老年人抗中性粒细胞胞浆抗体相关性小血管炎的临床表现

目的　为了解老年人抗中性粒细胞胞浆抗体 (ANCA)相关性小血管炎所引起的多器官损害的临床表现。方法　分析总结近 5年来检出的 1 4 5例 60岁以上老年ANCA相关性小血管炎患者的临床病理资料。结果　1 4 5例患者 ,男 74例 ,女 71例 ,平均年龄 68.2岁。胞浆型ANCA(c ANCA)阳性 1 7例 ,均识别蛋白酶 3(PR3) ;环核型ANCA (p ANCA)阳性 1 2 8例 ,其中 1 2 0 1 2 8例单独识别髓过氧化物酶 (MPO) ,8 1 2 8例同时识别PR3和MPO。不足 1 2的患者在 3个月内确诊。确诊病例均呈多器官损害 ,其中肾脏受累占 93 .8% ,且 75 %出现肾功能不全 ;肺脏受累占 74.5 % ,半数以上表现为肺出血和 (或 )肺部阴影 ,此外还有不同程度的消化道、神经系统、肌肉关节、眼、耳器官或组织损害及发热、乏力、消瘦等非特异症状。实验室检查 ,90 %以上的患者有中重度贫血、血沉增快和C反应蛋白升高。结论　老年人ANCA阳性小血管炎较为多见 ,临床表现为多器官损害 ,ANCA检测有助于诊断     

丙硫氧嘧啶导致的抗中性粒细胞胞浆抗体相关性血管炎的临床研究

目的提高临床对丙硫氧嘧啶（PTU）引起抗中性粒细胞胞浆抗体（ANCA）相关性血管炎的认识。方法分析近年诊治的6例刖引起ANCA相关小血管炎患者的临床表现、实验室检查、病理检查、治疗及随访情况,结合文献及联系显微镜下多血管炎（MPA）的临床特点进行分析。结果6例服用PITU的患者出现肺、肾、皮肤及消化道等多脏器受累,其中5例抗MPO—ANCA阳性,1例抗PR3-ANCA阳性。停用PTU,使用激素或联合免疫抑制剂治疗后病情好转,ANCA转阴。结论刑可引起ANCA相关小血管炎,尽管ANCA滴度高,但致病性较弱,及时停用PITU及给予相应治疗,预后较好。     

肺脏受累的原发性抗中性粒细胞胞浆抗体相关性小血管炎43例临床分析

目的 探讨肺脏受累的原发性抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎患者的临床特征,为肺部受累的原发性ANCA相关性小血管炎的诊断提供帮助.方法 回顾性分析2009年3月至2013年9月在湘雅二医院住院的符合2012年美国Chapel Hill会议关于系统性小血管炎诊断标准,并血清ANCA阳性的43例肺脏受累的的原发性ANCA相关性小血管炎患者的临床资料.结果 43例患者中,肺部症状首发就诊者22例,常见肺部症状依次为咳嗽咯痰、活动后气促、咯血、哮喘等,肺外受累器官依次为肾脏、神经系统、眼、鼻;肺外症状首发就诊者21例,肾脏为最常见受累器官;显微镜下多血管炎（MPA） 34例,其中抗髓过氧化物酶（MPO）抗体（P-ANCA）阳性率97.1％（33/34）,抗蛋白酶3（PR3）抗体（C-ANCA）阳性率2.9％ （1/34）;肉芽肿性多血管炎（GPA）（韦格纳肉芽肿）8例,抗蛋白酶3（PR3）抗体（C-ANCA）阳性率62.5％（5/8）,抗髓过氧化物酶（MPO）抗体（P-ANCA）阳性率为37.5％（3/8）;嗜酸细胞性肉芽肿性多血管炎（EGPA）1例,为抗髓过氧化物酶（MPO）抗体阳性;胸部影像学表现多为双肺间质病变,如双肺网格样改变、磨玻璃影、蜂窝肺、多发条索状及结节性病变等,或为条索或斑片状病变、支气管扩张、胸腔积液及肿块病变等.经激素和免疫抑制剂治疗多数患者病情可缓解,19例患者（44.2％）于住院及随访期间因血管炎活动并肺部感染、大咯血及肾功能衰竭等原因死亡.结论 肺脏受累的原发性ANCA相关性小血管炎临床表现无特异性,多数患者合并有肾脏受累,影像学多为肺间质病变,具有提示诊断价值,血清ANCA检查有特殊诊断价值,此类患者死亡率高,多为疾病活动并感染致死,应积极控制感染并合理采用抑制免疫治疗.     

