Systemic lupus erythematosus-associated PAH: is targeting inflammation the key to success?

Abstract

Pulmonary arterial hypertension (PAH) refers to a spectrum of diseases characterised by a permanent increase in pulmonary vascular resistance leading to right ventricular dysfunction and, ultimately, death [1]. PAH can be idiopathic, familial or associated with a number of conditions or diseases, such as congenital heart disease, portal hypertension, HIV infection, and exposure to toxins and drugs, including appetite suppressants. PAH may also develop in the context of connective tissue disease (CTD), and may complicate the course of systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and mixed connective tissue disease (MCTD) [2, 3]. Over the past decade, significant advances in the better understanding of PAH pathophysiology have led to the development of “specific” therapies that target aberrant molecular signalling pathways. Although these treatments favourably impact clinical status, long-term survival remains poor for patients with PAH, particularly for those with CTD [2, 4]. In the current issue of the European Respiratory Review , Johnson and Granton [5] give an overview of the epidemiology, prognostic factors and survival of SSc- and SLE-associated PAH. In this review, important differences between these two forms of PAH are highlighted. SSc is …