老年系统性血管炎合并肠梗阻2例并文献复习

目的提高对老年系统性血管炎的认识。 方法回顾性分析2006年1月至2017年1月广州军区广州总医院收治的89例老年系统性血管炎患者的临床资料,重点分析其中合并肠梗阻的2例患者,分析其临床特点、诊疗过程以及预后。 结果老年系统性血管炎临床表现多样,发热为常见症状,常伴有乏力、咳嗽、头晕、胸痛、失眠等非特异性症状;全身各个脏器均可受累,以肾脏、肺脏受累最为常见;多数患者血沉、C反应蛋白可升高。合并肠梗阻的2例患者中,1例诊断为"过敏性血管炎",经激素联合吗替麦考酚酯治疗后病情好转;另1例因肠梗阻入院行手术治疗,误诊为"缺血性肠病",1年后患者出现进行性肾功能损害,血清胞浆型和核周型抗中性粒细胞胞浆抗体强阳性,修正诊断为"显微镜下血管炎"。因患者高龄未接受激素治疗,迅速出现急性肾功能衰竭,治疗无效死亡。 结论以肠梗阻为首发症状或并发症的老年患者需排除系统性血管炎,以免延误诊断治疗。     

系统性小血管炎的分类

过去的150年间,随着对肾、肺等活体器官病理研究及抗中性粒细胞胞浆抗体（anti-neutrophil cytoplasmic autoantibodies, ANCA）的发现,人们对血管炎发病机制研究的深入,小血管炎的分类发生了很大的变化.根据目前的分类方法,寡免疫性小血管炎包括Wegner肉芽肿（Wegner＇s granulomatosis, WG）、微型多血管炎（microscopic polyangitis, MPA）、Churg-strauss综合征（Churg-strauss syndrome,CSS）[1].这是一类与ANCA相关,临床又难以区分的系统性小血管炎,因而统称为ANCA相关性小血管炎（简称ANCA小血管炎）.本文介绍血管炎分类的历史演变、ANCA致病机制,并对现行的血管炎分类进行评价.     

抗中性粒细胞胞浆抗体小血管炎24例临床分析

目的对我院24例抗中性粒细胞胞浆抗体小血管炎患者的临床资料进行临床分析。方法对确诊为抗中性粒细胞胞浆抗体小血管炎患者的临床表现、实验室检查、肾脏病理、诊治及预后进行分析。结果24例患者均为抗中性粒细胞胞浆抗体（ANCA）阳性,均有肾脏受累,同时还伴有肺、胃肠道、眼、耳、鼻、声带、皮肤、关节、甲状腺及外周神经受累。临床表现复杂多样,平均确诊时间为（9±4）个月。治疗以糖皮质激素加用环磷酰胺为主。总缓减率为78％。结论抗中性粒细胞胞浆抗体小血管炎为多系统受累疾病,漏诊误诊率高,早期诊治能提高生存率,但部分患者预后不佳。     

抗中性粒细胞胞浆抗体相关性小血管炎肺脏受累12例临床分析

目的 提高对抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎肺脏受累的认识.方法 对2004年11月至2007年12月广西医科大学第一附属医院住院的12例ANCA相关性小血管炎肺脏受累病例的临床特点进行回顾性分析.结果 (1)本组患者以中老年多见,年龄平均(59.17±16.85)岁.(2)主要临床表现为:①呼吸道症状为咳嗽(91.7%)、呼吸困难(50.0%)、咯血(16.7%);②常伴发热(83.3%)和体重下降(41.7%)等全身非特异性表现,肺外表现有贫血(100%)、关节肌肉痛(50.0%)及皮疹(50.0%)等;③泌尿系统症状:血尿(83.3%)、蛋白尿(75.0%);④所有患者均有3个或3个以上器官受累.(3)实验室检查:红细胞沉降率增快(100%),C-反应蛋白增高(83.3%);血肌酐及尿素氮均明显增高(83.3%),ANCA阳性.(4)肺部影像学表现:①肺部多发性病变,可单侧、双侧,以双侧(66.7%)受累多见;②病变多样:主要表现为不规则散在斑片状、斑点条索状致密影、多发结节影及胸腔积液;③经糖皮质激素联合免疫抑制剂治疗病灶明显吸收或消失.(5)12例行经皮肾穿刺活检,病理上主要为少或无免疫复合物沉积的局灶坏死性肾小球肾炎或新月体性肾小球肾炎.(6)12例患者首诊全部误诊,最常误诊疾病为肺炎(50.0%)、肺结核(25.0%);确诊后经糖皮质激素联合免疫抑制剂治疗有效;3例患者肺部病变复发,1例患者死于呼吸衰竭;复发及死亡病例与病理类型有关.结论 该病临床上以发热和呼吸系统、泌尿系统及其他多器官受累为特点,影像学表现形式多样,临床误诊率高,诊断需结合ANCA检测及病理检查,糖皮质激素联合免疫抑制剂治疗有效,预后与病理类型有关.     