误诊为肺部疾病的ANCA相关性小血管炎临床分析

目的对误诊为肺部疾病的抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎临床特征及其误诊原因进行分析。方法分析总结我院5例确诊ANCA相关性小血管炎的临床资料。结果5例患者,男3例,女2例,年龄15—65岁,C-ANCA阳性1例,P—ANCA阳性4例,其中1例C-ANCA,P-ANCA均阳性。诊病明确前曾误诊为肺炎2例,间质性肺炎1例,肺结核2例。ANCA对其确诊有重要意义。结论ANCA相关性小血管炎临床表现为首发肺部症状、多器官功能损害,误诊率高。ANCA检测有助于早期正确诊断,早期治疗。     

以肾损害为首发的ANCA相关性小血管炎误诊20例

抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎是一种主要累及小血管的系统性血管炎,常见受累器官为肾脏与肺。本病好发于中老年,临床表现多样,易被误诊。本文将我院近6年来收治的20例以肾损害为首发的ANCA相关性小血管炎患者误诊原因进行分析。     

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相关性系统性小血管炎的临床表现及诊断

抗中性粒细胞胞浆抗体 (ANCA)相关性系统性小血管炎 (AASV)是成人最常见系统性小血管炎。初略估计年发病率为 2 0 /百万。北大医院ANCA阳性的检出率为送检血清的 5 % ,提示该疾病在我国已不是少见病。在过去的几年里 ,在临床诊治方面已经有了较大的进步 ,但许多问题仍然悬而未决。由于受累是系统性 ,故临床表现呈现多样性 ,只有对受累器官特殊的临床表现、ANCA存在类型、血浆冷球蛋白存在与否有较深刻的认识 ,才可以帮助诊断并区分特殊类型血管炎。不同类型的小血管炎的分类显微镜下多血管炎 (MAP)　是最常见的一种ANCA相关性血管…     

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。     

咳嗽及肺部游走性阴影为主要表现的抗中性粒细胞胞浆抗体相关性血管炎1例

笔者成功诊治1例65岁男性抗中性粒细胞胞浆抗体（ANCA）相关性血管炎患者。该患者主要表现为慢性咳嗽和肺部游走性阴影,曾被诊断为淋巴细胞性间质性肺炎,激素治疗有效,病情复发后查MPO—ANCA阳性,结合肺穿刺活检明确诊断为ANCA相关性血管炎,加用糖皮质激素和环磷酰胺后病情再次缓解。根据文献报道和笔者的临床经验,ANCA相关性血管炎临床表现复杂,缺乏特异性,常被误诊。呼吸科医师对ANCA相关性血管炎肺部受累特点应熟悉,要重视血清ANCA的筛查,以便于本病的早期诊断。     

以肺部表现首发的抗中性粒细胞胞浆抗体相关性小血管炎临床分析

目的探讨以肺部表现首发的抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎的临床特征。方法回顾分析10例以肺部症状首发的ANCA相关性小血管炎患者的临床资料。结果10例患者均有肺病变,肾脏损害5例,皮肤损害1例。所有患者ANCA阳性。双肺病变8例,单侧肺部病变2例。大部分患者可见血白细胞升高、贫血、血沉明显增快、C反应蛋白升高等。应用糖皮质激素及免疫抑制剂治疗,死亡3例,完全缓解7例。结论ANCA相关性小血管炎临床表现缺乏特异性,ANCA检查有助于该病的早期诊断。糖皮质激素及免疫抑制剂治疗能改善预后。     

Antineutrophil cytoplasmic autoantibodies in systemic lupus erythematosus

Although antineutrophil cytoplasmic antibodies (ANCA) were first associated with the primary vasculitides, it is now clear that 15-20% of patients with lupus have detectable ANCA. In this short review we confirm that the major link is with perinuclear ANCA (pANCA) but not cytoplasmic ANCA (cANCA). ANCA to myeloperoxidase are associated with drug-induced lupus. There may be a link between pANCA levels and disease activity in some patients although the links to specific organ involvement are not proven. ANCA in lupus must be interpreted cautiously with particular attention paid to laboratory technique, the size, age and genetic background of the populations studied.     

