Background Pulmonary involvement is common in primary Sjögren’s syndrome (pSS), which seriously affects the prognosis and survival rate. However, the treatment of those patients has hardly been explored before. Most studies were specifically designed to evaluate sicca features.
Objectives To analyze efficacy and safety of corticosteroid therapy combined with hydroxychloroquine (HCQ) or intravenous cyclophosphamide (CTX) in pSS-associated lung disease.
Methods A total of 15 patients were recruited in the prospective study. Among them, 7 patients took oral prednisone combined with HCQ and 8 patients received oral prednisone combined with intravenous CTX. Prednisone was prescribed initially at a dosage of 30-40mg/day, and was tapered to 7.5mg/d within 4 months. This dosage of prednisone was maintained or tapered for the rest of the follow up as appropriate. HCQ was administered at a dosage of 200mg, twice a day. CTX was administered intravenously at an initial dosage of 400mg, every 2 weeks for 6 months, and then tapered to 400mg every 4 weeks.
Results Fourteen patients receiving oral prednisone combined with HCQ or intravenous CTX were followed up for 11.57±4.91 months. One patient from CTX group withdrew from the study for stopping DMARDs to have surgery. Six of 7 patients (85.71%) were female of both groups. The mean age was 55.57±14.52 and 55.43±14.33 years old for HCQ group and CTX group, with median disease duration 120 and 180months, respectively. During the follow up, there was evidence of improvement in HRCT for most of the patients, and no obvious deterioration of PFT was observed. One patient in HCQ group presented hypoleukemia, which was ameliorated after reducing to 100mg, twice a day. Two patients had pneumonia and one had herpes zoster virus (HZV) infection in CTX group. No life-threatening side effect was observed in both groups.
Conclusions Corticosteroid therapy combined with hydroxychloroquine or intravenous cyclophosphamide is administered with a favorable response seen in the majority of patients. This should be further confirmed in a large cohort.
References Ramos-Casals M, Tzioufas AG, Stone JH, Siso A, Bosch X. Treatment of primary Sjogren syndrome: a systematic review. JAMA. 2010; 304: 452-60.
Shi JH, Liu HR, Xu WB, et al. Pulmonary Manifestations of Sjogren’s Syndrome. Respiration. 2009; 78: 377-86.
Disclosure of Interest None Declared