Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management

Abstract

Interstitial lung disease (ILD) is a group of lung diseases characterized by thickening of the interstitium surrounding pulmonary alveolar walls. It is related to specific radiographic features in lung imaging and/or the presence of restrictive disorders in pulmonary function tests (PFTs). ILD is one of the leading causes of death in systemic sclerosis patients. Major risk factors of ILD associated with SSc (SSc-ILD) include male sex, diffuse type of cutaneous SSc and presence of anti-Scl-70 antibodies. SSc-ILD is challenging to diagnose at an early stage as the symptoms are non-specific. The greatest risk of its development is during the 4-5 years after the initial diagnosis of systemic sclerosis. Clinical vigilance at the time, including regular pulmonary function tests and/or high-resolution com-puted tomography (HRCT), is needed. The aim of this paper is to summarize the current knowledge on early diagnostic methods and progression risk factors for SSc-ILD.

Keywords: early diagnosis; interstitial lung disease; progression risk factors; systemic sclerosis.

Fig. 1

Management of SSc-ILD depending on the extent of lung changes on HRCT and FVC values [31, 32, 36, 37].