Prevalence and spectrum of rheumatic diseases associated with proteinase 3-antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA

OBJECTIVES: To evaluate the prevalence and association of antineutrophil cytoplasmic antibodies (ANCA) and their subtypes [proteinase 3 (PR3)-ANCA, myeloperoxidase (MPO)-ANCA] with distinct clinical features in various clinicopathological syndromes. METHODS: All consecutive ANCA-positive patients seen at the combined unit for rheumatology for Bad Bramstedt and the University of Lübeck between 1989 and 1999 were analysed. ANCA were detected by an immunofluorescence technique and ANCA subspecificities were determined by ELISA. Clinical features at presentation and diagnoses were recorded according to standardized procedures. RESULTS: Among 4620 patients tested, 333 were cytoplasmic ANCA-positive and 291 were perinuclear ANCA-positive. cANCA/PR3-ANCA were strongly associated with Wegener's granulomatosis (WG), whereas pANCA/MPO-ANCA were associated with a diverse disease spectrum. Further investigation of PR3-ANCA-positive (n=80) and MPO-ANCA-positive patients (n=40) revealed a greater extent of disease [disease extent index (DEI); median 8 vs 5, P<0.01] and more frequent involvement of the upper/lower respiratory tract and the eyes in PR3-ANCA-positive than in MPO-ANCA-positive patients. Fewer than 5% of WG patients were MPO-ANCA-positive. Compared with matched PR3-ANCA-positive WG patients, the MPO-ANCA-positive WG patients had a lower DEI (median 5 vs 8) and had a lower frequency of peripheral neuropathy. CONCLUSIONS: ANCA testing is useful due to its high sensitivity and specificity, especially for cANCA/PR3-ANCA in WG. We found a divergence in the disease spectrum between PR3- and MPO-ANCA-positive patients, characterized by higher DEI and extrarenal manifestations in the PR3-ANCA group. MPO-ANCA was rarely found in WG and was associated with less organ involvement.     

Antineutrophil cytoplasmic antibodies in systemic lupus erythematosus

Objective. To examine the prevalence, subspecificities, and clinical associations of antineutrophil cytoplasmic antibodies (ANCA) in patients with systemic lupus erythematosus (SLE). Methods. One hundred fifty-seven sera from 120 patients with SLE were examined for classic (c) and perinuclear (p) pattern ANCA by indirect immunofluorescence. Antibody subspecificities were determined by enzyme-linked immunosorbent assay (ELISA). Serologic results were correlated with clinical manifestations as categorized by the BILAG (British Isles Lupus Assessment Group) index. Results. ANCA were found in 40 of the 157 sera (25%). Only a pANCA, not a cANCA, pattern of fluorescence was seen. By ELISA testing, 16 sera reacted to lactoferrin, 8 to elastase, and 4 to lysozyme. There was no reactivity to proteinase 3 (PR3) or myeloperoxidase (MPO). No correlation of pANCA, or any of the ANCA subspecificities, with organ system involvement, as categorized by the BILAG index, was found. Notably, there was no correlation of ANCA results with lupus vasculitis. Conclusion. The absence of cANCA, anti-PR3, and anti-MPO shows that with appropriate assay conditions, ANCA testing assists in the differentiation between SLE and the ANCA-associated vasculitides. The lack of a correlation between pANCA or any ANCA subspecificity and clinical manifestations suggests that ANCA do not identify particular clinical subsets among SLE patients, including those with lupus vasculitis.     

