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Pathology and pathogenesis of sarcoidosis

Andrew Fontenot, MD
Talmadge E King, Jr, MD
Section Editors
Kevin R Flaherty, MD, MS
Andrew Nicholson, MD
Deputy Editor
Helen Hollingsworth, MD


Sarcoidosis is a multisystem disorder of unknown etiology characterized by the accumulation of T lymphocytes, mononuclear phagocytes, and noncaseating granulomas in involved tissues [1,2]. The lungs are affected in approximately 90 percent of patients, and pulmonary disease accounts for the majority of the morbidity and mortality associated with this disease. Other tissues commonly involved include the skin, eyes, and lymph nodes (table 1).

The pathology and pathogenesis of sarcoidosis will be reviewed here. The pulmonary and extrapulmonary clinical manifestations, diagnosis, and treatment are discussed separately. (See "Clinical manifestations and diagnosis of pulmonary sarcoidosis" and "Extrapulmonary manifestations of sarcoidosis" and "Cardiac sarcoidosis" and "Cutaneous manifestations of sarcoidosis" and "Gastrointestinal and hepatic sarcoidosis" and "Neurologic sarcoidosis" and "Renal disease in sarcoidosis" and "Treatment of pulmonary sarcoidosis: Initial therapy with glucocorticoids" and "Management of cutaneous sarcoidosis".)


Sarcoidosis has a predilection for the upper lung zones and bronchovascular bundles more than other lung compartments, although it can affect any area [3-5]. Lung involvement is often associated with hilar and mediastinal lymphadenopathy.

On histopathology, classic sarcoid granulomas are non-necrotizing with a tightly packed central area composed of macrophages, epithelioid cells, multinucleated giant cells, and T lymphocytes that are CD4 positive (picture 1) [3,5]. The central areas are surrounded by CD8 and CD4 positive T lymphocytes, B lymphocytes, monocytes, mast cells, and fibroblasts, which in turn are surrounded by lamellar rings of hyaline collagen. The proportions of lymphocytic infiltrate and fibrosis surrounding the granulomas vary depending on the patient and disease duration. Additional histopathologic features of sarcoid granulomas that may be present include asteroid bodies, Schaumann bodies, and birefringent crystalline particles (calcium oxalate and other calcium salts (picture 2 and picture 3 and picture 4)).

In rare patients with necrotizing sarcoid granulomatosis, areas of fibrinoid necrosis are noted within the granulomas.

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Literature review current through: Oct 2017. | This topic last updated: Nov 02, 2017.
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