The clinical and muscular pathological study of dermatomyositis with perifascicular atrophy changes
10.3760/cma.j.issn.0578-1426.2012.09.012
- VernacularTitle:以束周萎缩为肌肉病理特点的皮肌炎患者的临床病理分析
- Author:
Zhi LIU
;
Lin CHEN
;
Yupu GUO
;
Haitao REN
;
Yanhuan ZHAO
- Publication Type:Journal Article
- Keywords:
Dermatomyositis;
Perifascicular atrophy;
Complement membrane attack complex;
Lung diseases,interstitial
- From:
Chinese Journal of Internal Medicine
2012;51(9):698-701
- CountryChina
- Language:Chinese
-
Abstract:
ObjectiveTo investigate the clinical and pathological characteristics of dermatomyositis with muscular perifascicular atrophy (PFA).MethodsA series of 104 consecutive patients clinically and pathologically diagnosed as dermatomyositis by muscle biopsy in our laboratory from December,2003 to August,2011,were enrolled in this study. Muscle biopsy of all the enrolled patients had shown PFA of muscle fibers.ResultsAmong the 104 patients,34 were males and 70 were females with a mean age of 45 years old.Among them,8 cases had normal electromyogram;42 had normal serum creatine kinase level;11 were diagnosed as carcinoma;75 were found to be combined with interstitial lung disease (ILD).Based on morphologic changes of muscle biopsy,they were divided into pure PFA group with 54 cases and PFA plus focal damage group with 50 cases.Compared with the pure PFA group,there was prominent mononuclear cell infiltration into perimysial intermediate sized vessels and membrane attack complement (MAC) deposition in the intramuscular capillaries in the PFA plus group.Skin biopsy had been taken in 12 cases together with muscle biopsy and had shown the border effectof both PFA and interface dermatitis in muscle and skin.ConclusionsOur study suggests that chronic immune vascular damage and insufficiency in dermatomyositis may cause ischemia and focal myofiber damage in watershed regions. The incidence of ILD in our dermatomyositis patients with PFA is high.
