临床无肌病皮肌炎29例临床分析

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。

Clinical analysis of 29 patients with clinically amyopathic dermatomyositis

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. CONCLUSION: 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.