Pulmonary fibrosis in systemic sclerosis: Diagnosis & management

Abstract

Systemic sclerosis is a fascinating, although uncommon condition. However, fibrosing alveolitis in systemic sclerosis patients is common and is associated with significant morbidity and mortality. Investigations assist, not only with diagnosis, but also with assessment of disease severity and assessment of the likelihood of disease progression, thus helping to identify those requiring closer medical supervision. Treatment of fibrosing alveolitis in systemic sclerosis is complex, as the risks of some therapies may outweigh the potential benefits, and for those with advanced disease there is a lack of donor organs available for transplantation. Great strides continue to be made in unravelling the mysteries of the immunopathogenesis of fibrosing alveolitis in systemic sclerosis, and this should eventually lead to the development of more rational and targeted pharmacological therapies.