血管内皮细胞功能与结节性多动脉炎

血管内皮细胞功能与结节性多动脉炎中心发言杨俊伟１病史概要男性患者，４７岁，以高血压２年，大量蛋白尿，肾功能异常入我院。患者于１９９２年体检时发现高血压２４．０／１６．０ｋＰａ，伴轻度头昏、头痛，无明显视物模糊、耳鸣、浮肿等症状。当时未做全面检查，也未...     

结节性多动脉炎及微型多发性血管炎的比较

系统性血管炎的分类标准至今未能确定。几十年来，人们习惯将结节性多动脉炎（dyarterltlsnodosa，PAN）与微型多发性血管炎（mlcrosconlcnolvangiitis，MPA）等同考虑，甚至美国风湿病协会制定的PAN诊断标准亦未将PAN与MPA相区分。但近年研究表明，PAN与MPA是两种不同类型的血管炎，现将这两种血管炎的临床及组织学特点介绍如下：三微型多发性血管炎（MPA）Dovson，Bull及Putt首先描述了微型结节性多动脉炎的病理改变。他们发现，在PAN患者所见到的节段坏死性肾小管肾炎实际上是一种微血管型PAN，它与Maier及Kussmaul等描述…     

结节性多动脉炎累及冠状动脉的临床特点与诊治

<正>结节性多动脉炎(polyarteritisnodosa,PAN)是一种系统性坏死性血管炎,主要累及中小动脉,任何系统脏器的动脉均可受累。对PAN的诊断,目前均采用1990年美国风湿病学会(ACR)的分类标准[1]:(1)体质量下降≥4 kg(无节食或其他原因所致);(2)网状青斑(四肢和躯干);(3)睾丸痛和(或)压痛(并非感染、外伤或其他原因引起);(4)肌痛、乏     

结节性多动脉炎

结节性多动脉炎 (PAN) 186 6年由 Kaussmaul和 Maier首次提出。此后多年 PAN曾被用于小血管炎的通用名词 ,包涵了多种目前已独立出来的系统性血管炎 ,如 Wegener肉芽肿、巨细胞动脉炎等。近 10年来随着对血管炎认识的不断深入 ,特别是 1993年 Chapel- Hill会议新的分类标准出台 ,后显微镜下多血管炎 (MPA)也从 PAN中鉴别出来 ,PAN的概念更为明确。目前 PAN基本概念包括三个方面 :1中等或肌性小动脉受累 ,即不累及微小动脉、毛细血管或静脉系统 ;2PAN为非肉芽肿性的坏死性血管炎 ;3抗中性粒细胞胞浆抗体 (ANCA)阴性。1　诊断由于…     

结节性多动脉炎16例临床分析

结节性多动脉炎(PAN)是坏死性血管炎的一种，其特征主要是累及中、小动脉的节段性坏死性病变。该病是一少见疾病，不易早期诊断，而早期治疗是影响预后的因素之。本文总结了北京协和医院1990—2001年住院病人确诊PAN共16例，旨在提高临床医生对本病的认识，尽早明确诊断，合理治疗，改善预后。     

结节性多动脉炎合并心肌梗死8例临床分析

目的总结结节性多动脉炎累及冠状动脉导致心肌梗死患者的临床表现及冠状动脉受累特点。方法对北京协和医院有病案记录的结节性多动脉炎合并心肌梗死的8例患者的临床症状、系统受累、实验室检查、冠状动脉及其他血管造影、超声心动图和病理检查等进行回顾性分析。结果 8例患者中男性5例,女性3例,年龄21～52岁,平均(37.6±11.7)岁。胸痛6例,心力衰竭1例。心电图缺血性ST-T改变5例,除窦性心动过速、房性期前收缩外,未见其他恶性心律失常。左心室射血分数降低(≤50%)3例,节段性室壁运动异常6例,室壁瘤形成2例,心肌病变2例,肺高压1例。4例有冠状动脉影像学检查资料,均累及右冠状动脉,3例为三支病变,1例为两支病变,其造影结果描述为冠状动脉弥漫性病变、冠状动脉扩张、多发动脉瘤以及节段性狭窄、闭塞。这些患者均有其他多部位血管受累的表现和动脉造影检查的异常。结论结节性多动脉炎可累及冠状动脉导致心肌梗死,其冠状动脉受累常为多支病变、多有右冠状动脉受累,动脉瘤伴血栓形成和节段性狭窄。对冠心病低危心肌梗死的患者,需要完善血管检查,警惕结节性多动脉炎累及冠状动脉的情况。     

