Sarcoidosis is a multisystem inflammatory disease that causes noncaseating granulomas Noncaseating granulomas Crohn’s Disease. The exact etiology is unknown. Sarcoidosis usually affects 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 and thoracic lymph nodes Lymph Nodes They are oval or bean shaped bodies (1 - 30 mm in diameter) located along the lymphatic system. Lymphatic Drainage System: Anatomy, but it can also affect Affect The feeling-tone accompaniment of an idea or mental representation. It is the most direct psychic derivative of instinct and the psychic representative of the various bodily changes by means of which instincts manifest themselves. Psychiatric Assessment almost every system in the body, including the 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, heart, and eyes, most commonly. Acutely, sarcoidosis presents with lymphadenopathy Lymphadenopathy Lymphadenopathy is lymph node enlargement (> 1 cm) and is benign and self-limited in most patients. Etiologies include malignancy, infection, and autoimmune disorders, as well as iatrogenic causes such as the use of certain medications. Generalized lymphadenopathy often indicates underlying systemic disease. Lymphadenopathy, 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, malaise Malaise Tick-borne Encephalitis Virus, joint pains, a panniculitis Panniculitis General term for inflammation of adipose tissue, usually of the skin, characterized by reddened subcutaneous nodules. Erythema Nodosum on the shins Shins Erythema Nodosum known as erythema Erythema Redness of the skin produced by congestion of the capillaries. This condition may result from a variety of disease processes. Chalazion nodosum, and occasionally cough and shortness of breath Shortness of breath Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea. Chronic pulmonary sarcoidosis presents with an insidious onset of dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea, cough, chest pain Chest Pain Chest pain is one of the most common and challenging complaints that may present in an inpatient and outpatient setting. The differential diagnosis of chest pain is large and includes cardiac, gastrointestinal, pulmonary, musculoskeletal, and psychiatric etiologies. Chest Pain, and a variety of other symptoms depending on the organ systems involved. Diagnosis often requires a biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma of the granulomas. Management includes observation, NSAIDs NSAIDS Primary vs Secondary Headaches, 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 potentially one of several steroid-sparing agents. Acute sarcoidosis is usually self-limiting Self-Limiting Meningitis in Children with an excellent prognosis Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas, but chronic sarcoidosis can lead to severe pulmonary fibrosis Pulmonary Fibrosis Idiopathic pulmonary fibrosis is a specific entity of the major idiopathic interstitial pneumonia classification of interstitial lung diseases. As implied by the name, the exact causes are poorly understood. Patients often present in the moderate to advanced stage with progressive dyspnea and nonproductive cough. Pulmonary Fibrosis.
Last updated: 12 Mar, 2021
Sarcoidosis is a multisystem inflammatory disease characterized by the formation of noncaseating granulomas Noncaseating granulomas Crohn’s Disease that are most likely caused by a cell-mediated immune reaction of unknown etiology.
Pulmonary sarcoidosis is a restrictive interstitial lung disease with granuloma formation in the:
Extrapulmonary sarcoidosis is characterized by granuloma formation in:
Sarcoidosis may be acute or chronic:
Categorization
Categorization
Types of Variables of
interstitial lung diseases
Interstitial Lung Diseases
Interstitial lung diseases are a heterogeneous group of disorders characterized by the inflammation and fibrosis of lung parenchyma, especially the pulmonary connective tissue in the alveolar walls. It may be idiopathic (e.g., idiopathic pulmonary fibrosis) or secondary to connective tissue diseases, medications, malignancies, occupational exposure, or allergens.
Interstitial Lung Diseases
ILD
ILD
Interstitial lung diseases are a heterogeneous group of disorders characterized by the inflammation and fibrosis of lung parenchyma, especially the pulmonary connective tissue in the alveolar walls. It may be idiopathic (e.g., idiopathic pulmonary fibrosis) or secondary to connective tissue diseases, medications, malignancies, occupational exposure, or allergens.
Interstitial Lung Diseases: interstitial lung disease
Etiology is undetermined but is most likely multifactorial.
Theory: An infectious Infectious Febrile Infant or environmental agent triggers Triggers Hereditary Angioedema (C1 Esterase Inhibitor Deficiency) a cell-mediated inflammatory immune response in a genetically susceptible host, leading to granuloma formation.
Potential triggering exposures:
Genetic predispositions:
Sarcoidosis is the result of a cell-mediated immune reaction.