Diagnostic performance of antineutrophil cytoplasmic antibody tests for idiopathic vasculitides: metaanalysis with a focus on antimyeloperoxidase antibodies

OBJECTIVE: The diagnostic value of tests for antimyeloperoxidase antibodies (anti-MPO) for systemic vasculitis is less established than that for cytoplasmic antineutrophil cytoplasmic antibody (cANCA)/antiproteinase 3 antibodies (anti-PR3). Controversy exists regarding the optimal utilization of indirect immunofluorescence (IIF) ANCA testing versus antigen-specific ANCA testing. To summarize the pertinent data, we conducted a metaanalysis examining the diagnostic value of ANCA testing systems that include assays for anti-MPO. METHODS: We performed a structured Medline search and reference list review. Target articles in the search strategy were those reporting the diagnostic value of immunoassays for anti-MPO for the spectrum of systemic necrotizing vasculitides that includes Wegener's granulomatosis, microscopic polyangiitis, the Churg-Strauss syndrome, and isolated pauci-immune necrotizing or crescentic glomerulonephritis, regardless of other types of ANCA tests. Inclusion criteria required specification of a consecutive or random patient selection method and the use of acceptable criteria for the diagnosis of vasculitis exclusive of ANCA test results. Weighted pooled summary estimates of sensitivity and specificity were calculated for anti-MPO alone, anti-MPO + perinuclear ANCA (pANCA), and anti-MPO/pANCA + anti-PR3/cANCA. RESULTS: Of 457 articles reviewed, only 7 met the selection criteria. Summary estimates of sensitivity and specificity (against disease controls only) of assays for anti-MPO for the diagnosis of systemic necrotizing vasculitides were 37.1% (confidence interval 26.6% to 47.6%) and 96.3% (CI 94.1% to 98.5%), respectively. When the pANCA pattern by IIF was combined with anti-MPO testing, the specificity improved to 99.4%, with a lower sensitivity, 31.5%. The combined ANCA testing system (anti-PR3/cANCA + anti-MPO/pANCA) increased the sensitivity to 85.5% with a specificity of 98.6%. CONCLUSION: These results suggest that while anti-MPO is relatively specific for the diagnosis of systemic vasculitis, the combination system of immunoassays for anti-MPO and IIF for pANCA is highly specific and both tests should be used together given the high diagnostic precision required for these conditions. Because patients with ANCA associated vasculitis have either anti-MPO with pANCA or anti-PR3 with cANCA, and rarely both, a combined ANCA testing system including anti-PR3/cANCA and anti-MPO/pANCA is recommended to optimize the diagnostic performance of ANCA testing.     

Autoantibodies and target antigens in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides

In this study of antineutrophil cytoplasmic antibody (ANCA)-associated diseases, we determined the prevalence of other autoantibodies and the antigen specificities of ANCA. ANA were common, occurring in 7 of 36 (19%) patients with Wegener's granulomatosis, in 16 of 34 (47%) patients with microscopic polyarteritis, in 6 of 11 (55%) patients with segmental necrotising glomerulonephritis and in 8 of 18 (44%) of those with ANCA-associated systemic vasculitis without renal involvement. ANA were associated more often with pANCA and microscopic polyarteritis than with cANCA (P<0.05). Patterns were speckled (n=23), homogeneous (n=10) or nucleolar (n=4). Anticardiolipin antibodies were also common, occurring in 10 of 25 (40%) patients with Wegener's granulomatosis, in 8 of 14 (57%) patients with microscopic polyarteritis and in 6 of 18 (33%) of those with a systemic vasculitis. However, anticardiolipin antibodies did not correlate with the presence of ANCA in any of the disease groups. Anti-GBM antibodies were demonstrated in only 2 of 25 (8%) patients with Wegener's granulomatosis, in I patient with microscopic polyarteritis (1/14, 7%) and in 1 with segmental nectrotising glomerulonephritis (1/11, 9%). All four patients with anti-GBM antibodies had either cANCA or pANCA. In the second part of the study, the target antigens of ANCA were determined in Wegener's granulomatosis, microscopic polyarteritis, systemic vasculids, inflammatory bowel disease, rheumatoid arthritis and systemic lupus erythematosus (SLE). Of the 19 sera with cANCA, 13 (68%) were directed against proteinase 3; other antigens were myeloperoxidase (1/19,5%), elastase and lactoferrin together (1/19, 5%), lysozyme (1/19, 5%) or unknown (3/19,16%). Of the 12 (58%) sera from patients with Wegener's granulomatosis who had cANCA, 7 bound to proteinase 3. Antimyeloperoxidase antibodies were present in 14 of 45 (31%) sera with pANCA; other antigens were proteinase 3 (5/45,11 %), elastase (3/45, 78%), lactoferrin (1/45, 2%), cathepsin G (5/45, 11 %) or unknown (17/45, 38%). Antimyeloperoxidase antibodies were common in microscopic polyarteritis (6/14, 43%) and systemic vasculitis (5/16, 31%). However, the majority of target antigens in systemic vasculitis and rheumatoid arthritis with pANCA were not determined. Atypical ANCA were present in four patients, one with inflammatory bowel disease (1/8, 13%) and three with SLE (3/15, 20%). The specificities were cathepsin G, cathepsin G plus lactoferrin, or unknown in two sera. A recent report has suggested that bactericidal /permeability-increasing protein may be the target in patients with inflammatory bowel disease.     