抗中性粒细胞胞浆抗体阳性与阴性微型多血管炎的比较

目的比较抗中性粒细胞胞浆抗体(ANCA)阳性(ANCA+ve)与ANCA阴性(ANCA-ve)微型多血管炎(MPA)临床与病理的差异,探讨ANCA与MPA病情及预后的关系.方法31例微型多血管炎(MPA),其中男16例,女15例,同时采用标准间接免疫荧光(IF)和ELISA法检测血清ANCA,根据血清ANCA结果分为ANCA+ve组与ANCA-ve组,比较两组肾脏及肾外脏器损害、肾脏病理及预后.两组患者血管炎活动分数(BVAS1)无差异.结果①血清ANCA31例MPA中19例ANCA阳性(61.3%),其中IF-ANCA阳性者8例,ELISA法ANCA阳性者19例.血清抗-GBM抗体均阴性.②发病年龄及病程ANCA+ve组大于ANCA-ve组,分别为(56±11)岁vs(48.5±16.8)岁(P＜0.05)和(30.2±47.7)月vs(6.5±8.8)月(P＜0.01).ANCA+ve组女性多于男性(男/女8/11),而ANCA-ve组男性多见(男/女8/4).③ANCA+ve组需透析病例数及活检时SCr水平(588±334)μmol/L高于ANCA-ve(487±284)μmol/L(P＜0.05),贫血程度更为严重,Hb(78.9±16.2)g/Lvs(95.5±22.0)g/L(P＜0.01).肾活检显示ANCA+ve组新月体比例显著高于ANCA-ve组(61.7±33.2)%vs(33.4±32.8)%(P＜0.01),ANCA-ve组细胞性新月体所占比例高于ANCA+ve组.两组袢坏死、全球硬化、间质血管炎及酸性粒细胞浸润程度相似.④ANCA+ve组伴有2个以上肾外脏器受累比例高于ANCA-ve组(63.2%vs31.3%),肺损害较重,咯血(42.1%)及严重肺出血(15.8%)患者均见于ANCA+ve组.ANCA+ve组消化道出血及眼部受累多见,而皮肤损害ANCA-ve组发生率高于ANCA+ve组(41.7%vs15.8%).⑤ANCA-veMPA预后差.两组治疗方法相似,但治疗后ANCA+ve组仅1例肾功能恢复正常,绝大多数患者需维持透析或处于CRF,5例死亡,而ANCA-ve组3例需要维持性透析,4例肾功能恢复正常,无一例死亡,其余病例肾功能均显著改善.结论血清ANCA阳性与ANCA阴性MPA无论在临床表现、肾脏病理及预后方面均存在一定差异,临床应分别对待.造成这种差异的机制有待进一步研究.     

抗中性粒细胞胞浆抗体与血管炎

讨论Ｈｉｌｂｅｒｔ空间上标型谱算子的基本性质。推广关于次正规算子不变子空间存在性的Ｂｒｏｗｎ定理以及关于本质正规算子本质本等价的Ｂｒｏｗｎ－Ｄｏｕｇｌａｓ－Ｆｉｌｌｍｏｒｅ定理。     