Noncaseating granuloma Noncaseating Granuloma Inflammation
Image: “Granuloma 20x” by Mutleysmith. License: Public DomainSarcoidosis usually progresses slowly, with few symptoms initially. Symptoms appear as an increasing number of granulomas begin to affect Affect The feeling-tone accompaniment of an idea or mental representation. It is the most direct psychic derivative of instinct and the psychic representative of the various bodily changes by means of which instincts manifest themselves. Psychiatric Assessment organ function.
Organ system | 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 of sarcoidosis |
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Systemic symptoms (typically in acute presentations) |
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Pulmonary |
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Lymph Lymph The interstitial fluid that is in the lymphatic system. Secondary Lymphatic Organs |
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Ocular |
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Cutaneous |
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Musculoskeletal |
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Cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) |
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Nervous system Nervous system The nervous system is a small and complex system that consists of an intricate network of neural cells (or neurons) and even more glial cells (for support and insulation). It is divided according to its anatomical components as well as its functional characteristics. The brain and spinal cord are referred to as the central nervous system, and the branches of nerves from these structures are referred to as the peripheral nervous system. Nervous System: Anatomy, Structure, and Classification |
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Other potential sites of involvement |
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An acute 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 of sarcoidosis with a classic triad of symptoms:
Erythema Erythema Redness of the skin produced by congestion of the capillaries. This condition may result from a variety of disease processes. Chalazion nodosum
Image: “Bilateral shin Shin Erythema Nodosum lesions” by MRC Clinical Trials Unit at UCL, Aviation House, 125 Kingsway, London, WC2B 6NH, UK. License: CC BY 4.0Lupus pernio Pernio Recurrent localized itching, swelling and painful erythema on the fingers, toes or ears, produced by exposure to cold. Frostbite: cutaneous lesions of sarcoidosis
Image: “Cutaneous lesions of sarcoidosis” by M. Sand et al AL Amyloidosis. License: CC BY 2.0Diagnosing sarcoidosis typically requires:
Chest
X-ray
X-ray
Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source.
Pulmonary Function Tests in a patient with chronic sarcoidosis:
Green circles denote areas of upper lobe interstitial infiltrates. There is also
distortion
Distortion
Defense Mechanisms of the
hilum
Hilum
Lungs: Anatomy.
Non–contrast-enhanced chest CT scan demonstrating pulmonary nodules Pulmonary nodules A number of small lung lesions characterized by small round masses of 2- to 3-mm in diameter. They are usually detected by chest ct scans. Such nodules can be associated with metastases of malignancies inside or outside the lung, benign granulomas, or other lesions. Granulomatosis with Polyangiitis and calcified mediastinal and hilar lymphadenopathy Lymphadenopathy Lymphadenopathy is lymph node enlargement (> 1 cm) and is benign and self-limited in most patients. Etiologies include malignancy, infection, and autoimmune disorders, as well as iatrogenic causes such as the use of certain medications. Generalized lymphadenopathy often indicates underlying systemic disease. Lymphadenopathy
Image: “Noncontrast-computed tomography scan” by Penn State/Milton S, Hershey Medical Center Department of Medicine, 500 University Drive, Hershey PA 17033, Pennsylvania, USA. License: CC BY 4.0Noncaseating granuloma Noncaseating Granuloma Inflammation
Image: “Granuloma 20x” by Mutleysmith. License: Public DomainObtained to assess severity of respiratory involvement and monitor disease; less helpful in actually establishing diagnosis
These tests are important in assessing and monitoring the extent of disease rather than making the diagnosis. The following findings are often seen in sarcoidosis.
Stage | Chest X-ray X-ray Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source. Pulmonary Function Tests | Symptoms | Frequency |
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0 | Extrapulmonary disease Extrapulmonary disease Blastomyces/Blastomycosis only | None | 10% |
1 | BHL only | None or mild cough | 50% |
2 | BHL plus parenchymal infiltrates | None, dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea, or cough | 25% |
3 | Parenchymal infiltrates only | Dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea | 10% |
4 | Advanced pulmonary fibrosis Pulmonary Fibrosis Idiopathic pulmonary fibrosis is a specific entity of the major idiopathic interstitial pneumonia classification of interstitial lung diseases. As implied by the name, the exact causes are poorly understood. Patients often present in the moderate to advanced stage with progressive dyspnea and nonproductive cough. Pulmonary Fibrosis | Dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea | 5% |
Management is based on stage and location of disease and includes:
Stage | Remission Remission A spontaneous diminution or abatement of a disease over time, without formal treatment. Cluster Headaches rate |
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0 | – |
1 | 90% |
2 | 70% |
3 | 20% |
4 | < 5% |