Management overview for interstitial lung disease in systemic sclerosis

Interstitial lung disease, or pulmonary fibrosis, is frequently associated with systemic sclerosis, but is more likely to be clinically significant in people with the diffuse variant. Interstitial lung disease, together with pulmonary arterial hypertension and cor pulmonale, are the most common causes of significant morbidity and mortality in systemic sclerosis. As there may be few early symptoms or signs of pulmonary involvement, regular monitoring is important. Annual pulmonary function tests are recommended to screen for early identification of interstitial lung disease.

People with interstitial lung disease associated with systemic sclerosis require specialist management. The optimal management is uncertain. Treatment may not be required for patients with indolent disease. The aim of management is to improve pulmonary function. Immunomodulatory drugs have only modest benefitsRoofeh, 2021Roofeh, 2019.