Sarcoidosis (clinical lecture)

Le résumé fourni par la source

Sarcoidosis (S) is a systemic inflammatory disease of unknown etiology characterized by the formation of noncaseating granulomas, multisystemic organ involvement, and T-cell activation at the site of granulomatous inflammation with the release of various chemokines and cytokines. In Russia, a large team of specialists in various fields has developed clinical guidelines. Primary diagnosis of S. requires comprehensive laboratory and instrumental studies, including imaging diagnostics, and treatment is recommended only in cases of disease progression and a life-threatening course. Symptoms of S. vary widely, from acute to asymptomatic. Spontaneous remissions occur in up to 70% of cases, but fibrosis may develop in 10-15%. Dangerous variants of S. include cardiac sarcoidosis, sarcoidosis of the nervous system, and sarcoidosis of the eyes. In stable cases, long-term use of alpha-tocopherol and pentoxifylline is possible. Systemic glucocorticosteroids are considered first-line treatment for progressive sarcoidosis, with a starting dose of at least 20 mg per day and a duration of at least 10- 12 months. However, this therapy is more often associated with relapses, and low doses and short courses more often lead to subsequent fibrosis. Second-line treatments include methotrexate, but also leflunomide, azathioprine, mycophenolate, and cyclophosphamide. Third-line treatments include adalimumab and infliximab, but they themselves can cause sarcoid reactions. In refractory and recurrent cases, efferent therapies are used. The authors invite readers to discuss the updated version of clinical guidelines, available on the website of the Russian Respiratory Society to continue working on them.

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