary branches. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship are often young or middle-aged women. Signs and symptoms include tenderness of the carotid artery, limb claudication, and absent or weak pulses. Elevated inflammatory markers and CTA CTA A non-invasive method that uses a ct scanner for capturing images of blood vessels and tissues. A contrast material is injected, which helps produce detailed images that aid in diagnosing vascular diseases. Pulmonary Function Tests of the aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy can aid in the diagnosis. Treatment includes glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids and glucocorticoid-sparing agents. The patient’s demographics, history, and exam findings will differentiate Takayasu’s arteritis from GCA GCA Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis
  • Non-arteritic anterior ischemic optic neuropathy Neuropathy Leprosy: an idiopathic Idiopathic Dermatomyositis, ischemic injury to the optic nerve Optic nerve The 2nd cranial nerve which conveys visual information from the retina to the brain. The nerve carries the axons of the retinal ganglion cells which sort at the optic chiasm and continue via the optic tracts to the brain. The largest projection is to the lateral geniculate nuclei; other targets include the superior colliculi and the suprachiasmatic nuclei. Though known as the second cranial nerve, it is considered part of the central nervous system. The 12 Cranial Nerves: Overview and Functions. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship present with sudden, painless, and monocular vision Vision Ophthalmic Exam loss. A fundoscopic exam Fundoscopic Exam Head and Neck Examination will show optic disc Optic disc The portion of the optic nerve seen in the fundus with the ophthalmoscope. It is formed by the meeting of all the retinal ganglion cell axons as they enter the optic nerve. Eye: Anatomy swelling Swelling Inflammation. Giant cell arteritis Giant Cell Arteritis Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis should be considered in these individuals, and can be ruled out with normal inflammatory markers. There is no known effective treatment, but patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship are generally placed on aspirin Aspirin The prototypical analgesic used in the treatment of mild to moderate pain. It has anti-inflammatory and antipyretic properties and acts as an inhibitor of cyclooxygenase which results in the inhibition of the biosynthesis of prostaglandins. Aspirin also inhibits platelet aggregation and is used in the prevention of arterial and venous thrombosis. Nonsteroidal Antiinflammatory Drugs (NSAIDs).

References

  1. Cid, M. C. (2020). Pathogenesis of giant cell arteritis. UpToDate. Retrieved October 26, 2020, from https://www.uptodate.com/contents/pathogenesis-of-giant-cell-arteritis
  2. Docken, W. P., Rosenbaum, J. T. (2019). Clinical manifestations of giant cell arteritis. UpToDate. Retrieved October 25, 2020, from https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis
  3. Docken, W. P. (2019). Diagnosis of giant cell arteritis. UpToDate. Retrieved October 27, 2020, from https://www.uptodate.com/contents/diagnosis-of-giant-cell-arteritis
  4. Docken, W. P. (2020). Treatment of giant cell arteritis. UpToDate. Retrieved October 27, 2020, from https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis
  5. Seetharaman, M., Albertini, J. G., Paget, S. A. (2020). Giant cell arteritis (temporal arteritis). Medscape. Retrieved October 26, 2020, from https://emedicine.medscape.com/article/332483-overview
  6. Kasper, D.L., Fauci, A.S., Longo, D.L., Braunwald, E., Hauser, S.L., & Jameson, J.L. (2007). Harrison’s principles of internal medicine (16th edition.). New York: McGraw Hill Education.
  7. Al-Mousawi, A. Z., Gurney, S. P., Lorenzi, A. R., et al. (2019). Reviewing the pathophysiology behind the advances in the management of giant cell arteritis. Ophthalmology and Therapy, 8, 177–193. https://link.springer.com/article/10.1007%2Fs40123-019-0171-0
  8. Mackie S. L, Dejaco C., et al. (2020). British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology, 59(3), e1–e23. https://academic.oup.com/rheumatology/article/59/3/e1/5714024
  9. Scenario: Management | Management | Giant cell arteritis | CKS | NICE. Retrieved July 27, 2022, from https://cks.nice.org.uk/topics/giant-cell-arteritis/management/management/.
  10. Maz M., Chung S., et al. (2021). 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of giant cell arteritis and Takayasu arteritis. Arthritis Care & Research, 73(8), 1071–1087. https://www.rheumatology.org/Portals/0/Files/Guideline-Management-Giant-Cell-Arteritis-Takayasu-Arteritis-2021.pdf
  11. Ponte, C., et al. (2022). 2022 American College of Rheumatology/EULAR classification criteria for giant cell arteritis. Arthritis & Rheumatology, 74(12), 1881–1889. https://www.researchgate.net/publication/365260802_2022_American_College_of_RheumatologyEULAR_Classification_Criteria_for_Giant_Cell_Arteritis
  12. Ehlers, L., et al. (2019). 2018 EULAR recommendations for a core data set to support observational research and clinical care in giant cell arteritis. Annals of the Rheumatic Diseases, 78, 1160–1166. https://ard.bmj.com/content/78/9/1160
  13. Dasgupta, B. (2010). Diagnosis and management of giant cell arteritis. Clinical Medicine, 10(4). https://www.rcplondon.ac.uk/guidelines-policy/diagnosis-and-management-giant-cell-arteritis

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