Evaluation of antineutrophil cytoplasmic antibody seroconversion induced by minocycline, sulfasalazine, or penicillamine

OBJECTIVE: Case reports have suggested that minocycline, sulfasalazine, and penicillamine are associated with antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis. This study evaluated ANCA seroconversion due to these agents in serum samples prospectively collected in randomized, double-blind, controlled trials. METHODS: The sources of study sera were 3 clinical trials: 1) a 48-week trial of minocycline for early rheumatoid arthritis, with 64 patients receiving minocycline compared with 68 receiving placebo; 2) a 37-week trial of sulfasalazine for rheumatoid arthritis, with 51 receiving sulfasalazine compared with 38 receiving placebo; and 3) a 104-week trial of penicillamine for early systemic sclerosis, with 15 undergoing high-dose penicillamine treatment versus 12 receiving low-dose penicillamine. ANCA were measured in the baseline and study-end serum samples by indirect immunofluorescence (IIF) for perinuclear ANCA (pANCA) and cytoplasmic ANCA (cANCA) patterns, and by antigen-specific enzyme-linked immunosorbent assay (ELISA) for antibodies to myeloperoxidase (anti-MPO) and proteinase 3 (anti-PR3). Laboratory personnel were blinded to the group identity of the samples. ANCA results were interpreted using an ANCA scoring system that combines the results of IIF and ELISA testing. RESULTS: No patient in any of the active study drug groups demonstrated ANCA seroconversion according to the final interpretation of the combined IIF and ELISA results. Twelve of the 248 patients (5%) were positive for anti-MPO with pANCA at baseline. No subject was positive for anti-PR3 with cANCA. There were no findings suggestive of vasculitis in any of these patients. CONCLUSION: From our study results, there was no suggestion of ANCA seroconversion induced by minocycline, sulfasalazine, or penicillamine. However, these findings do not rule out the possibility of rare, sporadic cases of either ANCA seroconversion or true drug-induced vasculitis with these drugs.     

ANTINUCLEAR AND ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (ANCA) IN THE SERA OF PATIENTS WITH FELTY'S SYNDROME

The prevalence of antinuclear (ANA) and antineutrophil cytoplasmicantibodies (ANCA) has been studied in the sera of 62 patientswith rheumatoid arthritis and 32 patients with Felty's syndrome.The presence of ANA was less in RA than Felty's syndrome (37%versus 69%). Specific autoantibody identification, where possible,was usually of SS-A or SS-B although two sera from patientswith Felty's syndrome had low levels of DNA antibody. ANCA wasdetected in the sera of 33% of patients with Felty's syndromeand was absent in RA sera. The pattern of ANCA staining waseither of a diffuse homogenous cytoplasmic or peripheral (pANCA)nature. Classical cytoplasmic granular staining (cANCA) wasnot identified. KEY WORDS: Antinuclear antibodies, Antineutrophil cytoplasmic antibodies (ANCA), Felty's syndrome     

