Rheumatic diseases clinics of North America
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Rheum. Dis. Clin. North Am. · May 2015
ReviewConnective tissue disease-associated pulmonary arterial hypertension.
Pulmonary arterial hypertension (PAH) is characterized by vascular remodeling of pulmonary arterioles that leads to increased pulmonary vascular resistance, right heart failure, and death. It is associated with connective tissue diseases, including systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease. PAH is characterized by dyspnea on exertion and fatigue. ⋯ Patients may present with signs of right heart failure. One- and 3-year survival rates are approximately 81% and 52%, respectively. Given the high prevalence and mortality, algorithms for screening are currently under investigation and will hopefully lead to earlier diagnosis and improved survival.
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Rheum. Dis. Clin. North Am. · Jan 2015
ReviewCurrent landscape of antineutrophil cytoplasmic antibody-associated vasculitis: classification, diagnosis, and treatment.
This article provides an update on the diagnosis and management of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, granulomatosis with polyangiitis (formerly Wegener), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss). Focus is on new schemes of classification and the importance of ANCAs in the diagnosis and prognosis of these systemic vasculitides. Current therapeutic strategies consisting of glucocorticoids in conjunction with conventional or biologic agents for both induction of remission and remission maintenance are outlined. Future research directions include investigation of the optimal duration and frequency of maintenance therapy and development of targeted therapeutic agents.
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Musculoskeletal ultrasound has emerged as a key tool for the diagnosis, prognosis, and management of patients with RA (rheumatoid arthritis) and other rheumatic diseases. The most important sonographic findings in RA include erosions, effusions, synovitis, and tenosynovitis. Investigators have suggested various "optimal" numbers of joints to scan in RA to assess disease activity, gauge treatment response, provide prognostic information, and guide management decisions. The complexity of pediatric sonoanatomy has delayed its validation in juvenile idiopathic arthritis, yet ultrasound reliably measures the extent of synovitis/tenosynovitis and guides precise injections.
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Sarcoidosis is a systemic disease characterized by the development of epithelioid granulomas in various organs. Although the lungs are involved in most patients with sarcoidosis, virtually any organ can be affected. Recognition of extrapulmonary sarcoidosis requires awareness of the organs most commonly affected, such as the skin and the eyes, and vigilance for the most dangerous manifestations, such as cardiac and neurologic involvement. In this article, the common extrapulmonary manifestations of sarcoidosis are reviewed and organ-specific therapeutic considerations are discussed.