丙基硫氧嘧啶致抗中性粒细胞胞浆抗体相关小血管炎3例报告

目的分析丙基硫氧嘧啶(PTU)致抗中性粒细胞胞浆抗体(ANCA)相关小血管炎的机理。方法报告3例因长期服用丙基硫氧嘧啶所致ANCA相关小血管炎的临床资料,并复习近年相关文献加以讨论。结果 3例的临床表现主要是血尿、蛋白尿、肾功能受损、累及肺部和ANCA阳性等;3例发病前均服用PTU,故诊为PTU导致的ANCA相关小血管炎;3例均经停用PTU,给予激素和免疫抑制剂治疗症状明显好转,肾功能得到恢复。结论 PTU可导致ANCA相关小血管炎。甲亢病人使用PTU治疗时,特别是长时间、大剂量使用时,应定期进行ANCA检查;早期治疗是改善预后的关键     

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…     

丙基硫氧嘧啶致抗中性粒细胞胞质抗体阳性小血管炎二例

丙基硫氧嘧啶 (PTU)是硫脲类抗甲状腺药 ,是临床最常用的抗甲状腺功能亢进药物之一。抗中性粒细胞胞质抗体(ANCA)是原发性小血管炎的血清学诊断工具。近年来发现有少数服用PTU的甲状腺功能亢进患者临床上出现了小血管炎的表现 ,并且在这些患者血清中也可检测到ANCA。现将我院近年发现的 2例PTU相关的ANCA阳性小血管炎报道如下。例 1 :女性 ,6 0岁。因皮疹、关节痛半年 ,泡沫尿 1个月入院。患者 3年前查血小板减低 ,骨髓细胞学示“巨核细胞增多伴成熟障碍” ,查自身抗体均阴性 ,临床诊断为特发性血小板减少性紫癜 (ITP)。予环孢菌…     

丙基硫氧嘧啶致ANCA阳性相关小血管炎7例临床分析

丙基硫氧嘧啶（PTU）是硫脲类抗甲状腺药,为临床最常用的抗甲状腺药物之一.抗中性粒细胞胞质抗体（ANCA）是一种以中性粒细胞和单核细胞为靶抗原的自身抗体,是可用于小血管炎检测的特异性血清学诊断标记.我院近8年发现7例PTU所致的ANCA阳性小血管炎报道如下：     

抗中性粒细胞胞浆抗体相关性小血管炎1例并文献复习

抗中性粒细胞胞浆抗体（anti-neutrophil cytoplasmic antibody,ANCA）相关性小血管炎（ANCA associated systemic vasculitis,AASV）是一组以ANCA阳性为突出临床特征,累及多个系统的血管炎性疾病。因其临床表现缺乏特异性,易被漏诊、误诊。现将我科收治的1例以呕吐为首发表现的AASV患者的临床特点进行总结和分析。     

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。     

丙基硫氧嘧啶诱发抗中性粒细胞胞质抗体阳性小血管炎三例并文献复习

目的研究丙基硫氧嘧啶(PTU)诱发的抗中性粒细胞胞质抗体(ANCA)阳性小血管炎的临床病理表现。方法对我院近2年诊治的3例FPU引起的ANCA阳性小血管炎进行临床病理分析并复习相关文献。结果3例病人均为女性．年龄24～32岁．平均28．3岁。服PTU时间2～17年．3例均出现皮疹、关节痛．但不累及肾、脑、肺、造血等重要脏器，均为核周型ANCA(pANCA)．髓过氧化物酶ANCA(MPO-ANCA)显著增高(90～260EU／ml)，1例并胞质型ANCA．2例抗甲状腺球蛋白抗体升高，1例抗核抗体(ANA)阳性，而同期服DTU、他巴唑(MMI)无小血管炎临床表现的格雷夫斯病血清14份、31份pANCA均阴性，PTU组有2例MPO-ANCA低水平增高(20～26EU／ml)。3例患者均立即停用PTU，改服MMI、甲亢平各1例，同时应用糖皮质激素，临床症状均缓解，各种自身抗体滴度降低。结论PTU可诱发ANCA阳性小血管炎，应及时发现停药，糖皮质激素治疗有效。     

