Non-invasive ventilation in hypercapnic respiratory failure secondary to sclerodermic chest wall restriction

Abstract

Pulmonary involvement in scleroderma commonly presents as diffuse, progressive fibrosis (1,2). Rarely, chest wall skin infiltration can manifest in a restrictive pattern with Type II (hypercapnic) respiratory failure (3). This can often prove difficult to manage. Here, we present a case of severe sclerodermic chest wall restriction which was significantly improved by nocturnal intermittent non-invasive positive pressure ventilation (NIPPV).