Rheumatic diseases clinics of North America
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Neurosarcoidosis occurs in 3% to 10% of patients with sarcoidosis. Cranial neuropathy and meningeal involvement are the most common manifestations, but any part of the nervous system can be affected. ⋯ Moderate to high dose of glucocorticoids is the main therapy for neurosarcoidosis. Relapse often occurs after the dose of glucocorticoids is tapered down, often necessitating the use of steroid-sparing immunosuppressive agents.
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Rheum. Dis. Clin. North Am. · Aug 2017
ReviewGenomics of Systemic Lupus Erythematosus: Insights Gained by Studying Monogenic Young-Onset Systemic Lupus Erythematosus.
Systemic lupus erythematosus (SLE) is a systemic, autoimmune, multisystem disease with a heterogeneous clinical phenotype. Genome-wide association studies have identified multiple susceptibility loci, but these explain a fraction of the estimated heritability. ⋯ This article highlights insights into the pathogenesis of SLE provided by these monogenic diseases. It examines genetic causes of complement deficiency, abnormal interferon production, and abnormalities of tolerance, resulting in monogenic SLE with overlapping clinical features, autoantibodies, and shared inflammatory pathways.
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Sjögren's disease is associated with a high burden of illness, diminished quality of life, and increased health care costs. The Sjögren's Syndrome Foundation developed the first US clinical practice guidelines for management of the oral, ocular, and rheumatologic or systemic manifestations. ⋯ This initiative should improve the quality and consistency of care for Sjögren's disease in the United States, guide insurance reimbursement, and define areas for future study. Guidelines will be periodically reviewed and revised as new information becomes available.
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Glucocorticoid-induced osteoporosis (GIOP) is one of the most common and serious adverse effects associated with glucocorticoid use. This article highlights GIOP pathophysiology, epidemiologic associations, effective treatment, and lifestyle modifications that can reduce fracture risk for long-term glucocorticoid users and additionally emphasizes the importance of early intervention.