抗中性粒细胞胞浆抗体相关性小血管炎诊断和治疗几点体会

抗中性粒细胞胞浆抗体（ANCA）相关小血管炎属系统性自身免疫性疾病，肾脏是常见的受累器官，随着我国ANCA检测的普及，该病的检出率也逐年增高，北京大学肾脏病研究所2003年新检出ANCA阳性患者138例，说明该病在我国并不少见，然而，国内对此病的诊治水平在不同地区之间还存在较大偏差，仍存在严重的误漏诊和治疗不当，下面介绍几点体会。     

Propylthiouracil-induced antineutrophil cytoplasmic antibody-associated vasculitis

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) refers to a group of potentially life-threatening autoimmune diseases. A recent development in this field is the recognition that certain drugs can induce AAV. Among these agents, the drug most often implicated in causing disease is the commonly used antithyroid agent propylthiouracil (PTU). This Review provides an update on PTU-induced AAV. Clinical characteristics of PTU-induced AAV are similar to that of primary AAV, but usually have a milder course and better prognosis, provided early cessation of the disease-causing drug. PTU-induced ANCAs usually react to several components of myeloid granules, which is helpful in differentiating PTU-induced AAV from primary AAV. Early cessation of PTU is crucial in the treatment of PTU-induced AAV. The duration of immunosuppressive therapy might be shorter than in primary AAV, depending on the severity of organ damage, and maintenance therapy is not always necessary.     

Antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis

PURPOSE OF REVIEW: This review focuses on recent advances in the diagnosis, pathogenesis and treatment of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis. RECENT FINDINGS: Antineutrophil cytoplasmic autoantibodies are closely associated with Wegener's granulomatosis and microscopic polyangiitis. Within the Churg-Strauss syndrome, antineutrophil cytoplasmic autoantibodies, mostly directed towards myeloperoxidase, characterize patients with glomerulonephritis and small-vessel vasculitis. There is increasing evidence that myeloperoxidase-antineutrophil cytoplasmic autoantibodies are directly involved in the pathogenesis of necrotizing vasculitis. This is less clear for proteinase 3-antineutrophil cytoplasmic autoantibodies, markers for Wegener's granulomatosis. With respect to proteinase 3-antineutrophil cytoplasmic autoantibodies, complementary proteinase 3, a peptide translated from the antisense DNA strand of proteinase 3 and homologous to several microbial peptides, may be involved in induction of proteinase 3-antineutrophil cytoplasmic autoantibodies. Currently, various controlled trials have been initiated. Methotrexate has been shown to be effective for induction of remission in locoregional Wegener's granulomatosis. Other trials are underway. SUMMARY: Apart from its diagnostic potential, antineutrophil cytoplasmic autoantibodies, particularly myeloperoxidase-antineutrophil cytoplasmic autoantibodies, are directly involved in the pathogenesis of the associated vasculitides. New treatment modalities, supposedly more efficacious and less toxic than daily oral cyclophosphamide, are being tested in randomized controlled trials.     

Systemic sclerosis and antineutrophil cytoplasmic autoantibody-associated renal failure

We report three patients who developed antineutrophil cytoplasmic autoantibody (ANCA)-associated crescentic glomerulonephritis, two of whom showed clinical features of limited scleroderma and one whose results of serological tests were suggestive of limited scleroderma without cutaneous features. All had anticentromere antibodies and antimyeloperoxidase antibodies. No patient showed the features of typical scleroderma renal crisis such as accelerated hypertension or microangiopathy. Our patients were normotensive at the time of onset of renal failure, and the clinical picture was characterised by only modest features of limited scleroderma. All three patients had crescentic glomerulonephritis at various stages of chronicity. One patient responded to immunosuppressive therapy with improvement in renal function; the other two patients rapidly developed end-stage renal failure. These patients and others recently described may represent a newly described form of scleroderma renal disease. Received: 12 January 1999 / Accepted: 3 May 1999     

Cytapheresis for the treatment of myeloperoxidase antineutrophil cytoplasmic autoantibody-associated vasculitis: a pilot study of 21 patients

