Pulmonary Arterial Hypertension-A Deadly Complication of Systemic Sclerosis

Introduction

Pulmonary complications of systemic sclerosis (SSc) are a life threatening and commonly observed outcome. Pulmonary arterial hypertension (PAH) and pulmonary fibrosis are major contributors to mortality in the SSc patient, responsible for 61% of SSc-related deaths (PAH 26% and fibrosis 35%).1 The diagnosis of SSc alone carries an estimated 50% 10-year mortality.2–(4) When PAH develops in the SSc patient, the median survival rate falls, with reports as low as 1 year.4

Abstract

Pulmonary arterial hypertension (PAH) is a devastating disease with limited therapeutic options. Moreover, when PAH occurs in patients diagnosed with systemic sclerosis, worse outcomes are observed. The purpose of this review is to discuss the etiologies of PAH found in the systemic sclerosis patient, limitations of current medical therapies, and, finally, potential therapies for patients with this combination.

Keywords

connective tissue disease, pulmonary arterial hypertension, pulmonary hypertension, systemic sclerosis