Perinuclear antineutrophil cytoplasmic antibodies in patients with Crohn's disease define a clinical subgroup

BACKGROUND & AIMS: Antineutrophil cytoplasmic antibodies (ANCA) have been consistently detected in a subgroup of patients with Crohn's disease (CD). This study was designed to determine whether serum ANCA expression in patients with CD characterizes an identifiable clinical subgroup. METHODS: The study population consisted of 69 consecutive patients with an established diagnosis of CD as determined by a combination of characteristic clinical, radiographic, endoscopic, and histopathologic criteria. Sera from the patients were analyzed for the presence of ANCAs using the fixed neutrophil enzyme-linked immunosorbent assay (ELISA) assay. Perinuclear ANCA (pANCA)-positive and cytoplasmic ANCA (cANCA)-positive results by ELISA were confirmed by indirect immunofluorescence staining. Clinical profiles of the ANCA- positive patients with CD were compared with those of patients with CD not expressing ANCA (ANCA-negative). RESULTS: pANCA-positive patients with CD have endoscopically and/or histopathologically documented left- sided colitis and symptoms of left-sided colonic inflammation, clinically reflected by rectal bleeding and mucus discharge, urgency, and treatment with topical agents. One hundred percent of patients with CD expressing pANCA had "UC-like" features. CONCLUSIONS: In patients with CD, serum pANCA expression characterizes a UC-like clinical phenotype. Stratification of CD by serum pANCA provides evidence of heterogeneity within CD and suggests a common intestinal mucosal inflammatory process among a definable subgroup of patients with CD and UC expressing this marker. (Gastroenterology 1996 Jun;110(6):1810-9)     

Alternating antineutrophil cytoplasmic antibody specificity: drug-induced vasculitis in a patient with Wegener's granulomatosis

We describe a patient who presented with Wegener's granulomatosis associated with antineutrophil cytoplasmic antibodies (ANCA) directed against proteinase 3 (PR3) with a cytoplasmic immunofluorescence pattern (cANCA), whose ANCA type changed to antimyeloperoxidase antibodies with a perinuclear immunofluorescence pattern (pANCA) when treated with propylthiouracil, and changed back to anti-PR3 antibodies with cANCA after the medication was discontinued. The patient developed flares of vasculitis symptoms associated with rises in either type of ANCA. Tests for antimyeloperoxidase ANCA were repeatedly negative before the drug was started, strongly implicating the drug as the cause of the episode. This case demonstrates that patients with idiopathic ANCA-positive vasculitis may quickly develop a superimposed drug-associated ANCA-positive vasculitis. Iatrogenic vasculitis should be suspected when a patient with idiopathic vasculitis with one type of ANCA develops the other type of ANCA.     

Atypical, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibodies in patients with inflammatory bowel disease

Atypical, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibodies (x-, c- and pANCA, respectively) are associated with a variety of inflammatory diseases, including inflammatory bowel disease (IBD). Anti-neutrophil cytoplasmic antibodies are more common in patients with ulcerative colitis (UC) than in patients with Crohn's disease (CD). Most publications only refer to p- and cANCA in relation to IBD. We have prospectively evaluated the reactivity of sera from 58 patients with IBD and 10 healthy controls against human neutrophils with emphasis on the distinction of the ANCA types. The sera were incubated with ethanol- and formaldehyde-fixed granulocytes to differentiate between c-, p- and xANCA. The results showed that 10 of 24 patients with UC were positive for ANCA, whereas only one of 34 patients with CD was ANCA positive. These results correspond to a sensitivity of 42%, a specificity of 97%, a negative predictive value of 91% and a positive predictive value of 75% in UC. Of the 11 ANCA-positive sera, two showed a cytoplasmic staining pattern, three showed a perinuclear and six an atypical staining pattern. The disease activity was not correlated to either the ANCA titre or to the presence of ANCA in the serum. In conclusion, ANCA are of limited value in differentiating between UC and CD. Because the majority of ANCA in patients with IBD are xANCA, these ANCA should be explored by not only incubating on ethanol-fixed granulocytes, but also on formaldehyde-fixed granulocytes.