Sarcoidosis

Abstract

Sarcoidosis is a chronic granulomatous disorder of unknown cause with worldwide prevalence. Although the lung is the most common organ affected, virtually any organ system may be involved with the disease and physicians of various specialities may come across patients with sarcoidosis in their practice. Owing to the lack of a diagnostic gold standard, the diagnosis is one of exclusion and requires compatible clinical‐radiological features supported by the presence of noncaseating granulomatous in biopsy specimen after exclusion of alternative causes of granulomatous inflammation. The majority of patients experience resolution within 2–5 years, but in a sizeable minority, the disease runs a chronic progressive course and may be refractory to conventional corticosteroid therapy. The scarcity of effective therapies and the lack of reliable predictors of clinical course greatly contribute to making sarcoidosis such a challenging disease to manage.

Key Concepts

  • Sarcoidosis is a systemic granulomatous disorder of unknown origin that affects almost invariably the lung.
  • Sarcoidosis is thought to result from a complex interplay between infectious/environmental and host/genetic factors leading to an aberrant immune response.
  • Sarcoidosis is more common and severe in African Americans.
  • The diagnosis of sarcoidosis requires the combination of compatible clinical and radiological features, detection of noncaseating granulomas in affected organs and exclusion of other diseases that may present similarly.
  • In asymptomatic patients, the finding of bilateral hilar lymphadenopathy on chest radiography is virtually diagnostic of sarcoidosis.
  • Sarcoidosis is generally benign and the majority of patients do not require treatment; however, approximately one‐third of the patients develop chronic disease and may need long‐term treatment.
  • Corticosteroids are the mainstay of treatment despite the paucity of adequate supportive data.
  • Extrapulmonary manifestations of the disease are a major cause of morbidity.
  • Patients with refractory or life/organ‐threatening disease should be referred to expert centres.

Keywords: sarcoidosis; epidemiology; diagnosis; pathology; imaging; clinical manifestations; Löfgren syndrome; treatment

Figure 1. (a) Extensive infiltration by noncaseating granulomas (arrows) (low magnification). (b) Accumulation of T‐lymphocytes, macrophages, epithelioid cells and multinucleated giant cells organised into discrete granulomas (high magnification).
Figure 2. Symmetrical bilateral hilar lymphadenopathy without parenchymal infiltrates (radiographic stage 1).
Figure 3. Micronodules with a typical perilymphatic distribution.
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Further Reading

Baughman RP , Lower EE and Drent M (2008) Inhibitors of tumor necrosis factor (TNF) in sarcoidosis: who, what, and how to use them. Sarcoidosis, Vasculitis, and Diffuse Lung Diseases 25: 76–89.

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Rossi G , Cavazza A and Colby TV (2015) Pathology of sarcoidosis. Clinical Reviews in Allergy and Immunology 49 (1): 36–44.

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Spagnolo, Paolo(Jul 2019) Sarcoidosis. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1002/9780470015902.a0028610]