结节性多动脉炎

为提高医疗质量，规范各级医疗机构医师的诊疗行为，以切实保障我国广大人民群众的身体健康，在卫生部、中华医学会的直接领导下，各专科分会从2002年1月起着手编写“临床诊疗指南”。为适用于我国不同等级和不同地区医院医师水平的需要，中华医学会风湿病学分会组织了风湿病学专家编写了22种常见风湿性疾病的诊治指南。各位专家在撰写过程中倾注了心血，以严肃认真、严谨求精的态度完成了“指南”的编写。“指南”既代表了当前国际上的诊治水平，又符合我国的国情，具有实用性。历时1年余，几经易稿，终于完成。《中华风湿病学杂志》陆续将“指南”以“草案”刊出，以进一步征集广大医师的意见。有任何建议及意见可与中华医学会风湿病学分会唐福林联系。通信地址：北京市东城区王府井帅府园1号，北京协和医院风湿免疫科，邮编：100730，Email：Tangn@csc，pumch.ac.cn，传真：010-65296563     

结节性多动脉炎的诊治进展

结节性多动脉炎（PAN）在早期是指代大多数的血管炎.随着对血管炎研究的深入,越来越多的特异性血管炎被划分出来.以前的“PAN合并类风湿关节炎”现在被命名为“类风湿关节炎血管炎”;而大多数“累及肺部的结节性多动脉炎”则改称为“嗜酸性肉芽肿性多血管炎”（EGPA,既往称为churg-strauss综合征）;随着抗中性粒细胞胞浆抗体（ANCA）检查在临床中的普及以及对ANCA在血管炎中的致病作用研究,显微镜下多血管炎（MPA）也从PAN中被区分出来[1].     

结节性多动脉炎的诊断和治疗进展

结节性多动脉炎（PAN）是最早被阐述的系统性血管炎，是累及中等大小动脉或小动脉的一种坏死性血管炎，常有多系统受累。临床表现复杂且缺乏特异性，往往没有肾小球肾炎、肺损害及抗中性粒细胞胞浆抗体（ANCA）。PAN的诊断依赖于临床表现、实验室检查以及病理检查，找出其重要线索如：周围神经病变、胃肠道损害、高血压、HBsAg阳性等才能作出正确诊断。PAN的治疗主要是肾上腺糖皮质激素，对有重要脏器损害的患者使用细胞毒药物和血浆置换也是必要的。     

新分类标准对结节性多动脉炎诊断影响的研究初探

随着对抗中性粒细胞胞质抗体（ANCA）相关性血管炎的认识及乙型病毒性肝炎疫苗的广泛接种,结节性多动脉炎（PAN）的诊断发生了很大变化。本研究结合2012年Chapel Hill共识会议（CHCC）对PAN的定义和2007年欧洲药品管理局 （EMA）对中小血管炎分类法则,对2002年1月至2018年12月在北京协和医院住...     

Polyarteritis nodosa with mesenteric aneurysms demonstrated by angiography: report of a case and successful treatment of the patient with prednisolone and cyclophosphamide

Polyarteritis nodosa is a necrotizing angitis that predominantly affects small and medium-sized arteries. The prognosis of untreated polyarteritis nodosa is very poor. Since symptoms are diverse and no serologic test is specific for polyarteritis nodosa, the diagnosis is difficult and often delayed. We describe a patient with polyarteritis nodosa who had gastrointestinal involvement with multiple aneurysms of the inferior mesenteric artery; only abdominal angiography provided a conclusive diagnosis. Alleviation of symptoms and regression of aneurysms were observed after combination therapy of an immunosuppressive agent, cyclophosphamide, and prednisolone. We emphasize the importance of early diagnosis by angiography and aggressive therapy in patients in whom physical signs indicating definite polyarteritis nodosa are not present. Received: January 18, 1999 / Accepted: May 28, 1999     

Remission of Angiographically Confirmed Minocycline-induced Renal Polyarteritis Nodosa: A Case Report and Literature Review

A 23-year-old man presented with severe hypertension. Based on his history of minocycline treatment for over three years and clinical symptoms, such as myalgias and renovascular hypertension with multiple intrarenal aneurysms, he was diagnosed with minocycline-induced renal polyarteritis nodosa (PAN). After minocycline treatment cessation and management of the hypertension, his blood pressure, renin-aldosterone levels, and urinary protein levels gradually improved. Seven and a half years later, repeated angiography found that the aneurysms had resolved. This is the first report in English describing a case of minocycline-induced renal PAN that was reversed functionally and morphologically without steroids or immunosuppressive drugs.     

