以间质性肺疾病起病的皮肌炎临床特点分析

Interstitial lung disease as an initial manifestation of dermatomyositis

目的 探讨以间质性肺疾病（ILD）起病的皮肌炎患者的临床特点和预后.方法 北京协和医院1999年1月至2013年1月皮肌炎并ILD住院患者资料完整者共184例,回顾性分析其临床表现、生化指标、抗体谱、影像学、肺功能、肺活体组织检查学、治疗及转归等,并对ILD起病组及非ILD起病组皮肌炎患者进行上述指标的比较.结果 皮肌炎住院患者并发ILD的发生率为17％,平均年龄（48±12）岁,男∶女=63∶121.ILD起病组88例（47.8％）,包括ILD与皮肌炎同时发生（1个月内）42例（22.8％）,以及ILD先于皮肌炎发生46例（25.0％）,后者ILD平均先于皮肌炎（11±3）个月发生.ILD起病组活动后气短、咳嗽和肺部爆裂音的发生率高于非ILD起病组,而向阳疹、前胸Ⅴ区皮疹、披肩征和关节受累的发生率较后者低（P＜0.05）.ILD起病组与非ILD起病组相比,肌酶水平、抗核抗体和抗SSA阳性率更低（P＜0.05）,抗Jo-1抗体阳性率（13.6％）差异无统计学意义（P＞0.05）.ILD起病的皮肌炎主要表现为弥散功能及限制性通气功能障碍,一氧化碳弥散率显著低于非ILD起病组（P＜0.01）.ILD起病的皮肌炎临床病理类型主要是机化性肺炎和非特异性间质性肺炎.ILD起病组病死率19.3％,两组差异无统计学意义（P＞0.05）.死亡原因主要是ILD进展所致呼吸衰竭（13例,76.5％）.结论 以ILD起病的皮肌炎临床常见,是皮肌炎住院患者主要死亡原因.其主要临床病理类型是机化性肺炎和非特异性间质性肺炎.

Objective To explore the clinical features and prognosis of dermatomyositis patients with interstitial lung disease （ILD） as an initial manifestation.Methods Medical records of 184 dermatomyositis inpatients complicated with ILD,admitted into Peking Union Medical College Hospital from January 1999 to January 2013,were retrospectively analyzed.The clinical features,biochemical parameters,positive rates of autoantibodies,radiology,pulmonary function tests,pathology,treatments and prognosis were compared between two subgroups of ILD-initial and non-ILD-initial dermatomyositis.Results The incidence of ILD of dermatomyositis inpatients was 17％.The average age was 48 ± 12 years and the gender ratio of male-to-female was 63∶ 121.Eighty eight （47.8％） dermatomyositis patients had ILD as an initial manifestation,including （n =42,22.8％） of ILD concomitant dermatomyositis （within 1 month） and （n =46,25.0％） of ILD before dermatomyositis with an average ahead time of （11 ± 3） months.Patients of ILD-initial dermatomyositis had a higher incidence of dyspnea on exertion,cough and lung crackles,but there were lower incidences of heliotrope rash,chest Ⅴ area rash,shawl sign and joint involvement than non-ILD-initial dermatomyositis （P ＜ 0.05）.The positive rate of anti-Jo-1 antibodies of ILD-initial dermatomyositis group was 13.6％.The main performances of ILD-initial dermatomyositis on pulmonary function tests were diffusing and restrictive ventilation impairment.And there was a lower diffusing rate of carbon monoxide than non-ILD-initial dermatomyositis group （P ＜ 0.01）.Organic and non-specific interstitial pneumonias were the major clinical pathology types of ILD-initial dermatomyositis.The mortality rate of ILD-initial dermatomyositis patients was 19.3％ and there was no significant difference from non-ILD-initial dermatomyositis （P ＞ 0.05）.The main course of ILD-initial dermatomyositis was respiratory failure due to progressive ILD （n =13,76.5％）.Conclusions ILD as an initial manifestation is a common complication and a major mortality cause of dermatomyositis inpatients.And the frequent clinical pathology types are organic and non-specific interstitial pneumonias.Symptoms of skin and muscle,creatine kinase and anti-synthetase antibodies should be closely monitored.