CAJ | 학술논문

目的探讨Wegener's肉芽肿病(WG)的临床病理学特点,提高对本病的认识.方法回顾性分析1999年2月至2012年7月我院诊治的21例WG患者的临床、病理资料,其中男11例,女10例；年龄45～76岁,平均58.1岁.34个(包括2例尸检)不同部位的标本常规取材、制片,并进行苏木精-伊红(HE)、组织化学染色,13例肾穿标本全部行免疫荧光染色和电镜检查,观察病理形态特征. 结果从初发到确诊的时间为24 d至11.0个月,平均为5.3个月.眼、鼻和涎腺是本病病初最容易受累的部位,共11例(52.4％)；皮肤受累3例(14.3％),17例(81.0％)累及肺脏,15例(71.4％)累及肾脏.18例检查抗中性粒胞浆抗体(ANCA),13例(72.2％)胸型ANCA (cANCA)阳性；3例(16.7％)核周型ANCA(p-ANCA)阳性；2例(11.1％)ANCA阴性.病理学主要表现:(1)7种血管炎,依次为毛细血管炎、急性血管炎、慢性血管炎、坏死性纤维素性血管炎、坏死性肉芽肿性血管炎、非坏死性肉芽肿性血管炎、瘢痕性血管炎；(2)4种肉芽肿性炎,依次为散在分布的巨细胞、栅栏状排列的组织细胞、松散的小肉芽肿及微脓肿周围车轮状排列的组织细胞；(3)2种实质的变性坏死,地图状坏死、中性粒细胞微脓肿.2例尸检3类13种形态的表现均可观察到,活检小标本可见不同种类的形态变化和弥漫性肺出血等次要表现. 结论 Wegener's肉芽肿病有复杂的病理形态学的变化谱系,主要表现为血管炎、肉芽肿性炎和实质的坏死.
목적 탐토Wegener's육아종병(WG)적림상병이학특점,제고대본병적인식.방법 회고성분석1999년2월지2012년7월아원진치적21례WG환자적림상、병리자료,기중남11례,녀10례；년령45～76세,평균58.1세.34개(포괄2례시검)불동부위적표본상규취재、제편,병진행소목정-이홍(HE)、조직화학염색,13례신천표본전부행면역형광염색화전경검사,관찰병리형태특정. 결과 종초발도학진적시간위24 d지11.0개월,평균위5.3개월.안、비화연선시본병병초최용역수루적부위,공11례(52.4％)；피부수루3례(14.3％),17례(81.0％)루급폐장,15례(71.4％)루급신장.18례검사항중성립포장항체(ANCA),13례(72.2％)흉형ANCA (cANCA)양성；3례(16.7％)핵주형ANCA(p-ANCA)양성；2례(11.1％)ANCA음성.병이학주요표현:(1)7충혈관염,의차위모세혈관염、급성혈관염、만성혈관염、배사성섬유소성혈관염、배사성육아종성혈관염、비배사성육아종성혈관염、반흔성혈관염；(2)4충육아종성염,의차위산재분포적거세포、책란상배렬적조직세포、송산적소육아종급미농종주위차륜상배렬적조직세포；(3)2충실질적변성배사,지도상배사、중성립세포미농종.2례시검3류13충형태적표현균가관찰도,활검소표본가견불동충류적형태변화화미만성폐출혈등차요표현. 결론 Wegener's육아종병유복잡적병리형태학적변화보계,주요표현위혈관염、육아종성염화실질적배사.
Objective To study the clinical pathological features of Wegener's granulomatosis (WG) in middle-aged and elderly patients,and enhance understanding of this disease.Methods Totally 21 patients with WG (11 males,10 females,aged 45 to 76 years,mean age 58.1 years) in our hospial from February 1999 to July 2012 were selected.The clinical and pathological data of WG patients were retrospectively analyzed.34 biopsies including 2 autopsies from different organs were paraffin embedded and stained by hematoxylin and eosin and histochemistry.13 renal biopsies were all examined by immunofluorescence and electron microscope.Results The average time from the onset of clinical symptoms to the diagnosis was 5.3 months (from 24 days to 11.0 months).Eyes,nose and salivary glands were the most commonly involved parts at the beginning of Wegener's granulomatosis (52.4％,11 cases).The percentages of the skin,lung and renal involvement were 14.3％ (3 cases),81.0％ (17 cases) and 71.4％ (15 cases),respectively.Among 21 patients,18 patients were examined with anti-neutrophil cytoplasmic antibody (ANCA).c-ANCA was positive in 72.2 ％ patients (13 cases,13/18),p-ANCA was positive in 16.7％ patients (3 cases,3/18),and ANCA was negative in 11.1％ patients (2 cases,2/18).3 major pathological manifestations were observed:7 kinds of vasculitis including capillaritis,acute vasculitis,chronic vasculitis,fibrinoid necrosis in vasculitis,necrotizing granulomatous vasculitis,non-necrotizing granulomatous vasculitis and cicatricial vascular changes; 4 kinds of granulomatous inflammation including scattered giant cells,palisading histiocytes,poorly formed granulomas and microabscess surrounded by granulomatousinflammation;2 kinds of parenchymal necrosis including geographic necrosis and microabscess.13 kinds of histopathologic features in 3 major manifestations were found from 2 autopsies,but various kinds histopathologic features presented in small biopsy samples.Minor manifestations such as diffuse pulmonary hemorrhage were found at the periphery of WG.Conclusions The wide variation and broad spectrum of pathologic features can occur in WG.Vasculitis,granulomatous inflammation and parenchymal necrosis are the most important histopathological features.The correct diagnosis of WG requires careful correlation of pathology with complicated clinical features.