Diagnosis and treatment of scleroderma lung disease---how are we doing?

Now that scleroderma renal crisis has declined as the cause of death in patients with systemic sclerosis, pulmonary complications, namely ILD and PAH, are increasingly important and have emerged as the leading causes of death. This study shows the wide practice variation in the evaluation and treatment of pulmonary manifestations. This may be due to varying perspectives among different specialties, lack of familiarity with pulmonary aspects of the disease or a lack of guidelines and evidence.