CAJ | 학술논문

目的总结免疫介导性坏死性肌肉病(IMNM)患者的临床、电生理和骨骼肌病理改变特点.方法回顾性分析北京大学第三医院2011年1月—2014年1月的4例IMNM患者的临床、电生理特点及肌肉、皮肤活检的病理特点和治疗、随访情况.结果 4例患者中男女各2例,发病年龄37～58岁(平均47.5岁),病程1～60个月(平均17.8个月),急性或亚急性起病者2例,慢性起病者2例.4例患者均出现四肢近端力弱,3例颈屈肌受累,1例呼吸肌受累起病且需要呼吸机辅助呼吸,1例合并间质性肺病.4例患者信号识别颗粒(SRP)抗体强阳性;1例出现成组分布的变性坏死和再生肌纤维,3例仅出现少量散在分布的变性坏死和再生肌纤维;4例患者均可见肌纤维肥大、萎缩,伴随结缔组织轻～中度增生;部分病例可见少量炎性细胞浸润.1例患者糖皮质激素治疗及3例患者糖皮质激素联用免疫抑制剂治疗均有效.结论 IMNM临床相呈异质性,发病速度快慢不一,病情轻重不一,受累肌群分布不一;但骨骼肌病理改变相似,主要表现为:肌纤维变性、坏死、再生,无炎性细胞或很少炎性细胞浸润;糖皮质激素或联用免疫抑制剂有效.
목적 총결면역개도성배사성기육병(IMNM)환자적림상、전생리화골격기병리개변특점.방법 회고성분석북경대학제삼의원2011년1월—2014년1월적4례IMNM환자적림상、전생리특점급기육、피부활검적병리특점화치료、수방정황.결과 4례환자중남녀각2례,발병년령37～58세(평균47.5세),병정1～60개월(평균17.8개월),급성혹아급성기병자2례,만성기병자2례.4례환자균출현사지근단력약,3례경굴기수루,1례호흡기수루기병차수요호흡궤보조호흡,1례합병간질성폐병.4례환자신호식별과립(SRP)항체강양성;1례출현성조분포적변성배사화재생기섬유,3례부출현소량산재분포적변성배사화재생기섬유;4례환자균가견기섬유비대、위축,반수결체조직경～중도증생;부분병례가견소량염성세포침윤.1례환자당피질격소치료급3례환자당피질격소련용면역억제제치료균유효.결론 IMNM림상상정이질성,발병속도쾌만불일,병정경중불일,수루기군분포불일;단골격기병리개변상사,주요표현위:기섬유변성、배사、재생,무염성세포혹흔소염성세포침윤;당피질격소혹련용면역억제제유효.
Objective To characterize the clinical,electrophysiology and neuropathological features of 4 cases with immune-mediated necrotizing myopathy (IMNM).Methods We retrospectively analyzed the clinical,electrophysiology,neuropathological characteristics of 4 IMNM patients with muscular and skin biopsy in our department during 4 years (from January 2011 to January 2014).Results Among these 4 patients,2 were men and 2 were women (aged 37 to 58 years) with disease duration ranging from 1 month to 60 months.Two patients were with acute onset and 2 with chronic onset.All 4 patients had proximal muscle weakness with three patients with cervical flexor muscle weakness and one with respiratory muscles weakness and noninvasive ventilator assisted respiration.One patient had interstitial lung disease.The anti-signal recognition particle antibodies were strong positive in all 4 patients.Muscle biopsy showed group necrotizing and regenerating fibers in one patient and few scattered necrotizing and regenerating fibers in the other 3 patients.Both muscle fiber hypertrophy and muscle fiber atrophy together with proliferation of connective tissue on endomysium could be viewed in all 4 patients.However,very few inflammatory cells were detectable in patients.One patient was treated with corticosteroids and the other three were treated with combination of corticosteroids and immunosuppressant drugs.Conclusions IMNM is characterized by heterogeneity at disease onset,severity and ilnvolvement of muscles with,however,similary pathological changes including the presence of numerous necrotic and regenerating fibers with little or none inflammation.Corticosteroid and/or immunosuppressant is effective for patients.