CAJ | 학술논문

目的总结、分析无肌病皮肌炎(ADM)的临床特点.方法回顾性分析2006年1月-2010年1月解放军总医院风湿科住院收治的26例ADM患者的临床表现、实验室指标、影像学表现及治疗与转归.结果 26例患者中女性18例,男性8例,发病年龄30～ 68岁,病程2～18个月.26例患者均有Gottron丘疹,21例有眼眶周围紫红色斑.26例患者胸部高分辨率CT有肺间质纤维化表现,其中15例合并肺部感染.3例患者合并肿瘤.26例患者肌酶均在正常范围；9例抗核抗体阳性,4例Jo-1抗体阳性.5例患者有轻微肌源性损害；19例肌活检异常.糖皮质激素及免疫抑制剂治疗后20例患者病情好转,6例死亡,其中2例因并发肿瘤死亡,4例因重度感染死亡(1例卡氏肺孢子菌感染,1例曲霉菌感染,2例纵隔气肿合并感染).结论ADM约占皮肌炎的20％左右,其并发肺间质疾病的程度较其他皮肌炎重,进展迅速,且并发肿瘤的机会较高,应尽早采用糖皮质激素和免疫抑制剂治疗,改善患者的预后.
목적 총결、분석무기병피기염(ADM)적림상특점.방법 회고성분석2006년1월-2010년1월해방군총의원풍습과주원수치적26례ADM환자적림상표현、실험실지표、영상학표현급치료여전귀.결과 26례환자중녀성18례,남성8례,발병년령30～ 68세,병정2～18개월.26례환자균유Gottron구진,21례유안광주위자홍색반.26례환자흉부고분변솔CT유폐간질섬유화표현,기중15례합병폐부감염.3례환자합병종류.26례환자기매균재정상범위；9례항핵항체양성,4례Jo-1항체양성.5례환자유경미기원성손해；19례기활검이상.당피질격소급면역억제제치료후20례환자병정호전,6례사망,기중2례인병발종류사망,4례인중도감염사망(1례잡씨폐포자균감염,1례곡매균감염,2례종격기종합병감염).결론ADM약점피기염적20％좌우,기병발폐간질질병적정도교기타피기염중,진전신속,차병발종류적궤회교고,응진조채용당피질격소화면역억제제치료,개선환자적예후.
Objective To analyze the clinical characteristics of amyopathic dermatomyositis (ADM).Methods Twenty six patients diagnosed as ADM from January 2006 to January 2010 in PLA General Hospital were retrospectively analyzed.The clinical manifestation,laboratory findings,imaging manifestations,treatment and prognosis of the 26 patients were recorded.Results There were 18 females and 8 males with age of 30-68 years.Overall disease course after diagnosis was 2-18 months.All patients had Gottron rash and interstitial pneumonia.Fifteen patients had history of pulmonary infections.Three patients had comorbidity of tumor.Creatine phosphokinase,creatine phosphokinase isoenzyme,glutamicoxaloacetic transaminase and lactate dehydrogenase were normal in all 26 patients.Four patients had positive anti-Jo-1 antibodies.Antinuclear antibodies were positive in nine patients.Electromyogram was slightly abnormal in 5 patients.Muscle biopsy was abnormal in 19 patients.Twenty patients had improved after receiving corticosteroids and immunosuppressive agents.Six patients died.Conclusions It has been estimated that ADM represents approximately 20％ of all cases of dermatomyositis.It seems that patients with ADM have greater incidence of lung involvement and combined cancer.ADM patients need to be treated positively to improve the prognosis.