Significance of antineutrophil cytoplasmic antibody in adult patients with Henoch-Schönlein purpura presenting mainly with gastrointestinal symptoms

AIM： To test the clinical significance of antineutrophil cytoplasmic antibody （ANCA） in evaluation of adult Henoch-Schonlein purpura （HSP） patients presenting mainly with abdominal symptoms. METHODS： Twenty-eight consecutive HSP patients who presented predominantly with abdominal symptoms were enrolled in this study. Control subjects included 27 ageand sex-matched patients with peptic ulcer disease, colon cancer, acute gastroenteritis, irritable bowel syndrome and colonic polyps. ANCA was measured by indirect immunofluorescence （IIF） in all patients, and follow-up ELISA was performed in patients with positive IIF tests. RESULTS： ANCA was detected in 9 HSP patients by IIF （2 were positive for c-ANCA and 7 were positive for p-ANCA）. No ANCA was found in the control group. The sensitivity and specificity of a positive ANCA test （either c- or p-ANCA） were 32.1% and 100% respectively. Only one out of the 9 patients with positive ANCA by IIF had positive ANCA by ELISA and the antigen was myeloperoxidase （MPO）. The patients positive for ANCA had higher HSP clinical scores, and were more likely to have renal function impairment. Patients with late purpura development were also associated with more severe clinical manifestations. CONCLUSION： A positive ANCA test is associated with more severe symptoms in HSP. After inflammatory bowel disease is excluded, a positive ANCA test provides a clue to the diagnosis of HSP presenting predominantly with abdominal symptoms.     

Anti-neutrophil cytoplasmic antibodies (ANCA) and inflammatory bowel diseases in Chinese

Inflammatory bowel diseases are uncommon in the Chinese, but the incidence is rising. Their differentiation from infective colitis is often not clear-cut and diagnosing inflammatory bowel diseases can be difficult in Asia. We have studied Chinese patients with ulcerative colitis (N=19) and Crohn's disease (N=12) for anti-neutrophil cytoplasmic antibodies (ANCA) by indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assays (ELISA). Patients with enteric fever (N=29) and irritable bowel syndrome (N=24) were recruited as controls. Seventy-three percent of ulcerative colitis patients exhibited either p-ANCA (31%) or c-ANCA (42%) by IIF. Twenty-five percent of Crohn's disease patients were found to be p-ANCA positive. However, these ANCA were nonreactive to anti- granule, antiproteinase 3, antimyeloperoxidase, or antilactoferrin. All positive patients had extensive colitis. Sera collected from patients suffering from enteric fever and irritable bowel syndrome were negative for ANCA by IIF and ELISA. We concluded that the detection of ANCA is helpful in diagnosing inflammatory bowel diseases. Further attempts to characterize these autoantibodies are needed.This study was partly supported by the Croucher Foundation (grant 1634-29) and Research Grant Committee (grant CUHK/34/92M).     

Prevalence of antineutrophil cytoplasmic autoantibodies in patients with tuberculosis

OBJECTIVE: To determine the prevalence of antineutrophil cytoplasmic autoantibodies (ANCA) in sera of patients with tuberculosis compared with healthy control subjects and a group of patients with atopic asthma. METHODS: The presence of ANCA was examined in patients with tuberculosis, and in asthmatic patients and healthy subjects as control groups, by means of indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assay (ELISA) to detect anti-proteinase 3 (PR3-ANCA) and antimyeloperoxidase (MPO-ANCA) antibodies. RESULTS: ANCA were present in 20 (44.4%) of 45 tuberculosis patients by IIF (16 c-ANCA, four p-ANCA) and in 18 (40%) patients by ELISA (15 PR3-ANCA, three MPO-ANCA). High odds ratios for ANCA positivity were observed for tuberculosis patients when compared with both control groups. ANCA results were not related to the category of tuberculosis, stage of disease, presence of concomitant diseases or pharmacotherapy. CONCLUSIONS: As many clinical similarities between tuberculosis and Wegener's granulomatosis exist, we propose that a positive ANCA test in patients living in countries with a high prevalence of tuberculosis must be carefully interpreted as indicative of systemic vasculitis, especially when no signs of extrapulmonary involvement occur.     