系统性硬化症合并抗中性粒细胞胞浆抗体相关性血管炎一例并文献复习

<正>1临床资料患者,女性,53岁,主因“双手皮肤紧硬26年,咳嗽7年,血肌酐升高1个月余”来诊。1995年下半年双手遇冷后变白、变紫,逐渐出现双手指对称性肿胀及关节疼痛、手足皮肤增厚,1996年就诊于中国人民解放军总医院第一医学中心门诊,完善检查（具体不详）后诊断“系统性硬化症”,口服青霉胺0.2 g/d,病情稳定。2014年7月出现咳嗽、白痰,伴喘憋,当地胸部CT示：肺间质病变,加“醋酸泼尼松30 mg/d、羟氯喹（0.2 g 2次/d）、环磷酰胺100 mg/d”治疗,后自行停药。     

肺血管炎及其诊治概述

1肺血管炎的概念和分类血管炎系病理学名词,指以血管壁炎症和坏死为特征的病理改变。血管炎可以累及动脉、静脉和毛细血管。临床上,血管炎是指病理主要表现为血管炎的一组异质性疾病。根据血管炎的病因可将其分为原发性血管炎和继发性血管炎。病因不明的血管炎称为原发性血管炎     

Systemic Vasculitis During the Course of Systemic Sclerosis: Report of 12 Cases and Review of the Literature

Although the presence of antineutrophil cytoplasmic antibodies (ANCA) has been reported in patients with systemic sclerosis (SSc), the association of SSc and systemic vasculitis has rarely been described. We obtained information on cases of systemic vasculitis associated with SSc in France from the French Vasculitis Study Group and all members of the French Research Group on Systemic Sclerosis.We identified 12 patients with systemic vasculitis associated with SSc: 9 with ANCA-associated systemic vasculitis (AASV) and 3 with mixed cryoglobulinemia vasculitis (MCV). In all AASV patients, SSc was of the limited type. The main complication of SSc was pulmonary fibrosis. Only 2 patients underwent a D-penicillamine regimen before the occurrence of AASV. The characteristics of AASV were microscopic polyangiitis (n = 7) and renal limited vasculitis (n = 2). Anti-myeloperoxidase antibodies were found in 8 of the 9 patients. The Five Factor Score was above 1 in 3 of the 9 patients. Of the 3 patients with MCV, Sjögren syndrome was confirmed in 2. We compared our findings with the results of a literature review (42 previously reported cases of AASV with SSc).Although rare, vasculitis is a complication of SSc. AASV is the most frequent type, and its diagnosis can be challenging when the kidney is injured. Better awareness of this rare association could facilitate earlier diagnosis and appropriate management to reduce damage.     

无肌病性皮肌炎15例临床分析

目的：探讨无肌病性皮肌炎（ADM）的临床特征、诊断、治疗和预后。提高临床医师对ADM的诊治水平。方法：将89例患者分为2组．其中有Gouron丘疹。或有眶周水肿性淡紫色斑疹,而临床缺乏明显肌炎者15例为ADM组,皮肌炎组（1）M组）74例。分别记录其临床特征、肌酸激酶、肌电图、肌肉病理检查、胸部影像学检查、器官受累情况、治疗方案和转归。结果：ADM组关节痛、发热、继发肺间质炎症和抗Jo-1抗体阳性的发生率分别为40％、13．3％、66．7％和13．3％,并发肿瘤6．7％,病死率20％。DM组关节痛、发热、肺间质炎症和抗Jo-1抗体阳性的发生率分别为37．8％、14．9％、32．4％和14、9％,病死率5．4％。二者比较,ADM组肺间质炎症发生率明显高于DM组。结论：ADM组虽然没有明显肌病表现,但是同样可以累及内脏,其中肺间质炎症的发生率较高,联合糖皮质激素和细胞毒免疫抑制剂治疗可以改善患者预后。临床医师需提高对ADM的认识。     

25例脑脉管炎临床资料分析

目的:探讨脑脉管炎的临床特征、血清学及影像学特点以指导临床诊疗.方法:回顾性分析25例拟诊为脑脉管炎患者的临床资料,结合近年文献讨论脑脉管炎的特点.结果:25例患者中40～50岁发病数最多.发病与钩端螺旋体感染有关.临床主要表现为脑病、头痛和不同程度的局灶性神经体征.MRI示颅内病变多累及皮层和皮层下,多发病灶22例(...     

