Editorial Review: Pulmonary Hypertension in Systemic Sclerosis: Bete Noire No More?
Posted Dec 02 2010 9:37pm
As the articles in this section of Current Opinion in Rheumatology make clear, substantial advances in the management of systemic sclerosis (SSc) have been acheived. Nowhere is progress more dramatic and gratifying than in the treatment of SSc-associated pulmonary arterial hypertension (PAH). When first recognized over 2 decades ago, isolated PAH in patients with SSc was regarded as an infrequent late complication of the CREST (calcinosis, Raynaud, esophageal, dysmotility, sclerodactyly, telangiectasia) variant of the disease. In a characteristically insightful editorial entitled " Pulmonary hypertension : the bete noire of the diffuse connective tissue diseases" the late Carwile Leroy wrote of "an especially destructive type of vascular involvement" in the lungs, with abrupt onset and often lethal outcome. As the management of scleroderma renal crisis and pulmonary fibrosis became more successful, PAH emerged as the most severe complication and leading cause of death in SSc.
We now know that PAH is actually not rare in SSc. Rather, PAH may be regarded as one of the manifestations of the widespread small vessel arteriopathy that characterizes this disease. During the past decade, intensive research has yielded greatly increased understanding of the cellular and molecular pathophysiology of PAH. These insights have informed the emergence of novel paradigms for the treatment of PAH. Consequently, we can now add powerful new drugs to our therapeutic armamentarium for SSc. This triumph of bench-to-bedside research is critically reviewed in this issue by Lionel Schachna, MBBS, FRACP, and Fredrick M. Wigley, MD, while below I focus on clinical features of PAH and contemporary approaches to its evaluation and management.