Antineutrophil cytoplasmic antibody ( ANCA ANCA Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. Rapidly Progressive Glomerulonephritis)-associated 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 (AAV) includes granulomatosis with polyangiitis Granulomatosis with Polyangiitis A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (vasculitis) leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies (antineutrophil cytoplasmic antibodies) against myeloblastin. Granulomatosis with Polyangiitis ( GPA GPA A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (vasculitis) leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies (antineutrophil cytoplasmic antibodies) against myeloblastin. Granulomatosis with Polyangiitis), microscopic polyangiitis Microscopic polyangiitis A primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls. Vasculitides ( MPA MPA A primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls. Vasculitides), and eosinophilic granulomatosis with polyangiitis Granulomatosis with Polyangiitis A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (vasculitis) leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies (antineutrophil cytoplasmic antibodies) against myeloblastin. Granulomatosis with Polyangiitis (EGPA). All 3 diseases cause life-threatening small-vessel vasculitis Small-Vessel Vasculitis Henoch-Schönlein Purpura with a wide range of systemic manifestations, which can involve the lungs Lungs Lungs are the main organs of the respiratory system. Lungs are paired viscera located in the thoracic cavity and are composed of spongy tissue. The primary function of the lungs is to oxygenate blood and eliminate CO2. Lungs: Anatomy, kidneys Kidneys The kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine. Kidneys: Anatomy, skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Skin: Structure and Functions, and heart. Diagnosis is suspected by clinical presentation Presentation The position or orientation of the fetus at near term or during obstetric labor, determined by its relation to the spine of the mother and the birth canal. The normal position is a vertical, cephalic presentation with the fetal vertex flexed on the neck. Normal and Abnormal Labor and a positive cytoplasmic (c)- or perinuclear (p)-ANCA test. Biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma of involved tissue confirms the diagnosis. 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 immunosuppressive therapy are the mainstays of treatment.
Last updated: 8 Apr, 2022
Antineutrophil cytoplasmic antibody ( ANCA ANCA Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. Rapidly Progressive Glomerulonephritis)-associated 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 (AAV) is characterized by necrotizing small 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 and a positive ANCA ANCA Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. Rapidly Progressive Glomerulonephritis test without significant immune complex deposition.
AAV includes the following diseases:
Granular, cytoplasmic pattern of staining of proteinase 3 Proteinase 3 Granulomatosis with Polyangiitis (PR3)-antineutrophil cytoplasmic antibody ( ANCA ANCA Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. Rapidly Progressive Glomerulonephritis) (c-ANCA)
Image: “C anca ANCA Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. Rapidly Progressive Glomerulonephritis” by Malittle. License: Public DomainPerinuclear pattern of staining of myeloperoxidase Myeloperoxidase Acute Myeloid Leukemia ( MPO MPO Acute Myeloid Leukemia)-antineutrophil cytoplasmic antibody ( ANCA ANCA Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. Rapidly Progressive Glomerulonephritis) (p-ANCA)
Image: “P anca ANCA Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. Rapidly Progressive Glomerulonephritis” by Malittle. License: Public DomainPathogenesis of antineutrophil cytoplasmic antibody ( ANCA ANCA Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. Rapidly Progressive Glomerulonephritis) damage
Image by Lecturio.Antineutrophil cytoplasmic antibody ( ANCA ANCA Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. Rapidly Progressive Glomerulonephritis) autoantibody formation
Image by Lecturio.AAV presents with a wide range of signs and symptoms depending on the organ system(s) involved.
Constitutional symptoms may be present weeks or months before specific clinical features develop:
Example of the collapsed nasal bridge (saddle
nose
Nose
The nose is the human body’s primary organ of smell and functions as part of the upper respiratory system. The nose may be best known for inhaling oxygen and exhaling carbon dioxide, but it also contributes to other important functions, such as tasting. The anatomy of the nose can be divided into the external nose and the nasal cavity.
Nose and Nasal Cavity: Anatomy
deformity
Deformity
Examination of the Upper Limbs) seen in
granulomatosis with polyangiitis
Granulomatosis with Polyangiitis
A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (vasculitis) leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies (antineutrophil cytoplasmic antibodies) against myeloblastin.
Granulomatosis with Polyangiitis (
GPA
GPA
A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (vasculitis) leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies (antineutrophil cytoplasmic antibodies) against myeloblastin.
