ABSTRACT

Scleroderma has a prevalence of 242 cases per million population in the United States. One of the most commonly involved organs is the lung, where the most common manifestations are interstitial lung disease (ILD) and pulmonary arterial hypertension. ILD progresses to severe restriction in nearly 15% of patients and it is a leading cause of death with a median survival of 6.5 years following diagnosis. Patients at greatest risk for developing ILD are those with diffuse cutaneous scleroderma and those with anti-SCL-70 antibodies. In this review, we discuss the radiographic and pathologic manifestations of scleroderma-associated ILD, the utility of pulmonary function tests and bronchoalveolar lavage to screen for presence of disease and in the decision of who to treat. While not proven, treatment with either cyclophosphamide or mycophenolate mofetil may be effective.

KEYWORDS

Scleroderma, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), anti-SCL-70 antibodies, cyclophosphamide, mycophenolate mofetil.

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