Twenty-one patients with myeloperoxidase-antineutrophil cytoplasmic autoantibody (MPO-ANCA)-associated vasculitis were treated using cytapheresis. Of these, 17 were treated for glomerulonephritis and four were treated for pulmonary hemorrhage. The overall survival rate was 85.7% with a follow-up duration of 24.0 +/- 13.8 months. In the 17 patients with MPO-ANCA-associated glomerulonephritis, pretreatment creatinine was 3.2 +/- 1.6 mg/dL, and renal function recovered in 76.5%. Pulmonary hemorrhage was ameliorated in all four patients. Abdominal pain occurred in three of the 21 patients but symptoms resolved soon after the cytapheresis procedure was completed. No other adverse effects occurred during cytapheresis. From these results, cytapheresis can be considered a safe and effective treatment for MPO-ANCA-associated vasculitis. As for the mechanism of its action, soluble tumor necrosis factor receptor 1 (sTNFR), sTNFR2 and interleukin 1 receptor antagonist were elevated soon after cytapheresis and those levels 2 h after the cytapheresis procedure were higher than before the procedure in some cases. These elevations might be related to the efficacy of cytapheresis.     

Antineutrophil cytoplasmic autoantibody-associated vasculitis in older patients

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is increasingly recognized in older patients. The differences in disease presentation and outcome between older and younger patients remain controversial. We conducted the current study to analyze the characteristics of patients aged over 65 years with AAV and to compare the younger and older cohorts. We recruited 234 consecutive Chinese patients with AAV. We compared clinical and pathologic characteristics as well as outcomes between younger and older patients. Among the 234 patients with AAV, 99 were older than 65 years. Compared with the 135 younger patients, the older patients had a significantly higher proportion of positive myeloperoxidase-ANCA (94.9% vs. 80.0%, p < 0.01) and a higher proportion of microscopic polyangiitis (79.8% vs. 50.4%, chi = 11.8, p < 0.001), but had a lower proportion of Wegener granulomatosis (18.2% vs. 37.8%, p < 0.01) and renal-limited vasculitis (0% vs. 11.1%, p < 0.001).Older patients had more prevalent and severe pulmonary involvement than younger patients, including pulmonary infiltration, interstitial fibrosis, and mechanical ventilation dependence at presentation (47.5% vs. 31.9%, p < 0.05; 37.4% vs. 18.5%, p < 0.01; and 9.1% vs. 1.5%, p < 0.05, respectively). Older patients were less likely to respond to treatment (p < 0.01) and had worse survival than younger patients (p = 0.000). During follow-up, older patients had a higher risk of secondary pulmonary infection (p < 0.001), and those with pulmonary interstitial fibrosis were more likely to develop secondary pulmonary infections (p < 0.05). In conclusion, compared with younger patients, older patients with AAV had more severe and more prevalent pulmonary lesions, which might contribute to subsequent pulmonary infections after the initiation of immunosuppressive therapy. Age and pulmonary infection were independent predictors of death.     

Relapses in patients with antineutrophil cytoplasmic autoantibody-associated vasculitis: likely to begin with the same organ as initial onset

OBJECTIVE: In antineutrophil cytoplasmic antibody-associated vasculitis (AAV), relapses constitute a challenge despite initial good control. Our objective was to investigate whether patients with relapses shared the same initial organ involvement. METHODS: One hundred sixty patients with AAV in our center were investigated. Of these 160, 38 experienced relapse during followup. Clinical and laboratory data were analyzed. RESULTS: Among the 38 patients, there was a total of 55 relapse events, 39 (70.9%) of which had the same initial organ as in the initial onset. CONCLUSION: Relapses in AAV were likely to begin with the same organ as in the initial onset; this facilitates early recognition of relapses.     

抗中性粒细胞胞浆抗体相关血管炎的治疗

抗中性粒细胞胞浆抗体（ANCA）相关血管炎是成人最常见的原发性系统性小血管炎,多器官系统受累,临床表 现高度异质性,未早期有效治疗患者近期死亡率高。近年来随着对其发病机制的深入了解,可选择治疗药物和治疗 策略也在不断发展。需要根据患者临床-病理分型、受累器官、疾病活动性和严重性评价、ANCA血清型进行多维度 评价,并依据现有的循证医学证据,个体化地选择治疗方案。