胶原血管病伴肌炎69例临床和病理研究

目的:研究胶原血管病伴肌炎(CTM)的临床、病理特征。方法:回顾性总结69例CTM患者的临床资料,研究其临床表现、血清免疫学、肌酶学、肌电图、肌肉病理的特点。结果:本病临床上主要有肌无力、肌痛、关节痛、发热、肌萎缩、雷诺征等;血清免疫学可见免疫球蛋白增高和补体降低,部分患者ANA、ENA阳性;CK等血清肌酶增高不及单纯性多发性肌炎(PM)明显;肌电图呈肌原性损害,也可有神经原性损害。肌肉病理显示免疫炎性改变,表现为区域性肌纤维变性坏死,肌间质血管狭窄、闭塞、炎症细胞浸润,血管炎和肌束周萎缩远较PM常见(P<0.01)。结论:CTM的临床和肌肉病理与PM有所不同,广泛的血管炎性病变是CTM特征性的病理改变。

The study of clinic and pathology on 69 patients with connective tissue disease associated myositis

Objective: TO study the clinical and pathological manifestation of connective tissue disease associated myositis(CTM). Methods: The clinical situation, serological immunology, serum emzymes, electromyography (EMG) and muscular pathology were investigated Routine histological and histochemical methods were used in muscular pathology. Results: The clinical features of CTM included muscle weakless, myalgia, arthralgia, muscle atrophy, fever, Raynaud's phenomenon, et al. The elevation of serum emzymes, such as creatine kinase, were not so obviously high as PM. Some of the CTM patients had abnormal EMG of myogenic damage, others being neurogenic damage, and the latter could be seen more frequently than in PM (P<0. 01) The muscle biopsy showed immune inflammation. Degeneration, necrosis of the regional muscle fibre, constriction, emphraxis of the interstitial blood vessel, and perimysial inflammatory infiltrate were the main pathological manifestation of CTM, Vasculitis and perifascicular atrophy were more frequntly to see in CTM than in PM (P<0. 01). Cunclusion: The manifestation of clinic and muscular pathology in CTM is different from in PM. Very popular vasculitis is the characteristic change of pathology in CTM.