如何提高小血管炎肾损害的远期预后

微型多血管炎(Microscopie polyangiitis,MPA)、Wegener肉芽肿等系统性小血管炎累及肾脏以及局限于肾脏的血管炎(称为renal-limited MPA)是临床常见的原发性肾小血管炎,因多数患者血清抗中性粒细胞胞浆抗体(ANCA)阳性而称为ANCA相关性血管炎.     

以游走性肺内阴影为首发表现的大动脉炎4例报告并文献复习

<正>血管炎是一组以血管炎症及破坏为主要病理改变的异质性疾病[1]。大血管炎(大动脉炎、巨细胞动脉炎)、白塞病(大、中、小血管均受累)以及抗中性粒细胞胞浆抗体(ANCA)相关小血管炎(肉芽肿性多血管炎、嗜酸性肉芽肿性多血管炎及显微镜下多血管炎)等均可累及肺[1-4]。其中,大动脉炎或者白塞病等可以累及肺动脉主干及叶段肺动脉等主要分支,临床表现及影像表现复杂多样、缺乏特异性,     

抗中性粒细胞胞质抗体相关性小血管炎发病机制的研究进展

抗中性粒细胞胞质抗体相关性小血管炎是指以微小动脉、毛细血管、小静脉等小血管管壁的炎症和纤维素样坏死为病理特征的一组系统性疾病。目前发现的原发性小血管炎主要有以下几类：韦格纳肉芽肿病（WG）、显微镜下多血管炎（MPA）、变应性肉芽肿性血管炎（CSS）、节段坏死性新月体性肾炎（NCGN）。而抗中性粒细胞胞浆抗体（ANCA）则是一种以中性粒细胞和单核细胞胞浆成分为靶抗原的自身抗体。     

肺小血管炎的诊治

肺小血管炎是全身性、系统性坏死性血管炎 (ｓｙｓｔｅｍｉｃｎｅｃｒｏｔｉｚｉｎｇｖａｓｃｕｌｉｔｉｓ ,ＳＮＶ)累及肺脏血管的一组疾病。随着抗中性粒细胞胞浆抗体 (ＡＮＣＡ)的发现及应用 ,对这类疾患有了更深入的认识。这类疾病本质上归属于风湿免疫类疾患 ,多累及全身多个脏器。肺脏是这组疾患的重要靶器官 ,可以孤立侵犯肺 ,也可同时或先后侵犯其它多个器官。临床表现复杂多变 ,属于典型的跨学科疾病 ,极易引起误诊和漏诊 ,本文主要从肺脏损害的角度阐述血管炎诊断治疗的有关问题。1.肺血管炎的定义、分类及病理改变　坏死性血管炎…     

老年人抗中性粒细胞胞浆抗体相关小血管炎肾损害

原发性小血管炎是指一组病因不明、以血管壁炎症和纤维素样坏死为病理特征的系统性疾病,部分与抗中性粒细胞胞浆抗体(ANCA)密切相关,称之为ANCA相关小血管炎(AAV),包括韦格纳肉芽肿病(WG)、显微镜下型多血管炎(MPA)、变应性肉芽肿性血管炎和肾脏局限型血管炎.     

加强抗中性粒细胞胞浆抗体检测方法的规范化及合理应用

加强抗中性粒细胞胞浆抗体检测方法的规范化及合理应用赵明辉章友康王海燕抗中性粒细胞胞浆抗体（ＡＮＣＡ）是近１０年来发展起来的，用于诊断原发性小血管炎的极为重要的血清学诊断工具。与ＡＮＣＡ相关的小血管炎主要指韦格纳肉芽肿（ＷＧ）、显微镜下型多血管炎（ＭＰ...     

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原发性小血管炎是以血管壁的炎症和纤维素样坏死为病理特征的一组自身免疫性疾病.其中韦格纳肉芽肿病(WG)和显微镜下型多血管炎(MPA)与抗中性粒细胞胞浆抗体(ANCA)密切相关,故又称为ANCA相关系统性血管炎(AASV).     