抗嗜中性粒细胞胞浆抗体与狼疮性肾炎的关系

目的探讨抗嗜中性粒细胞胞浆抗体（ANCA）与狼疮性肾炎（LN）临床相关表现和发病机制的关系。方法分别应用间接免疫荧光法和酶联免疫吸附分析的方法，检测81例LN患者血清中的ANCA，并分析ANCA与LN临床表现和其它实验室检查结果之间的关系。结果单用间接免疫荧光法检测时，ANCA在LN中的阳性率是30.9％（25／81）。对间接免疫荧光法检测为阳性的血清，用酶联免疫吸附分析法进行验证，结果仅有72.0％（18／25）仍为阳性，全部是核周型ANCA（p-ANCA），未见中央型ANCA（c-ANCA）出现。ANCA阳性组LN患者合并浆膜炎、神经系统累及、贫血、抗ds-DNA抗体阳性和低补体的频率均显著高于ANCA阴性组LN患者。结论ANCA在LN中的阳性率为30.9％，并与LN特定的临床表现相关，提示ANCA可能参与了LN的发病过程。     

Contribution of immunofluorescence to identification and characterization of antineutrophil cytoplasmic antibodies in inflammatory bowel disease

We evaluated the combined use of different fixatives for the identification of atypical perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) in patients with inflammatory bowel diseases (IBD) by indirect immunofluorescence (IIF). Sera from 59 ulcerative colitis (UC) and 37 Crohn’s disease (CD) patients, and from 64 healthy controls were studied. The IIF on ethanol-, formalin-, and methanol-fixed neutrophils was used for the detection of ANCA. Enzyme linked immunosorbant assay (ELISA) was performed to identify the antigens recognized by ANCA. ANCAs were present in 35 of 59 (59.3%) UC patients and in 10 of 37 (27.02%) CD patients. Atypical p-ANCA positivity was strongly associated with UC disease (44.1% in UC vs. 8.1% in CD; p = 0.0002). The combined application of different fixatives contributed to make easy the differentiation between typical p-ANCA and atypical p-ANCA. Atypical p-ANCA determination appears to be a useful parameter for the distinction between UC and CD.     

抗中性粒细胞胞浆抗体在肾炎综合征中的检测及其临床意义

目的　探讨检测抗中性粒细胞胞浆抗体 (ANCA)及其靶抗原在肾炎综合征中的临床意义。方法　应用间接免疫荧光 (IIF)法检测 10 0例肾炎综合征患者血清抗中性粒细胞胞浆抗体 ,对其阳性的 2 9例用酶联免疫吸附试验 (ELISA )检测靶抗原髓过氧化物酶 (MPO)和蛋白酶 3(PR3 )。结果　IIF检测肾炎综合征ANCA阳性率为 2 9% ,其中胞浆型 10 %、核周型 19%。急进型肾炎、狼疮性肾炎、紫癜性肾炎阳性率分别为 5 6%、2 0 %和 15 %。ELISA急进性肾炎和紫癜性肾炎大多数识别靶抗原MPO ,狼疮性肾炎ANCA不识别MPO或PR3。结论　ANCA在急进性肾炎和狼疮性肾炎中阳性率较高 ,检测ANCA对判断狼疮性肾炎活动及疗效具有参考价值     

Antineutrophil cytoplasmic antibodies in Japanese patients with inflammatory bowel disease: prevalence and recognition of putative antigens

OBJECTIVE: Our aim was to investigate the prevalence of antineutrophil cytoplasmic antibodies (ANCA) in Japanese patients with ulcerative colitis (UC) and Crohn's disease (CD), and the putative antigens recognized by perinuclear staining pattern ANCA (p-ANCA)-positive sera. METHODS: Sera from UC (n = 52) and CD (n = 43) patients, and from healthy controls (n = 74) were studied. The indirect immunofluorescence (IIF) method was used for the detection of ANCA and its binding pattern. p-ANCA-positive sera were studied further for putative antigens. ELISAs using lactoferrin (Lf), myeloperoxidase (MPO), and cathepsin G (Cat G) as antigens were performed. RESULTS: ANCA was positive in 40 of the 52 (76.9%) UC (p-ANCA in 33) and in 32 of the 43 (74.4%) CD (p-ANCA in 31) patients. UC and CD patients showed significantly higher titers of p-ANCA than controls; however, no significant difference was observed between UC and CD. In UC, 23, 17, and nine of the 33 patients with p-ANCA-positive sera showed reactivity with Lf, MPO, and Cat-G, respectively. In CD, 21, 20, and 11 of the 31 patients with p-ANCA-positive sera showed reactivity with Lf, MPO, and Cat-G, respectively. Fourteen of the UC and six of the CD patients showed reactivity with two different antigens, and seven of the UC and 11 of the CD patients showed reactivity with all three antigens. The presence of anti-Lf and anti-MPO antibodies was further confirmed by Western blotting. CONCLUSIONS: ANCA is useful in distinguishing patients with IBD from normal subjects but is not sufficient for the differential diagnosis of CD and UC. p-ANCA reactivity might be derived from the recognition of heterogeneous neutrophil-associated antigens.     

