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Interstitial lung fibrosis significantly contributes to morbidity and mortality in systemic sclerosis (SSc),1 but only a few established treatment options exist.2 Cyclophosphamide (CYC)3 is one of the effective options for interstitial lung disease and skin thickening.4 ,5 However, not all patients respond to this medication and based on the clinical routine practice in one centre, there has been no analysis using reduced intravenous cyclophosphamide (800–1200 mg monthly). 6,–,8 Therefore, we evaluated the response to intravenous cyclophosphamide treatment in 39 patients with SSc in our department. Responders were defined by any of the three outcome parameters: the modified Rodnan skin score (mRSS; responders: decrease by ≥5/51 points), forced vital capacity (FVC; responders: increase or no decline >10%) or diffusing lung capacity for carbon monoxide (DLCO; responders: increase or no decline >10%). Lung function test (LFT) denominated responders that fulfilled …
Competing interests None.
Provenance and peer review Not commissioned; externally peer reviewed.
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