丙基硫氧嘧啶与抗中性粒细胞胞浆抗体相关小血管炎

抗中性粒细胞胞浆抗体 (ａｎｔｉｎｅｕｔｒｏｐｈｉｌｃｙｔｏｐｌａｓｍｉｃａｎｔｉｂｏｄｉｅｓ,ＡＮＣＡ)是一种以中性粒细胞和单核细胞为靶抗原的自身抗体 ,是近 10年来发展起来的一种用于原发性小血管炎的特异性血清学诊断工具。ＡＮＣＡ相关的原发性小血管炎主要指韦格纳肉芽肿病、显微镜下型多血管炎、变应性肉芽肿血管炎和原发性局灶坏死性肾小球肾炎〔1〕。间接免疫荧光法 (ＩＩＦ)可将ＡＮＣＡ分为二种类型 :核周型称为ｐ ＡＮＣＡ ,胞浆型称为ｃ ＡＮＣＡ。ｃ ＡＮＣＡ和韦格纳肉芽肿病密切相关 ,其特异性靶抗原是蛋白酶 3(Ｐ…     

韦格纳肉芽肿合并2型糖尿病1例

<正>韦格纳肉芽肿是一种多系统坏死性肉芽肿性血管炎的自身免疫性疾病。其病因与抗中性粒细胞胞浆抗体(ANCA)相关,病变主要累及于小动脉、静脉及毛细血管,偶尔累及大动脉,其病理以血管壁的炎症为特征,通常以鼻黏膜和肺组织的局灶性肉芽肿性炎症为开始,继而进展为血管的弥漫性坏死性肉芽肿性炎症。韦格纳肉芽肿的报道相对较少,本文报道2014年我院韦格纳肉芽肿合并糖尿病1例,进行临床讨论。     

肺部原发性小血管炎的临床表现和诊断

目的 研究肺脏受累的原发性小血管炎的临床特点和早期诊断要点，减少其误诊率。方法 对26例原发性小血管炎伴肺脏受累患者的临床资料进行回顾性分析。结果 26例中，显微镜下多血管炎(MPA)15例，韦格纳肉芽肿(WG)9例，变应性肉芽肿性血管炎(CCS)2例。抗中性粒细胞胞浆抗体(ANCA)阳性率61．5％。肺脏受累的临床表现有咯血、呼吸困难、干咳、胸痛、胸闷等，胸部X线多表现为多发斑片状或点状阴影、大片高密度阴影或团块影、结节影、网格状影，可有空洞形成。疾病早期多误诊为肺部感染、肿瘤、肺间质病变等。应用肾上腺皮质激素和环磷酰胺治疗肺部病变可明显吸收，临床危重症得以改善。结论 伴肺脏受累的原发性小血管炎临床表现复杂多样，诊断困难，误诊率高，易延误病情，须引起临床医师的重视。     

Update in the diagnosis and management of pulmonary vasculitis

The term vasculitis encompasses a number of distinct clinicopathologic disease entities, each of which is characterized pathologically by cellular inflammation and destruction of the blood vessel wall, and clinically by the types and locations of the affected vessels. While multiple classification schemes have been proposed to categorize and simplify the approach to these diseases, ultimately their diagnosis rests on the identification of particular patterns of clinical, radiologic, laboratory, and pathologic features. While lung involvement is most commonly seen with the primary idiopathic, small-vessel or antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides of Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome, one should remember that medium-vessel vasculitis (ie, classic polyarteritis nodosa), large-vessel vasculitis (ie, Takayasu arteritis), primary immune complex-mediated vasculitis (ie, Goodpasture syndrome), and secondary vasculitis (ie, systemic lupus erythematosus) can all affect the lung. However, for the purpose of this review, we will focus on the ANCA-associated vasculitides.     