The significance of IgA class of antineutrophil cytoplasmic antibodies (ANCA) in childhood Henoch–Schönlein purpura

Antineutrophil cytoplasmic antibodies (ANCA) have been identified in a wide variety of vasculitic disorders, but it is controversial whether ANCA are present in the sera of patients with HSP. This prospective study was designed to assess the place of ANCA, particularly their IgA subclass, in HSP. Thirty-five patients (18 boys, 17 girls) aged 9.4±4 (3–16) years with a clinical diagnosis of HSP based on American College of Rheumatology (ACR) criteria were enrolled. Thirteen patients (6 boys, 7 girls) aged 8.3±5.5 (2–21) years with other vasculitides, consisting of classic polyarteritis nodosa (PAN) (n=2); cutaneous polyarteritis nodosa (n=1); acute infantile hemorrhagic edema (n=2); acute urticarial vasculitis (n=2); hypocomplementemic vasculitis (n=1); and unclassified vasculitis (n=5) served as disease controls and 10 healthy children served as normal controls. Twenty-five HSP patients and 7 disease controls were re-evaluated in the resolution phase that was described as 4–6 weeks after all symptoms subsided and all medications were stopped. Blood samples for ANCA and IgA rheumatoid factor (RF) were studied by indirect immunofluorescence (IIF) and ELISA, respectively. IgG ANCA was significantly lower in percentage in HSP patients (2.8%) than in disease controls (40%) (p=0.002). In contrast, IgA ANCA in cytoplasmic pattern was detected in a significantly higher percentage of HSP patients (82.3%) in the acute phase compared to those in the disease controls (38%) (p=0.004). In the resolution phase, IgA ANCA was negative in 88% of the patients (p= 0.001 for acute vs resolution phases). Neither IgG nor IgA ANCA were seen in normal controls. No relationship was found between disease severity of HSP and IgA ANCA. Positive IgA rheumatoid factor was present in only two patients with HSP. In conclusion, our results suggest that IgA ANCA may be useful to confirm the diagnosis of HSP in children.Abbreviations ANCA Antineutrophil cytoplasmic antibodies - EBV Epstein–Barr virus - HSP Henoch–Schönlein purpura - IIF Indirect immunofluorescence - PAN Polyarteritis nodosa     

A case of Henoch-Sch?nlein purpura with psoas muscle abscess and full-blown gastrointestinal complications]

Henoch-Sch?nlein purpura (HSP) is a vasculitis involving small vessels of skin, joints, gastrointestinal (GI) tract, and kidneys. The patients typically show palpable purpura with one or more characteristic manifestations including abdominal pain, hematuria or arthritis. HSP shows gastrointestinal symptoms in 50~85% of patients, and in 14~40% of patients GI symptoms precede purpuric rash which makes the diagnosis of HSP difficult. We present a case of Henoch-Schonlein purpura with GI bleeding, septic shock by ileal microperforation, small bowel obstruction as a result of ileal stricture and psoas muscle abscess.     

Fatal adult Henoch-SchÖnlein purpura due to small intestinal infarction

A 57-year-old woman presented with classical symptoms of Henoch-SchÖnlein purpura including arthralgia, abdominal pain, palpable purpuric rash and abnormalities of the urinary sediments. The clinical course was subsequently complicated by severe paralytic ileus leading to respiratory embarrassment. Laparotomy confirmed patchy areas of small bowel infarction due to necrotizing vasculitis. Skin biopsy revealed extensive leukocytoclastic vasculitis with granular deposits of IgA in the blood vessels. Anti-neutrophil cytoplasmic autoantibodies (ANCA) were not detectable. The patient continued to deteriorate despite bowel resection, intensive immunosuppressive therapy and plasma exchange, and eventually died. Gastrointestinal involvement in adult Henoch-SchÖnlein purpura has previously been reported to have a favourable clinical course. However, our case illustrates the rare but lethal complication of intestinal infarction in patients who fail to respond to conservative treatment.     

