CAJ | 학술논문

目的研究肌肉活体组织检查(简称活检)出现束周萎缩的青少年肌炎患者的临床及肌肉病理特点.方法对21例肌肉活检病理以束周萎缩为病理特征的青少年肌炎患者的临床资料和病理改变进行回顾性分析.结果临床表现:21例中11例确诊为青少年皮肌炎(典型皮疹),9例为可能的青少年皮肌炎(不典型皮疹),1例为混合结缔组织病.21例中19例有肌肉无力表现.9例肌酸激酶正常,4例轻度升高(3倍以下),8例明显升高(3倍以上),其中11例确诊的皮肌炎患者中10例肌酶正常或轻微升高.6例患者合并肺间质病变,11例抗核抗体阳性.2例合并EB病毒感染,1例巨细胞病毒感染.肌肉病理:21例均可见束周萎缩,其中4例束周萎缩形态学不典型,但相应束周肌纤维还原型辅酶、乳酸脱氢酶、细胞色素C氧化酶染色异常;12例免疫组织化学染色中9例显示膜补体攻击复合物染色阳性.比较分析发现肌酸激酶升高程度与肌纤维坏死相关,与束周萎缩无明显相关;膜补体攻击复合物沉积的标本中束周萎缩、局灶损伤及线粒体异常的病理改变更突出.结论肌肉萎缩呈束周分布现象主要见于典型或不典型皮肌炎,可能与免疫复合物沉积和小血管异常有关.
목적 연구기육활체조직검사(간칭활검)출현속주위축적청소년기염환자적림상급기육병리특점.방법 대21례기육활검병리이속주위축위병리특정적청소년기염환자적림상자료화병리개변진행회고성분석.결과 림상표현:21례중11례학진위청소년피기염(전형피진),9례위가능적청소년피기염(불전형피진),1례위혼합결체조직병.21례중19례유기육무력표현.9례기산격매정상,4례경도승고(3배이하),8례명현승고(3배이상),기중11례학진적피기염환자중10례기매정상혹경미승고.6례환자합병폐간질병변,11례항핵항체양성.2례합병EB병독감염,1례거세포병독감염.기육병리:21례균가견속주위축,기중4례속주위축형태학불전형,단상응속주기섬유환원형보매、유산탈경매、세포색소C양화매염색이상;12례면역조직화학염색중9례현시막보체공격복합물염색양성.비교분석발현기산격매승고정도여기섬유배사상관,여속주위축무명현상관;막보체공격복합물침적적표본중속주위축、국조손상급선립체이상적병리개변경돌출.결론 기육위축정속주분포현상주요견우전형혹불전형피기염,가능여면역복합물침적화소혈관이상유관.
Objective To describe the clinical and muscle pathological characters of juvenile myositis patients with perifascicular atrophy (PFA).Methods A series of 21 consecutive muscle biopsies with clinically and pathologically confirmed juvenile myositis were studied.All biopsies had PFA of muscle fibers.Results Clinical manifestation:11/21 had typical dermatomyositis rash,9/21 possible dermatomyositis because of atypical rash,1/21 mixed connective tissue disease;9/21 had weakness complaint;9/21with normal creatine kinase (CK) level,4/21 low grade rise,8/21 apparently elevated,10/ 11 definite dermatomyositis patients with normal or low grade level;6/21 combined with interstitial lung disease,2 with EB virus infection,1 with cytomegalo virus infection.Muscle pathology:4 specimens with atypical PFA also had enzyme abnormality in nicotinamide adenine dinucleotide,succinate dehydrogenase and cytochrome c oxidase staining,9/12 cases had membrane attack complement deposition intramuscular capillaries,and they had prominent regional mitochondrial abnormalities,protrude PFA and focal muscle damage.Skin biopsy of one case showed perivasculitis in dermis.Conclusions PFA could be seen in juvenile dermatomyositis and mixed connective tissue disease,and PFA could not increase the CK level.The capillary and intermediate-sized vessels damage may cause chronic ischemia,which could explain the PFA with mitochondrial abnormalities.