Pathogenesis of pulmonary vasculitis

Vasculitis is inflammation of blood vessels and can affect any type of vessel in any organ. Pulmonary vasculitis usually is a component of a systemic small vessel vasculitis. Three major forms of small vessel vasculitis that often affect the lungs are Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. These forms of vasculitis are strongly associated with antineutrophil cytoplasmic autoantibodies (ANCA) directed against enzymes contained in the primary granules of neutrophils and peroxidase-positive lysosomes of monocytes. This review discusses the evidence for a pathogenic role of ANCA. In vitro, ANCAs can activate cytokine-primed neutrophils and monocytes resulting in oxygen radical formation and release of lysosomal enzymes. In vivo, antimyeloperoxidase ANCA has been shown to induce crescentic glomerulonephritis and systemic vasculitis. Overall, the available data suggest that ANCA are indeed a pathogenic factor in the development of small-vessel vasculitis. Antiglomerular basement membrane (anti-GBM) disease also causes pulmonary vasculitis through immune attack on alveolar capillaries and glomerulonephritis through antibody mediated injury to glomerular capillaries. Thus, there is evidence that antibodies are important pathogenic factors in both ANCA disease and anti-GBM disease, however, there are also indications that T cells may play important pathogenic roles in both categories of disease as well.     

Rapidly progressive ANCA positive glomerulonephritis as the presenting feature of infectious endocarditis

The association of positive cytoplasmic antineutrophil antibody (ANCA) necrotizing crescentic glomerulonephritis with endocarditis raises diagnostic issues. Indeed, it is often difficult to determine if the kidney injury is either secondary to an infectious disease or caused by an ANCA-associated small vessel vasculitis. We report a 59-year-old man admitted in nephrology for acute glomerular syndrome in whom the renal biopsy showed a crescentic necrotizing glomerulonephritis. A diagnosis of vasculitis was initially considered in the presence of high titer of ANCA (anti-proteinase 3). Because of associated Staphyloccocus aureus endocarditis the patient received both corticosteroids and antibiotics that allowed remission of both kidney injury and endocarditis. The renal presentation and the disappearance of ANCA support the infectious etiology of this glomerulonephritis rather than an ANCA-associated small vessel vasculitis. It is important to be cautious in the presence of ANCA positive extracapillary glomerulonephritis and endocarditis should be ruled out before initiation of corticosteroids that may be nevertheless necessary in severe acute glomerulonephritis.     

Glomérulonéphrite rapidement progressive à ANCA révélant une endocardite infectieuse subaiguë

The association of positive cytoplasmic antineutrophil antibody (ANCA) necrotizing crescentic glomerulonephritis with endocarditis raises diagnostic issues. Indeed, it is often difficult to determine if the kidney injury is either secondary to an infectious disease or caused by an ANCA-associated small vessel vasculitis. We report a 59-year-old man admitted in nephrology for acute glomerular syndrome in whom the renal biopsy showed a crescentic necrotizing glomerulonephritis. A diagnosis of vasculitis was initially considered in the presence of high titer of ANCA (anti-proteinase 3). Because of associated Staphyloccocus aureus endocarditis the patient received both corticosteroids and antibiotics that allowed remission of both kidney injury and endocarditis. The renal presentation and the disappearance of ANCA support the infectious etiology of this glomerulonephritis rather than an ANCA-associated small vessel vasculitis. It is important to be cautious in the presence of ANCA positive extracapillary glomerulonephritis and endocarditis should be ruled out before initiation of corticosteroids that may be nevertheless necessary in severe acute glomerulonephritis.     

