Seminars in respiratory and critical care medicine
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Sarcoidosis is an immune-mediated systemic syndrome of unknown etiology. The treatment of the granulomatous inflammation in sarcoidosis is thus dependent on nonspecific suppression of the immune response. ⋯ For patients with persistent disease, or for patients who are intolerant of systemic steroids, there are several steroid-sparing immune-modulating medications that have been effective for treating sarcoidosis. Besides immunosuppressives, we will also overview several sarcoidosis-related issues that contribute to patient-reported symptoms, a "sarcoidosis penumbra." Recognition and judicious therapy for these related issues are often key to optimizing outcomes and quality of life.
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Sarcoidosis is a systemic disease with skin manifestations. Skin manifestations are classified as nonspecific if they are not characterized by granulomatous inflammation and specific if the lesions have granulomas histologically. Erythema nodosum is the most common nonspecific skin manifestation, and it portends a good prognosis. ⋯ Local and systemic corticosteroids are the mainstay of treatment for the disease. Corticosteroid-sparing agents used to manage the disease include antimalarials, methotrexate, and tetracycline antibiotics. Tumor necrosis factor-alpha (TNF-alpha) antagonists such as infliximab may have a role in cutaneous sarcoidosis, especially in refractory cases that are resistant to the standard regimens.
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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
ReviewSarcoidosis-associated pulmonary hypertension: assessment and management.
Pulmonary hypertension (PH) is a recognized complication of sarcoidosis, with increased morbidity and poor prognosis. Sarcoidosis-associated pulmonary hypertension (SAPH) is typically seen in advanced cases, with pulmonary fibrosis, destruction and obliteration of the pulmonary vasculature, and chronic hypoxemia. PH can, however, occur in the absence of pulmonary fibrosis, suggesting alternative pathophysiological mechanisms. ⋯ This has encouraged the study of PAH-specific therapeutic agents in the treatment of SAPH. In small series, prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors have been shown to improve hemodynamics, functional status, and outcomes. This article reviews the most recent data available in the epidemiology, pathophysiology, diagnosis, and treatment of SAPH.