来氟米特联合间歇小剂量环磷酰胺治疗原发性干燥综合征合并间质性肺疾病八例

目的探讨来氟米特(LEF)联合间歇小剂量环磷酰胺(CTX)治疗原发性干燥综合征(pSS)合并间质性肺疾病(ILD)的有效性及安全性。方法给予8例pSS合并ILD患者LEF(10 mg/d)联合间歇小剂量CTX(200~400 mg/3周)治疗,随访1~2.5年,观察8例患者治疗前后的临床转归及实验室检查改变。结果8例患者口眼干燥及干咳、劳力性气促症状明显减轻,肺功能有所恢复,肺总量(TLC)、一氧化碳弥散度(DLco)均有不同程度的增加,肺高分辨率CT病变面积减少,但肺底吸气末期Velcro音变化不明显。无明显不良反应发生。结论LEF联合间歇小剂量CTX治疗pSS合并ILD是有效、安全、可行的。

Leflunomide and intermittent low-dose cyclophosphamide for treatment primary Sj(o)gren's syndrome complicated with interstitial lung disease:A follow-up of 8 cases

Objective To explore the efficacy and saiety ot lellunomlde （LEF） and intermittent low-close cyclophosphamide （CTX） for treatment of primary SjOgren＇s syndrome （pSS） complicated with interstitial lung disease OLD）. Methods Eight patients with pSS and ILD received LEF （10 mg/d） and intermittent low-dose CTX therapy （200～400 mg/3w） for 1 to 2.5 years. The clinical outcomes and changes in laboratory parameters were monitored. Resuits The symptoms of xerostomia, xerophthalmia, tussiculation, exertional dyspnea and pulmonary function significantly improved in all cases. The levels of TLC and DLco were both increased. The size of lung lesions was reduced as shown by HRCT. However, the Velcro sound during endinspiration at the base of lung did not change remarkably. No severe adverse effects occurred. Conclusion Combination therapy of leflunomide and intermittent low-dose cyclophosphamide appeared to be effective and safe.