Does the presence of Systemic Sclerosis-ILD require treatment?
Not necessarily. ILD requires treatment only when it is “clinically significant” or progressive in nature. The ILD evaluation includes assessment of breathlessness, determining extent of disease by thoracic HRCT, complete PFT (lung volumes, spirometry, and DLco) and assessment for oxygen desaturation with exercise (6-minute walk test). Research has shown that greater extent of involvement on HRCT (>20%–50% of lung affected) is associated with an increased risk of progressive ILD (≥10% drop in forced vital capacity [FVC]) as well as a more robust response to therapy with cyclophosphamide.