J Rheumatol: rituximaandaandiphosphamide therapy antisynthetic syndrome associated with ILD

Anti-synthetic enzyme syndrome (ASyS) correlation interstitial pulmonary disease (ILD) has a poor prognosisIntravenous cyclophosphamide (CYC) and rituxima (RTX) are the main treatments for moderate to severe ILDThe study compared CYC and then conducted standard immunosuppressive therapy (IST) vsThe efficacy of RTx on ASyS correlation ILDconducted this observational retrospective study at three tertiary treatment centers from 2003 to 2016Included in all ASyS-associated ILD patients who received CYC or RTX treatment and were followed up for at least 6 monthsAssess lung progression survival (PFS) as defined in the American Thoracic Society guidelines at 6 months and 2 yearsRecord all serious adverse eventsincluded 62 patientsThirty-four patients received 2-12 monthly CYC venous shock treatments, and 30 (88%) were subsequently treated with standard ISTThe RTX group included 28 patients who, after infusion on day 1 to 15, had 26 patients (93%) repeated RTX treatment every six months and 15 patients (54%) receiving IST treatment at the same timeThe median dose of steroids in both groups was similarAlthough the 6-month PFS of RTX and CYC are similar (92%vs.81%, respectively), the PFS of RTX is better in 2 years (risk ratio of 0,263 (0,094-0,732) p.011)Interestingly, the lower baseline DLCO can independently predict 2 years of poor PFS (0.965 (0.936-0.995), p.023)Both groups of FVC and DLCO improved, and the difference was not significantThe two groups of severe adverse events are similar in Patients with ASyS-associated ILD, although PFS was similar at 6 months, RTX had better 2-year PFS than CYC