Polyarteritis nodosa presenting as acute myocardial infarction with coronary dissection

Coronary manifestations of polyarteritis nodosa (PAN) are rarely identified pre-mortem. We report a 51-year-old female with PAN causing a coronary dissection and new lesions developing over the next 5 days. PAN should be added to the list of differential diagnoses for spontaneous coronary dissections. Cathet. Cardiovasc. Diagn. 44:320–324, 1998. © 1998 Wiley-Liss, Inc.     

A Case of Polyarteritis Nodosa Presenting Initially as Peripheral Vascular Disease

Polyarteritis nodosa is a rare necrotizing vasculitis that can be progressive and fatal, and its initial presenting symptom may be leg claudication due to peripheral vascular ischemia. To date, there have been fewer than ten case reports of polyarteritis nodosa presenting as peripheral vascular disease. We report a case of a 38-year-old man initially diagnosed to have premature peripheral vascular disease who presented 1 year later with symptoms consistent with giant cell arteritis and subsequently developed bowel ischemia leading to a fatal outcome. Based on the autopsy and the patient’s clinical course, the final diagnosis was polyarteritis nodosa. This case illustrates the challenges in diagnosing polyarteritis nodosa and the importance of considering vasculitis in young patients presenting with atypical presentations of diseases such as peripheral vascular disease or giant cell arteritis.     

Classic Polyarteritis Nodosa Presenting Rare Clinical Manifestations in a Patient with Hemophilia A

A 35-year-old patient with hemophilia A presented with rapidly progressive polyarteritis nodosa (PAN). He had been infected with hepatitis B virus (HBV) by repeated transfusion and was positive for hepatitis B surface antigen but negative for hepatitis B surface antibody. The patient presented symptoms of acute epididymitis followed by emergency admission because of acute appendicitis. On day 7 of admission, he complained of severe back pain, and computerized tomography (CT) showed massive perirenal hematoma. On day 49, mild monoplegia in the left arm suddenly developed, and CT and magnetic resonance imaging revealed multiple cerebral infarctions. Factor VIII replacement therapy was attenuated; however, cerebral infarction was progressive and extended throughout the cerebral hemispheres. He was diagnosed with classic polyarteritis nodosa (cPAN), and pulse methylprednisolone was continued. The patient died of supratentorial herniation, and autopsy revealed that vasculitis associated with intimal thickening was present in the liver, pancreas, intestine, kidneys, and larger-sized cerebral arteries. The development of cPAN appeared to have originated from chronic HBV infection, and this is the first report of cPAN in hemophilia patients. Concomitant hemorrhagic and thrombotic manifestations of cPAN are hardly treatable in patients with coagulation disorders, and the current case may represent a rare transfusion-related complication in hemophilia patients.     

Pneumatosis Cystoides Intestinalis Following Steriod Pulse Therapy in a Patient with Polyarteritis Nodosa

We present a case of a 67‐year‐old female with polyarteritis nodosa, complicated by pneumatosis cystoides intestinalis. The definite diagnosis of polyarteritis nodosa was obtained by histological findings on a biopsy specimen from her sural nerve. Following steroid pulse therapy, she developed gastrointestinal symptoms such as nausea, abdominal fullness and watery diarrhea. Abdominal X‐ray and computed tomography showed an abundance of gas contrast within the large intestinal wall. Colonoscopy revealed submucosal tumor‐like multiple elevated lesions from the sigmoid to transverse colon. These findings indicated pneumatosis cystoides intestinalis and the patient was accordingly treated with a high concentration of oxygen via a nasal catheter. One week later, her symptoms and the gas in the bowel wall completely disappeared. Pneumatosis cystoides intestinalis may be one of the complications of steroid pulse therapy.