engleski

Systemic sclerosis-associated pulmonary disorders

Systemic sclerosis is a chronic, progressive multiorgan disease in which considerable fibrosis, vascular alterations, and autoantibodies against diverse antigens are among the principal features. The disease is of unknown cause. Most frequent extracutaneous involvement is the lung. Interstitial lung disease and pulmonary hypertension are the most common clinical expressions of lung involvement and most serious events concerning the prognosis. Clinically significant interstitial lung disease in patients with systemic sclerosis is observed in about 40% of cases, varying from 19% to 47. The rate of pulmonary hypertension varies between 8% and 32% in different studies. The prognosis of patients with systemic sclerosis today in most instances depends upon pulmonary complications. The systemic sclerosis associated interstitial lung disease and pulmonary hypertension will be described and their current and future therapies presented.

Sytemic sclerosis, pulmonary manifestations

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