抗中性粒细胞胞浆抗体相关性小血管炎3例临床分析

原发性小血管炎 ,又称抗中性粒细胞胞浆抗体 (ＡＮＣＡ)相关性小血管炎 ,是致中老年人继发性肾损害的一种自身免疫性疾病之一 ,我科近 4年来诊断 3例 ,介绍如下。临床资料例 1　男性 ,74岁。因发热、咳嗽、咯少许的痰半个月入院 ,伴消瘦、乏力、纳差、腹胀 ,发病前 1周皮肤带状疱疹 ,既往体健。体检 :Ｔ 37～ 38℃ ,热型不规则 ,神志清楚 ,倦怠。无皮疹及出血。浅表淋巴结无肿大。双球结膜明显充血 ,并见栓塞点 ,巩膜无黄染。双肺呼吸音粗 ,未闻及干湿性罗音 ,心脏听诊无异常。腹平软 ,无压痛 ,肝脾未扪及肿大。双肾区无叩痛。无浮肿。化验 …     

Single-Organ Gallbladder Vasculitis: Characterization and Distinction From Systemic Vasculitis Involving the Gallbladder. An Analysis of 61 Patients

Systemic vasculitis (SV) involving abdominal structures usually has a poor prognosis. Gallbladder vasculitis (GV) has been reported as part of SV (GB-SV) and focal single-organ vasculitis (GB-SOV). We analyzed clinical and histologic characteristics of patients with GV to identify features that differentiate GB-SOV from the systemic forms of GV. To identify affected patients with GV we used pathology databases from our institution and an English-language PubMed search. Clinical manifestations, laboratory and histologic features, treatment administered, and outcomes were recorded. Patients were divided in 2 groups, GB-SOV and GB-SV. As in previous studies of single-organ vasculitis, GB-SOV was only considered to be a sustainable diagnosis if disease beyond the gallbladder was not apparent after a follow-up period of at least 6 months. Sixty-one well-characterized patients with GV were included (6 from our institution). There was no significant sex bias (32 female patients, 29 male). Median age was 52 years (range, 18–94 yr). GB-SOV was found in 20 (33%) and GB-SV in 41 (67%) patients. No differences were observed in age, sex frequency, or duration of gallbladder symptoms between groups. Past episodes of recurrent right-upper quadrant or abdominal pain and lithiasic cholecystitis were more frequent in GB-SOV patients, whereas acalculous cholecystitis occurred more often in GB-SV. In GB-SV, gallbladder-related symptoms occurred more often concomitantly with or after the systemic features, but they sometimes appeared before SV was fully developed (13.5%). Constitutional and musculoskeletal symptoms were reported only in GB-SV patients. Compared to GB-SOV, GB-SV patients presented more often with fever (62.5% vs 20%; p = 0.003) and exhibited higher erythrocyte sedimentation rate levels (80 ± 28 vs 37 ± 25 mm/h, respectively; p = 0.006). All GB-SV patients required glucocorticoids and 50% of them also received cytotoxic agents. Mortality in GB-SV was higher than in GB-SOV (35.5% vs 10%; p = 0.05). Nongranulomatous inflammation with fibrinoid necrosis of medium-sized vessels occurred equally in both groups (>90%). Forms of SV affecting the gallbladder included polyarteritis nodosa (n = 10), hepatitis B virus-associated vasculitis (n = 8), cryoglobulinemic (essential or hepatitis C virus-associated) vasculitis (n = 6), vasculitis associated with autoimmune diseases (n = 6), microscopic polyangiitis (n = 4), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (n = 4), IgA vasculitis (Henoch-Schönlein) (n = 2), and giant cell arteritis (n = 1).GV is uncommon. Its histology most often consists of a nongranulomatous necrotizing vasculitis affecting medium-sized vessels. GB-SOV is usually discovered after routine cholecystectomy performed because of the presence of local symptoms, gallstone-associated cholecystitis, and contrary to GB-SV, GB-SOV is usually not associated with systemic symptoms. Acute phase reactants and surrogate markers of autoimmunity are usually normal or negative in GB-SOV. GB-SOV does not require systemic antiinflammatory or immunosuppressive therapy; surgery is adequate to achieve cure. GB-SV always warrants immunosuppressant therapy and is associated with high mortality. The finding of GV may precede the generalized manifestations of SV. Therefore, once GV is discovered, studies to determine disease extent and a vigilant follow-up are mandatory.