Assessment of Interstitial Lung Disease in Systemic Sclerosis and Impact on Disease Progression and Outcomes

Review Article


E Koumakis , A Kahan  and Y Allanore

Affiliation: Paris Descartes University, Rheumatology A Department, Cochin Hospital, APHP, 75014 Paris, France


ABSTRACT

Interstitial lung disease (ILD) has emerged as a major cause of mortality in systemic sclerosis (SSc), representing about 30% of SSc‐related deaths. ILD occurs in up to 75% of patients with SSc, but progresses to severe restrictive lung disease in only about 10–15% of these patients. Histopathologic lesions are characterized in most cases by non‐specific interstitial pneumonia. Risk factors for the occurrence of pulmonary fibrosis include diffuse cutaneous SSc and anti‐topoisomerase I antibodies. ILD often remains asymptomatic, and the presence of symptoms often indicates advanced lung disease. Therefore, early detection of ILD is a major concern in SSc patients. The diagnosis and follow‐up of SSc‐related ILD (SSc‐ILD) is based essentially upon non‐invasive examinations including pulmonary function tests and high‐resolution pulmonary computed tomography scan (HRCT). The utility of outcome measures for pulmonary involvement in SSc has been assessed by the OMERACT filter, according to several criteria (truth, discrimination, and feasibility). Risk factors for the deterioration of lung function include low baseline forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO) values, and initial HRCT abnormalities. This article details and summarizes the available data on prognostic factors for SSc‐ILD and the association of lung involvement with disease progression in SSc.

Keywords: systemic sclerosis, interstitial lung disease, pulmonary function tests, computer tomography (CT) scan
Correspondence: Yannick Allanore, Service de Rhumatologie A, Hôpital Cochin, Université Paris Descartes, 27 rue du Faubourg Saint‐Jacques, 75014 Paris, France. Tel: (33)‐1‐58‐41‐25‐72; Fax: (33)‐1‐58‐41‐26‐24; e‐mail: yannick.allanore@cch.aphp.fr