Prevalence of antineutrophil cytoplasmic antibodies in patients with various pulmonary diseases or multiorgan dysfunction

OBJECTIVE: To determine the prevalence of antineutrophil cytoplasmic antibodies (ANCA) in patients with diseases that may mimic systemic vasculitides, such as severe multiorgan dysfunction (MOD) and parenchymal pulmonary disorders. METHODS: We conducted a prospective study of patients with MOD admitted to the medical intensive care unit and patients with various lung diseases seen at the outpatient pulmonary clinic of a tertiary care hospital. Patients with a documented diagnosis of Wegener's granulomatosis (WG) served as positive controls. ANCA were determined in serum samples from each patient by a combination of indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assays (ELISAs) for antibodies to proteinase-3 and myeloperoxidase (anti-MPO). RESULTS: Ninety-nine patients with MOD, 29 outpatients with various lung disorders, and 18 patients with WG were included in the study. ANCA were detected by IIF alone in 16% (15/96) of patients with nonvasculitic MOD and 17% (5/29) of outpatients with various pulmonary disorders. The majority of the positive IIF specimens from each group displayed an atypical IIF pattern (73% and 80%, respectively). Only 1 specimen from patients with nonvasculitic disorders was positive for anti-MPO. ANCA by both IIF and ELISA were detected in 78% (12/14) of control patients with WG. CONCLUSION: Detection of ANCA by the combination of IIF and antigen-specific assays for proteinase 3 and myeloperoxidase in diseases that mimic systemic vasculitides is highly specific for WG, microscopic polyangiitis, and Churg-Strauss syndrome.     

Predictive factors for nephritis, relapse, and significant proteinuria in childhood Henoch-Schönlein purpura

OBJECTIVE: To identify predictive factors for nephritis, relapse, and significant proteinuria in childhood Henoch-Sch?nlein purpura (HSP). METHODS: Two hundred and six consecutive patients with HSP (93 female, 113 male), followed up at a single centre between 1996 and 2001, were analysed retrospectively. They were regularly monitored for clinical and laboratory parameters for renal sequelae and relapse. RESULTS: Nephritis was seen in 78 patients (38%), relapse in 52 (25%), and significant proteinuria in 39 (19%). In univariate analysis, an older age at onset (>10 years), persistent purpura, severe bowel angina, and relapse were identified as factors associated with nephritis and significant proteinuria. Relapse-related factors were an older age, persistent purpura, severe bowel angina, and leucocytosis. Logistic regression analysis showed that nephritis was significantly associated with an older age, persistent purpura, and relapse, and significant proteinuria was closely related to severe bowel angina and relapse. CONCLUSION: We identified some predictors for nephritis, relapse, and significant proteinuria in childhood HSP, and close attention should be paid to those patients with the risk factors, such as an older age at onset, persistent purpura, severe bowel angina, and relapse.     

Autoantibodies to proteinase 3 and myeloperoxidase in systemic sclerosis

OBJECTIVE: We evaluated the prevalence and clinical significance of proteinase 3 (PR3-) and myeloperoxidase (MPO-) antineutrophil cytoplasmic antibodies (ANCA) in 115 patients with systemic sclerosis (SSc, scleroderma). METHODS: Sera were assayed by 2 independent centers, which used indirect immunofluorescence (IIF) and direct ELISA as screening tests. Inhibition-ELISA for PR3- and MPO-ANCA and PR3 capture-ELISA experiments were also performed. The clinical features of the ANCA positive were compared with those of the ANCA negative scleroderma patients. RESULTS: The IIF test revealed 5 P-ANCA positive sera (4.34%). Surprisingly, by ELISA 2 of these were PR3-ANCA positive, one was MPO-ANCA positive, and 2 were both PR3- and MPO-ANCA positive. In addition, 3 IIF negative sera were ELISA positive, 2 for PR3- and one for MPO-ANCA. ELISA results were confirmed by fluid phase inhibition experiments. Only 2 out of the 6 PR3-direct ELISA positive sera were positive by PR3-capture ELISA at low titers. Neither PR3- nor MPO-ANCA were significantly associated to any clinical feature of patients with SSc. CONCLUSION: As well as the previously described MPO-ANCA, even PR3-ANCA may be detected in some sera from patients with SSc. The IIF pattern and the negative results obtained with PR3-capture ELISA suggest that different epitopes from those recognized by vasculitis sera might be involved with PR3-ANCA in SSc, and show the importance of combining IIF and ELISA tests for ANCA detection.     

Antineutrophil cytoplasm autoantibodies against bactericidal/permeability-increasing protein in inflammatory bowel disease.

