Pulmonary hypertension in autoimmune rheumatic diseases: Where are we now?.
Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (mPAP) >25 mm Hg at rest or >30 mm Hg with exercise (1). PH is an increasingly recognized complication of the autoimmune rheumatic diseases (ARDs), and since other manifestations respond to therapy, it is becoming an important cause of morbidity, leading to right-sided cardiac overload, with exercise intolerance, dyspnea, arrhythmia, and premature death. During the last decade, prevalence data for PH in ARDs have accumulated, together with some initial observations about clinical, laboratory, and immunologic features that may have value in predicting which individuals are at highest risk for developing PH. Therapeutic strategies, largely adopted from primary PH (PPH), have been applied to ARDs with some encouraging results, and quite recently, results of controlled trials have become available. In this review, we briefly describe our current understanding of the pathogenic mechanisms of PH, summarize the data on
