Scleroderma lung disease

Abstract

Systemic sclerosis (SSc) is a systemic autoimmune disease that is characterised by endothelial dysfunction resulting in a small-vessel vasculopathy, fibroblast dysfunction with resultant excessive collagen production and fibrosis, and immunological abnormalities. The classification of SSc is subdivided based on the extent of skin involvement into diffuse cutaneous sclerosis (dcSSc), limited cutaneous sclerosis (lcSSc) or SSc sine scleroderma [1]. While virtually any organ system may be involved in the disease process, fibrotic and vascular pulmonary manifestations of SSc, including interstitial lung disease (ILD) and pulmonary hypertension (PH), are the leading cause of death. As new therapies targeting these pulmonary conditions emerge, early recognition of lung involvement is essential for the care of these patients. In this article we review the direct and indirect pulmonary manifestations of SSc and recent therapeutic trials that have attempted to target these manifestations. When a patient with SSc disease presents with signs or symptoms referring to the chest, a number of potential disorders must be considered (table 1) for: direct pulmonary involvement (ILD with or without PH or pulmonary arterial hypertension (PAH), airways disease and pleural involvement); indirect pulmonary complications (aspiration, infection, drug toxicity, malignancy, respiratory muscle weakness, restrictive lung disease from chest wall involvement and lung disease secondary to cardiac involvement); combinations of direct and indirect pulmonary manifestations; and other lung diseases not related to SSc (chronic obstructive pulmonary disease/emphysema, asthma and lung nodules). View this table: Table 1. Pulmonary involvement in systemic sclerosis In scleroderma, the two most common types of direct pulmonary involvement are ILD and PH, which together account for 60% of SSc-related deaths [2]. While certain pulmonary manifestations may occur more commonly in a subset of SSc ( i.e. ILD is more common in dcSSc while PH is more common in lcSSc) [3], all of the known pulmonary manifestations reported have been described in each of …