Granulomatosis with Polyangiitis):
The individual also shows orbital involvement with
proptosis
Proptosis
Retinoblastoma (protrusion of the eyeball), eyelid
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 limited ocular movements. Orbital involvement typically does not occur until years after disease onset.
Progression to diffuse alveolar hemorrhage in an individual with
granulomatosis with polyangiitis
Granulomatosis with Polyangiitis
A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (vasculitis) leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies (antineutrophil cytoplasmic antibodies) against myeloblastin.
Granulomatosis with Polyangiitis (
GPA
GPA
A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (vasculitis) leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies (antineutrophil cytoplasmic antibodies) against myeloblastin.
Granulomatosis with Polyangiitis):
A)
frontal
Frontal
The bone that forms the frontal aspect of the skull. Its flat part forms the forehead, articulating inferiorly with the nasal bone and the cheek bone on each side of the face.
Skull: Anatomy
chest X-ray
Chest X-ray
X-ray visualization of the chest and organs of the thoracic cavity. It is not restricted to visualization of the lungs.
Pulmonary Function Tests on admission
B)
frontal
Frontal
The bone that forms the frontal aspect of the skull. Its flat part forms the forehead, articulating inferiorly with the nasal bone and the cheek bone on each side of the face.
Skull: Anatomy
chest X-ray
Chest X-ray
X-ray visualization of the chest and organs of the thoracic cavity. It is not restricted to visualization of the lungs.
Pulmonary Function Tests in the same individual after 4 days
Multiple ulcerations in an individual with microscopic polyangiitis Microscopic polyangiitis A primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls. Vasculitides ( MPA MPA A primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls. Vasculitides)
Image: “UlcĂ©rations cutanĂ©es aux membres infĂ©rieurs” by Khammassi N, Chakroun A. License: CC BY 2.0Eyelid 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, scleritis Scleritis Refers to any inflammation of the sclera including episcleritis, a benign condition affecting only the episclera, which is generally short-lived and easily treated. Classic scleritis, on the other hand, affects deeper tissue and is characterized by higher rates of visual acuity loss and even mortality, particularly in necrotizing form. Crohn’s Disease, chemosis Chemosis Conjunctivitis, and subconjunctival hemorrhage in an individual with granulomatosis with polyangiitis Granulomatosis with Polyangiitis A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (vasculitis) leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies (antineutrophil cytoplasmic antibodies) against myeloblastin. Granulomatosis with Polyangiitis ( GPA GPA A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (vasculitis) leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies (antineutrophil cytoplasmic antibodies) against myeloblastin. Granulomatosis with Polyangiitis)
Image: “The eye involvement of Wegener’s granulomatosis and signs of an acute epistaxis Epistaxis Bleeding from the nose. Granulomatosis with Polyangiitis” by Bîrluţiu V, Rezi EC, Bîrluţiu RM, Zaharie IS. License: CC BY 4.0Suspected diagnosis:
Confirmation of diagnosis includes biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma of affected organs:
Other laboratories:
Imaging: chest X-ray Chest X-ray X-ray visualization of the chest and organs of the thoracic cavity. It is not restricted to visualization of the lungs. Pulmonary Function Tests and CT:
All individuals with a confirmed diagnosis of AAV require immunosuppressive therapy. Therapy can be initiated early based on a presumptive diagnosis of the following criteria:
Once remission Remission A spontaneous diminution or abatement of a disease over time, without formal treatment. Cluster Headaches is achieved, individuals switch to less toxic agents:
Table showing suggestive laboratory and clinical features of the 3 main types of AAV:
AAV disease | ANCA ANCA Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. Rapidly Progressive Glomerulonephritis | Granuloma on biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma | Typical case |
---|---|---|---|
GPA GPA A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (vasculitis) leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies (antineutrophil cytoplasmic antibodies) against myeloblastin. Granulomatosis with Polyangiitis |
|
+ | Adult with:
|
MPA MPA A primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls. Vasculitides |
|
– | Similar to GPA GPA A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (vasculitis) leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies (antineutrophil cytoplasmic antibodies) against myeloblastin. Granulomatosis with Polyangiitis, but without serious nasal/sinus disease (only in 30%) |
EGPA |
|
+ | Young adult with:
|