CAJ | 학술논문

目的研究面肩肱型肌营养不良(FSHD)肌组织的血管炎性病理改变特点,探讨血管因素在FSHD发病机制中的作用.方法回顾性分析26例FSHD患者的临床及肌肉病理学资料,按有尤炎性细胞浸润分为非炎性浸润组和炎性浸润组,并据浸润的部位不同将后者分为肌内膜炎、血管周围炎及跨壁血管炎3个亚组.对炎性浸润组行抗CD3、抗CD4、抗CD8、抗CD20及抗血管平滑肌肌动蛋白免疫组织化学染色.收集20例炎症性肌病患者(多发性肌炎、皮肌炎各10例)肌活体组织检查组织作为对照组,行抗CD4、抗CD8、抗CD20免疫组织化学染色.结果 26例FSHD患者发病年龄为(25.2±12.6)岁,病程(7.8±7.3)年.男女之比为1.6∶1,其中5例患者有家族史.所有的患者均表现为面肌、肩胛带肌及上肢肌群的进行性无力和萎缩,18例患者骨盆带肌和(或)下肢远端受累,24例两侧不对称受累.主要病理学特点为:17例(65.4%)可见灶性炎性细胞浸润,其中4例炎细胞仅位于肌内膜,7例为血管周嗣炎,6例血管壁内可见炎细胞浸润,为跨壁的血管炎.免疫组织化学染色证实浸润的炎细胞主要为CD4+T淋巴细胞及CD+20 B淋巴细胞.炎性浸润组的肌组织内残存的肌纤维(48.0%±23.6%)较非炎性浸润者(94.3%±3.1%)明显减少(T=198.000,P=0.000),表明前者肌肉病理损害重于后者.对照组皮肌炎浸润的炎细胞类型与FSHD类似,而多发性肌炎以CD8+T淋巴细胞为主.结论 FSHD肌组织内可见显著的炎细胞浸润,表现为肌内膜炎、血管周围炎及跨壁血管炎.跨壁血管炎为血管壁受损,提示血管病理因素可能与本病的发病机制有关.
목적 연구면견굉형기영양불량(FSHD)기조직적혈관염성병리개변특점,탐토혈관인소재FSHD발병궤제중적작용.방법 회고성분석26례FSHD환자적림상급기육병이학자료,안유우염성세포침윤분위비염성침윤조화염성침윤조,병거침윤적부위불동장후자분위기내막염、혈관주위염급과벽혈관염3개아조.대염성침윤조행항CD3、항CD4、항CD8、항CD20급항혈관평활기기동단백면역조직화학염색.수집20례염증성기병환자(다발성기염、피기염각10례)기활체조직검사조직작위대조조,행항CD4、항CD8、항CD20면역조직화학염색.결과 26례FSHD환자발병년령위(25.2±12.6)세,병정(7.8±7.3)년.남녀지비위1.6∶1,기중5례환자유가족사.소유적환자균표현위면기、견갑대기급상지기군적진행성무력화위축,18례환자골분대기화(혹)하지원단수루,24례량측불대칭수루.주요병이학특점위:17례(65.4%)가견조성염성세포침윤,기중4례염세포부위우기내막,7례위혈관주사염,6례혈관벽내가견염세포침윤,위과벽적혈관염.면역조직화학염색증실침윤적염세포주요위CD4+T림파세포급CD+20 B림파세포.염성침윤조적기조직내잔존적기섬유(48.0%±23.6%)교비염성침윤자(94.3%±3.1%)명현감소(T=198.000,P=0.000),표명전자기육병리손해중우후자.대조조피기염침윤적염세포류형여FSHD유사,이다발성기염이CD8+T림파세포위주.결론 FSHD기조직내가견현저적염세포침윤,표현위기내막염、혈관주위염급과벽혈관염.과벽혈관염위혈관벽수손,제시혈관병리인소가능여본병적발병궤제유관.
Objective To investigate the pathological features of blood vessel inflammation in facioscapulohumeral muscular dystrophy ( FSHD ) and the role of vasculitis on the pathogenesis of FSHD. Methods The clinical manifestations and myopathological features of 26 FSHD patients were retrospectively analyzed and summarized. All of the patients were divided into 2 groups; inflammatory infiltration group and non-inflammatory infiltration group. The latter was further divided into 3 subgroups;endomysial inflammation subgroup, perivasculitis subgroup and transmural vasculitis subgroup.Immunohistochemical staining were carried out in inflammatory infiltration group with anti-CD3, anti-CD4,anti-CD8,anti-CD20 and anti-SMA antibody. The control group was composed of 10 dermatomyositis ( DM)cases and 10 polymyositis ( PM) cases. Results The age of onset was (25. 2 ± 12. 6) years old and the average course was (7. 8 ±7. 3) years. The sex ratio of male to female was 1.6: 1. Five of them had family history. The main clinical features were progressive weakness and atrophy of facial, shoulder girdles and proximal upper limbs muscles. The lower distal limbs and (or) lower distal limbs and pelvic girdle muscles were involved in 18 cases. The main pathological features were shown as followed. Seventeen of them had focal inflammatory cell infiltration, including endomysial inflammation (4/17) , perivasculitis (7/17) , and transmural vasculitis (6/17). Immunohistochemical staining confirmed the major types of inflammatory cells were CD4* T lymphocytes and CD20B lymphocytes, which was familiar with DM. While in PM, CD8+ T lymphocytes were dominant The proportionality of residual muscle fibers obviously decreased in inflammatory infiltration group ( 48. 0% ± 23. 6% ) than non-inflammatory infiltration group ( 94. 3% ±3. 1% , T = 198. 000, P = 0. 000). As to CK levels, there were no significant deviation. Conclusions Obvious inflammatory cell infiltration can be seen in FSHD, the locations of inflammatory cells are endomyosium inflammation, perivasculitis and transmural vasculitis. Transmural vasculitis indicates vascular pathological factor may have something to do with pathogenesis of FSHD.