Shah P A, Hassan I, Choudhary N D, Nafae A
Department of Dermatology, Government Medical College, Srinagar, Kashmir.
J Assoc Physicians India. 1999 May;47(5):480-2.
The present study is a retrospective and prospective study of 24 cases of idiopathic inflammatory myopathies from Kashmir valley. The study was conducted to depict the clinical profile of polymyositis from this part of the world.
The diagnosis of polymyositis/dermatomyositis was established by following the criteria of Bohan and Peter (1975). Besides relevant clinical examination, investigations like complete blood count, ESR, muscle enzymes, LE cell phenomenon, antinuclear antibodies and rheumatoid factor were also done. Electrophysiological study and open muscle biopsy was performed in 21 and 24 cases respectively.
The mean age at presentation was 34 years with 62.5% cases presenting in fourth decade. Pelvic girdle weakness was observed in all the 24 cases. Male:Female ratio was 1:1.4. Four (16.8%) cases had associated collagen vascular disease. No case was associated with malignancy or childhood vasculitis. Raynauds' phenomenon was seen in seven (29.1%) cases. Creatine phosphokinase and lactic dehydrogenase was elevated in 22 (91.6%) and 12 (50%) cases respectively. Electromyography revealed myopathic features in 74.3% cases. Muscle biopsy revealed features of inflammatory myopathy in 22 (91%) cases.
The conclusions drawn from this study are as follows: a. Younger age at presentation b. Shorter duration of illness at presentation c. Increased frequency of Raynaud's phenomenon as compared to other Indian series. d. No case of polymyositis/dermatomyositis associated with malignancy or childhood vasculitis was seen. e. Biochemical, electrophysiological and histopathological features suggestive of shorter duration of illness were observed.
本研究是一项对克什米尔山谷地区24例特发性炎性肌病的回顾性和前瞻性研究。该研究旨在描述世界这一地区多发性肌炎的临床特征。
根据Bohan和Peter(1975年)的标准确立多发性肌炎/皮肌炎的诊断。除了进行相关临床检查外,还进行了全血细胞计数、血沉、肌肉酶、狼疮细胞现象、抗核抗体和类风湿因子等检查。分别对21例和24例患者进行了电生理研究和开放性肌肉活检。
就诊时的平均年龄为34岁,62.5%的病例在第四个十年出现。24例患者均观察到骨盆带肌无力。男女比例为1:1.4。4例(16.8%)患者合并有胶原血管病。无病例与恶性肿瘤或儿童血管炎相关。7例(29.1%)患者出现雷诺现象。肌酸磷酸激酶和乳酸脱氢酶分别在22例(91.6%)和12例(50%)患者中升高。肌电图显示74.3%的病例有肌病特征。肌肉活检显示22例(91%)有炎性肌病特征。
本研究得出的结论如下:a.就诊时年龄较轻;b.就诊时病程较短;c.与其他印度系列相比,雷诺现象的发生率增加;d.未发现与恶性肿瘤或儿童血管炎相关的多发性肌炎/皮肌炎病例;e.观察到生化、电生理和组织病理学特征提示病程较短。