肺血管炎的临床与影像学特点解析

肺部血管炎包括原发性与继发性两大类.继发性血管炎包括感染性疾病、结缔组织病、恶性肿瘤和过敏性疾病所致肺血管炎.原发性血管炎的分类通常根据受累血管的大小分为大血管炎、中血管炎和小血管炎.肺部血管受累常见于原发性大血管炎[大动脉炎(Takayasu arteritis),巨细胞动脉炎(giant cell arteritis,GCA),白塞病(Behcetdisease)]和原发性抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic antibody,ANCA)相关性小血管炎[肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA),显微镜下多血管炎(microscopic polyangiitis),嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)].原发性肺血管炎的影像学表现极具多样性,包括血管壁增厚、结节影、空洞、磨玻璃影和实变影等.原发性肺部小血管炎常导致弥漫性肺泡出血(diffuse alveolar hemorrhage,DAH).相比于胸片,胸部CT更能够显示肺血管炎的病变特征和侵及范围.肺部血管炎的诊断极具挑战性,需要通过患者的临床特征、影像学特点、实验室检查结果和组织病理学特征作出综合判断.     

原发性小血管炎52例临床特点分析

目的 提高对原发性小血管炎临床特点和诊断方法的认识.方法 对我院2004年1月～2010年1月确诊的52例原发性小血管炎患者的症状、胸部影像学表现、实验室检查、病理检查结果等进行回顾性分析.结果 52例患者中显微镜下多血管炎33例,韦格纳氏肉芽肿病9例,变应性肉芽肿性血管炎10例;肺脏和肾脏受累最为明显,分别为80.77%和69.23%;抗中性粒细胞胞浆抗体阳性37例（71.15%）.应用糖皮质激素和环磷酰胺治疗后好转46例,放弃治疗自动出院5例,死亡1例.结论 原发性小血管炎临床表现复杂多样,诊断困难,误诊率高,应引起临床医师的重视.     

Churg-Strauss Syndrome: An Update

Churg-Strauss syndrome is an uncommon disease of unknown cause described initially by Churg and Strauss in 1951. Even though it was initially thought to be a variant of polyarteritis nodosa, its pathological, clinical, and laboratory features show that it is related to the small vessel vasculitides, and it is now classified as an antineutrophil cytoplasmic antibody-associated vasculitis. The presence of asthma, usually of adult onset, along with other allergic symptoms, peripheral and tissue eosinophilia, is specific to this disease. These features usually help clinicians distinguish it from other types of small vessel vasculitis and should alert clinicians about its presence. Two different clinical subtypes defined by the presence of antineutrophil cytoplasmic antibodies recently have been recognized. Recent advances in the treatment and pathophysiology of Churg-Strauss syndrome are reviewed in this article.     

Diffuse alveolar hemorrhage and pulmonary capillaritis due to propylthiouracil

Propylthiouracil (PTU) has recently been observed to be associated with antineutrophil cytoplasmic antibody (ANCA)-positive small vessel vasculitis, resulting in crescentic glomerulonephritis and, infrequently, diffuse alveolar hemorrhage (DAH). We describe a case of a 23-year-old pregnant woman who developed a perinuclear ANCA and antimyeloperoxidase-positive small vessel vasculitis manifesting as DAH and crescentic glomerulonephritis after she began taking PTU. An open lung biopsy was consistent with pulmonary capillaritis. She responded to corticosteroid therapy and discontinuation of PTU. DAH can be caused by pulmonary capillaritis, bland hemorrhage, or diffuse alveolar damage. To our knowledge, this represents the first documentation of an underlying pulmonary capillaritis in a case of PTU-induced DAH.     

Takayasu arteritis: A distinct syndrome of large vessel vasculitis: A view point by late Professor Paul Bacon

Takayasu arteritis (TA), despite being classified as a large vessel vasculitis, has distinct genetic, pathological and clinical features as compared to giant cell arteritis. It is a rare disease seen more commonly in Asian countries. The challenge lies in assessing the degree of inflammation in a narrowed vessel and immunosuppressive therapy improves inflammatory features but is unable to open up an occluded vessel. It may have a positive effect on retarding further occlusion. Like antineutrophil cytoplasmic antibody‐associated vasculitis, TA needs a collaborative effort to do randomized controlled therapy to provide benefit to patients.