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壞死性肉芽腫性血管炎一例誤診分析并文獻復習

壞死性肉芽腫性血管炎( necrotizing granulomatous vasculitis, NGV) 是一種病因未明的系統性血管炎[ 1] , 臨床上分全身型和局限型[ 2 ] 。全身型以上、下呼吸道和腎臟為常見受累器官, 無腎臟受累者則為局限型。該病男性略多于女性, 40 ～50 歲為高發年齡段。因其臨床表現多樣, 且無特異性, 常誤診為各亞專科疾病[ 3 ] 。2012 年11 月6 日, 鄭州大學第一附屬醫院呼吸及睡眠科收治1 例青年女性NGV患者, 現結合文獻復習如下。

盤狀紅斑狼瘡并發視網膜血管炎一例

HLA -A/B, HLA-DRB/DQB alleles in Eales disease

Objective To analyze the association between histocompatibility leukocyte antigen （HLA-A/B,HLA-DRB/DQB） alleles and Eales disease, and to explore susceptible genes and protective genes associated with Eales disease in a population of Han from ZUN YI city in Guizhou province. Methods The subjects were analyzed by casecontrol study consisted of two groups such as normal control group and Eales disease group. DNA samples from 100 healthy people and 26 patients with Eales disease were detected by polymeriase chain reaction with sequencespecific primers (PCR-SSP). The alleles of HLA-A/B and HLA-DRB/DQB from Eales disease group were compared with those from control group by SPSS 13.0. Results The distribution frequency in Eales disease was HLAA01(P=0.041, OR=20.5), A02(P=0.000, OR=54.667, OR 95%CI:11.837-252.473), B55 (P=0.047, OR=4.524; OR 95%CI:1.200-17.047), HLA-DRB01(P=0.048, OR=5.879, OR95%CI:1.227-28.169). DQB05 (P=0.021, OR=2.769, OR95%CI=1.145-6.692) alleles, and obviously higher than control, but the frequency of HLAA11 (P=0.031, OR=0.383, OR95%CI=0.158-0.930) was obviously lower than control (P＜0.05). Conclusion The results showed that the alleles of HLAA01, A02, B55, DRB01, DQB05 may associate with an antagonist effect in Eales disease, but HLAA11 may be a protective gene of this disease.

阿托伐他汀鈣對香煙暴露大鼠肺血管炎癥模型組蛋白脫乙酰酶2的影響

目的 探討阿托伐他汀鈣對香煙暴露大鼠肺血管炎癥的作用及對肺組織、血清中組蛋白脫乙酰酶2（HADC2）的影響。方法 將18只雌性SD大鼠隨機分為空白組（A組）、肺血管模型組（B組）、阿托伐他汀鈣干預組（C組），每組6只，使用煙熏加氣道滴入脂多糖經典造模方法對B組和C組大鼠進行香煙暴露肺血管炎癥模型制作，C組于第2天開始，每天熏煙前1 h用阿托伐他汀片灌胃治療，劑量5 mg/（kg·d），A、B組同期予以等量生理鹽水進行灌胃，35 d后處死大鼠。使用蘇木精–伊紅染色對大鼠各組病理組織進行染色，并對血管的炎癥進行評分。酶聯免疫吸附試驗檢測血清中HDAC2水平，蛋白印跡法檢查肺組織中HDAC2蛋白水平，實時熒光定量聚合酶鏈反應檢測肺組織HDAC2 mRNA水平。結果 與A組比較，B、C組的肺血管炎癥明顯，但C組輕于B組。A組炎癥評分為（0.5±0.5）分，略低于C組［（1.7±0.7）分］，顯著低于B組［（2.7±1.0）分］，C組炎癥評分顯著低于B組。A組大鼠血清中HADC2水平為（12.76±0.63）ng/mL，略高于C組［（11.59±0.60）ng/mL］，但C、A組明顯高于B組［（2.27±0.42）ng/mL］，三組之間的差異有統計學意義（P<0.05）。B組大鼠肺組織中HDAC2蛋白表達明顯減少，而通過阿托伐他汀鈣干預后，C組的HDAC2表達（0.30±0.02）相較于B組（0.09±0.01）明顯增加，略低于A組（0.35±0.04）；A組（1.23±0.06）、C組（0.82±0.03）的HDAC2 mRNA水平明顯高于B組（0.27±0.02），三組之間的差異有統計學意義（P<0.05）。結論 阿托伐他汀鈣可減輕香煙暴露大鼠肺血管炎癥的程度，其機制可能與增加HDAC2的表達有關。

特發性視網膜血管炎、視網膜動脈瘤、視神經視網膜炎綜合征11例

樹冰狀視網膜血管炎（附二例報告）

報告二例較典型的樹冰狀視網膜血管炎，均為小兒。一例用皮質激素治療，另一例辯證內服中藥結合局部激素眼藥水點眼，均獲治愈，本文結合文獻對本病的病因、臨床特點、眼底血管熒光照影表現、治療、預后及鑒別診斷進行簡要討論。 （中華眼底病雜志，1992，8：36-37）

霜樣樹枝狀視網膜血管炎

霜樣樹枝狀視網膜血管炎(frosted branch angiitis)是一種特殊類型的視網膜血管炎，以廣泛的視網膜血管旁滲出為特征，可分為不合并全身表現的特發型與合并全身病變，特別是人類免疫缺陷病毒(human immunodefecient virus,HIV)感染的全身型。前者主要全身使用皮質類固醇治療，預后好，后者則需聯合病因治療，預后較差。

Clinical Characteristics and Renal Outcome in Elderly Patients with Antineutrophil Cytoplasmic Autoantibody Associated Vasculitis with Renal Involvement

ObjectiveTo analyze the clinical characteristics and renal outcome of elderly patients with antineutrophil cytoplasmic autoantibody (ANCA) associated vasculitis (AAV) with renal involvement. MethodsWe retrospectively analyzed the clinical data of 147 patients with ANCA relate vasculitis treated between June 2006 and June 2012. Based on the age, the patients were divided into elderly group (65 years or older, n=50) and non-elderly group (younger than 65, n=97). The disease course, clinical characteristics, ANCA serological indexes, renal pathological change and prognosis of patients in the two groups were compared and studied. ResultsIn the elderly group, there were 3 cases of Wegener granulomatosis (WG), 45 of microscopic polyangiitis (MPA), and 2 of pauci-immune crescentic glomerulonephritis (PICGN). The non-elderly group had 8 cases of WG, 82 of MPA, 6 of PICGN, and 1 of allergic angitis granulomatosis. There were 5 cases of positive cANCA and 44 of positive pANCA in the elderly group. The elderly patients had significantly more pulmonary involvement than the younger patients (P=0.030). No significant difference was detected between the two groups in combined pulmonary infection (P=0.281) or combined infectious index C-reactive protein (P=0.326). Elderly patients were less likely to respond to sufficient treatment with pulse intravenous methylprednisolone therapy (P=0.035) and cyclophosphamide (P=0.043), and had worse renal outcome than younger patients (P=0.040). ConclusionElderly patients with AAV have more prevalent pulmonary involvement and have severe complication of pulmonary infection, which affects mortality and morbidity of ANCA-associated systemic vasculitis.

視盤靜脈血管炎11例

雙眼特發性視網膜血管炎、動脈瘤、視神經視網膜炎綜合征二例