Recent Treatments of Interstitial Lung Disease with Systemic Sclerosis.

Two pilot open-label studies showed a trend toward the improvement of skin thickening and FVC (400 mg/day) 122 (up to 600 mg/day), 123 but no efficacy was found in a randomized, double-blind, placebo-controlled trial (400 mg/day). 124 Patients with advanced ILD were excluded from these trials, but it should be noted that many patients dropped out because of adverse events, including gastrointestinal symptoms and peripheral edema, and one trial was terminated because of safety concerns.

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With increased awareness of the morbidity and mortality associated with SSc-ILD, there is an increasing trend toward performing HRCT and PFT even in patients with asymptomatic SSc, since some patients with moderate-to-severe loss of lung function or advanced fibrosis do not show dyspnea.

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