Scleroderma lung disease: evolving understanding in light of newer studies

To review therapeutic goals in pulmonary fibrosis in systemic sclerosis in the light of pathogenetic thinking and therapeutic data, with particular attention to recent data questioning the importance of the identification of alveolitis.

Immunological/inflammatory activation remains the primary therapeutic target, based on recent data. Other effective therapies have not been developed, despite investigation of many pathogenetic pathways. In most cases, lung disease is predominantly fibrotic and prevention of progression is the only practicable therapeutic goal. Indications for introducing treatment remain uncertain. A granulocytosis on bronchoalveolar lavage and ground-glass attenuation on computed tomography, previously thought to denote an inflammatory histological picture ('alveolitis'), are usually indicative of fibrotic disease. By contrast, a recent staging system, integrating computed tomography and pulmonary function data, might, with refinement, identify patients likely to benefit from treatment.

Treatment benefits consist of the prevention of progression and are largely confined to patients with extensive pulmonary fibrosis. Historical algorithms for the identification of alveolitis, using computed tomography and bronchoalveolar lavage, are inaccurate and do not identify patients most likely to benefit from treatment. Accurate prognostic evaluation by staging the severity of lung disease remains central to management and will be a major focus of future studies.