临床无肌病皮肌炎29例临床分析

Clinical analysis of 29 patients with clinically amyopathic dermatomyositis

目的了解中国临床无肌病皮肌炎(C-ADM)病人的临床特点及预后。方法对29例C-ADM 病人的皮肤、肌肉损害以及肺部病变等临床资料进行总结。29例病人中男6例,女23例,平均起病年龄(44±8)岁。对5例病人进行了皮肤活检,全部病人接受了肺 CT 检查。结果所有病人均出现皮肌炎特征性的皮肤改变,其中 Gottron 疹27例(91.3%),向阳疹22例(75.9%),颈部 V 区疹11例(37.9%),披肩疹7例(24.1%);甲周红斑和毛细血管扩张6例(20.7%),其中4例病人皮损有破溃。Gottron 疹最常见于肘关节(86.2%),其次为近端指间关节(58.6%)、掌指关节(48.3%)、膝关节(23.8%)、髋关节(20.7%)、肩关节(13.8%)和踝关节(9.5%)。5例皮肤活检病人的组织病理学改变符合皮肌炎的皮肤损害特点。19例病人合并肺间质病变(65.5%),其中5例病人在随访中死亡,主要死亡原因为合并肺间质病变进展至呼吸衰竭。结论 C-ADM 多发生于中年女性,典型皮疹是诊断的关键。肺间质病变是其最常见和最严重的并发症,临床上需高度警惕,一旦发现应积极治疗。

Objective To explore the clinical features and prognosis of clinically amyopathic dermatomyositis （C-ADM）. Methods The clinical data, including skin lesion, muscle involvement, and lung disease, of 29 patients with C-ADM, 6 males and 23 females with a male/female ratio of 1 ： 3.83, aged 44 ± 8, were analyzed. Skin biopsy was taken in 5 patients and lung HRCT was done in all the patients. Results The mean age at onset was （44±8）years. All patients presented with characteristic skin lesions such as Gottron＇ s papulae （ 91.3% ） ; heliotropic periorbital erythrema （ 75.9% ） ; Ⅴ-sign rash （37.9%） ; shawl-sign rash （24. 1% ） ; and periungual erythema and telangiectasias （20. 7% ）. Gottron＇s papule was most commonly seen on the dorsal aspect of elbow （86. 2% ） , proximal interphalangeal joints （58.6%）, metacarpophalangeal joints （ 48. 3% ） , patellae （ 23.8% ） , hip （ 20. 7% ） , shoulder （ 13. 8% ） , and ankle （9. 5% ）. The cutaneous histopathologic pictures of these patients were all compatible with the skin lesions of dermatomyositis. Interstitial lung disease （ILD） was found by lung HRCT in 19 patients （65.5%）. All patients received steroids combined with immunosuppressants, but rapidly progressive ILD happened to some of the patients and finally led to death due to respiratory failure in 5 of them. Condusion C-ADM is most commonly seen in the middle-aged women. ILD is the commonest respiratory problem arising in C-ADM patients and can be fatal, therefore should be properly treated.