Sarcoidosis

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sarcoidosis

[‚sär‚kȯi′dō·səs]
(medicine)
A disease of unknown etiology characterized by granulomatous lesions, somewhat resembling true tubercles, but showing little or no necrosis, affecting the lymph nodes, skin, liver, spleen, heart, skeletal muscles, lungs, bones in distal parts of the extremities (osteitis cystica of Jüngling), and other structures, and sometimes by hyperglobulinemia, cutaneous anergy, and hypercalcinuria.

Sarcoidosis

 

in man, a systemic granulomatous disease that affects various organs and tissues and produces a variety of symptoms. It was initially described as a skin disease in 1889 by the French dermatologist E. Besnier and the Norwegian dermatologist C. Boeck. Sarcoidosis was first studied as a systemic disease by the Swedish dermatologist J. Schaumann in the first quarter of the 20th century.

The etiology of sarcoidosis is unknown. The disease was once believed to be tuberculous in origin, but this theory has been abandoned by most specialists. Sarcoidosis seems to be similar to reticulosis. It affects the skin (sarcoids of Boeck and other manifestations), the peripheral lymph nodes, and, most commonly, the thoracic lymph nodes and the lungs. Sarcoidosis of the eyes is often in the form of iridocyclitis, and sarcoidosis of the bones is often in the form of osteitis fibrosa cystica. The nervous system and other organs are less commonly affected. There is no cardinal symptom.

Sarcoidosis is diagnosed on the basis of all available data. The disease process, which is usually chronic, is studied, as are the roentgenologic symptoms of pulmonary involvement. The morphological structure is examined: sarcoid granuloma, which consists of epithelioid, lymphoid, and giant noncaseating cells, is characteristic.

The prognosis is usually favorable, and spontaneous involution, especially of the thoracic lesions, is possible. Relapses are common. The prognosis is worse if pneumosclerosis has developed and if the eyes and central nervous system are involved. When the disease is not treated, the mortality rate is approximately 5 percent. Sarcoidosis is treated by the prolonged administration of corticosteroids.

REFERENCES

Raben, A. S. Sarkoidoz. Moscow, 1964. (Bibliography.)
Lebacq, E. La sarcoïdose de Besnier-Boeck-Schaumann. Brussels, 1964.
5th International Conference on Sarcoidosis. Prague, 1971.

A. S. RABEN

References in periodicals archive ?
As cardiac sarcoidosis progresses, the granulomatous inflammation elicits a repair response with scarring.
Endomyocardial biopsy can provide a definitive diagnosis of cardiac sarcoidosis but has low sensitivity due to the patchy distribution of granulomas and lower incidence of disease in the right heart (more accessible for biopsy) compared with the left heart and septum.
The primary histologic differential diagnosis for cardiac sarcoidosis includes giant cell myocarditis, idiopathic (nonspecific) granulomatous myocarditis, tuberculous myocarditis, fungal myocarditis, and Whipple disease.
The two entities at the top of the histologic differential diagnosis for cardiac sarcoidosis are idiopathic granulomatous myocarditis and idiopathic giant cell myocarditis.
whereas the lymphocytes in cardiac sarcoidosis are predominantly [CD4.
The anatomic distribution of cardiac disease, serologic test results, and other clinical features can also help differentiate systemic lupus erythematosus involving the heart from cardiac sarcoidosis.
66) Both rheumatoid arthritis and sarcoidosis generally involve extracardiac organs and the clinical manifestations of disease in these other organs can also make it easy to differentiate rheumatoid arthritis involving the heart from cardiac sarcoidosis.
Like the other entities in the expanded differential diagnosis, giant cell arteritis has epidemiologic and clinical features that help distinguish it from cardiac sarcoidosis.
The epidemiologic and clinical features of tertiary syphilis are also helpful in differentiating it from cardiac sarcoidosis, and chief among these is the current rarity of tertiary syphilis.