Fibrosing alveolitis in systemic sclerosis: the need for early screening and treatment

Abnormalities in lung function occur in 70% of patients with systemic sclerosis (SSc). Fibrosing alveolitis in SSc (FASSc) is more commonly seen in the diffuse cutaneous form of SSc, particularly in the presence of antitopoisomerase antibodies (Scl70), and with the decreasing incidence of scleroderma renal crisis it is now the major cause of mortality in this patient population. Screening of patients recently diagnosed with SSc by pulmonary function tests and the performance of high resolution computed tomography when physiological abnormalities are identified has resulted in the identification of significant numbers of patients with early, asymptomatic FASSc. Whether these patients should be further investigated with a surgical lung biopsy or receive immunosuppression is unclear, because it cannot yet be reliably predicted who will develop progressive disease and the evidence to support the efficacy of treatment is not strong. The objective of the present article was to review the evidence to support the use of immunosuppressive therapy in FASSc and, based on these data, to propose an algorithm for the investigation and management of this difficult clinical problem.