Seminars in respiratory and critical care medicine
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Sarcoidosis is a multisystemic inflammatory granulomatous disease that affects both the central and peripheral nervous system. The neurological manifestations depend on the areas of the neuroaxis affected. In the brain, patients with neurosarcoidosis have leptomeningeal and intraparenchymal infiltration of granulomas that leads to, for example, cranial nerve palsies, basal meningitis, and endocrine dysfunction. ⋯ Diagnosis and management of patients with neurosarcoidosis are challenging given that the gold standard is tissue-proven biopsy, which, in most cases of neurological illness, is difficult to obtain. Treatment strategies have not been rigorously evaluated but corticosteroids are considered the drug of choice. Other immunosuppressant agents such as cyclophosphamide, mycophenolate mofetil, and infliximab are efficacious in the treatment of neurosarcoidosis.
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Semin Respir Crit Care Med · Aug 2010
The etiologic role of infectious antigens in sarcoidosis pathogenesis.
Sarcoidosis is a disease of unknown etiology, characterized pathologically by noncaseating granulomas that most commonly involve the lung, skin, lymph nodes, and eyes. Syndromes with similar pathological and immunologic features to sarcoidosis such as chronic beryllium disease, hypersensitivity pneumonitis, and tuberculosis illustrate that granulomatous diseases may or may not have an infectious etiology. Although the etiology of sarcoidosis remains unknown, recent molecular, genetic, and immunologic studies strengthen the association of sarcoidosis with infectious antigens. ⋯ Recent discoveries regarding MHC class II alleles provide additional insight regarding the role of microbial antigens in sarcoidosis pathogenesis. Although further investigation is warranted, the recent progress of independent laboratories, using complementary techniques, strengthens the role of microbial antigens in sarcoidosis pathogenesis. These studies lay a strong foundation toward identifying therapeutic targets.