BACKGROUND: Bactericidal/permeability-increasing protein (BPI), a constituent of primary neutrophil granules, is a potent natural antibiotic and an antineutrophil cytoplasm antibody (ANCA) antigen in cases of vasculitis in which the target antigen is neither myeloperoxidase (MPO) nor proteinase-3 (PR3). AIM: To investigate BPI as a possible target antigen for ANCAs in inflammatory bowel disease. METHODS: ANCAs were detected by routine immunofluorescence (IIF) and solid phase enzyme linked immunosorbent assay (ELISA) performed for antibodies to the purified neutrophil granule proteins; MPO, PR3, cathepsin-G, lactoferrin, and BPI in serum samples from 88 patients with inflammatory bowel disease (36 with Crohn's disease, 52 with ulcerative colitis). Thirty patients with bacterial enteritis acted as controls. RESULTS: Significantly more patients with ulcerative colitis were ANCA positive by IIF (60%) than patients with Crohn's disease (28%) or infectious enteritis (23%) (p < 0.001). IgG anti-BPI antibodies were present in 29% of patients with ulcerative colitis, 14% of patients with Crohn's disease, and 23% of patients with infectious enteritis, occurring in 44% of those patients with inflammatory bowel disease who were ANCA positive by IIF. Antibodies to other ANCA antigens were rare. The presence of ANCAs was not related to either disease activity or extent; presence of anti-BPI antibodies was significantly related to both a lower serum albumin concentration (p = 0.001) and a higher erythrocyte sedimentation rate (p = 0.02) in patients with ulcerative colitis, and to colonic involvement in patients with Crohn's disease (p = 0.01). CONCLUSION: BPI is a significant minority target antigen for ANCAs in inflammatory bowel disease that seems related to colonic Crohn's disease and disease activity in ulcerative colitis. Anti-BPI antibodies occur in infectious enteritis.     

Clinical and immunological study of 7 patients with minocycline-induced autoimmune phenomena

PURPOSE: Prolonged treatment with minocycline for acne vulgaris has been associated with the development of arthralgia, arthritis, and other autoimmune phenomena. We characterized the clinical, laboratory, and immunological profiles of seven patients with this syndrome.SUBJECTS AND METHODS: Clinically the patients were studied with special emphasis on prior minocycline treatment, presenting symptoms, physical findings, course, and outcome. Laboratory tests included fluorescent antinuclear and antineutrophil cytoplasmic (ANCA) antibodies, as well as antibodies to myeloperoxidase, bactericidal permeability increasing protein, elastase, cathepsin G, lactoferrin, cardiolipin, and histone.RESULTS: All 7 patients presented with polyarthritis or arthralgia, morning stiffness, and fever after 6 to 36 months of minocycline treatment. The skin was involved in five patients (three with livedo reticularis and two with subcutaneous nodules). Two patients had chronic active hepatitis. Increased titers of perinuclear ANCA (p-ANCA) were detected in all seven patients; five patients had fluorescent antinuclear antibodies, two had antihistone autoantibodies and one had anticardiolipin antibodies. Antigenic characterization of p-ANCA disclosed antibodies to bactericidal permeability increasing protein in one patient, to elastase in three patients, and to cathepsin G in five patients. Symptoms resolved in five patients upon discontinuation of minocycline; the other two patients were treated with corticosteroids and also achieved remissions.CONCLUSION: Minocycline-induced autoimmune syndrome is characterized by reversible polyarthralgia or arthritis, morning stiffness, fever, frequent skin involvement, occasional chronic active hepatitis, and increased titers of p-ANCA with various minor p-ANCA–related antigens.     

腹型过敏性紫癜的临床及内镜表现

目的 总结腹型过敏性紫癜的临床和内镜特点,以加强对此病的认识。方法 对1994 年1月以来收治的197名过敏性紫癜患者病例资料进行回顾性分析,其中81名为腹型紫癜。19例次 接受了胃镜或肠镜检查。结果 腹型紫癜占紫癜病例的41.1％,其中51例(63.0％)在发病前1-3 周有明确诱因。21例以消化道症状首发,其消化道症状可早于皮损1-40 d出现。74例(91.4％)出 现腹痛,37例(45.7％)表现为消化道出血。内镜表现为胃肠道黏膜弥漫性充血水肿,广泛多发的出 血点、红斑、糜烂、溃疡,小肠病变较重。胃肠道黏膜组织病理学表现为黏膜及黏膜下层中到大量中性 粒细胞浸润、小血管壁纤维素性坏死、灶性出血、糜烂和溃疡。患者的内镜和临床病理表现的范围和 严重程度与消化道症状的程度一致。结论 41.1％的紫癜患者表现为腹型过敏性紫癜,腹型紫癜中 25.9％以消化道症状为首发。小肠病变程度重于胃或结肠。其典型的临床和内镜表现对早期诊断有 一定帮助。     

Anti-neutrophil cytoplasmic antibodies (ANCA) in malaria

Various autoantibodies like anti-nuclear antibodies (ANA), anti-double stranded DNA (anti-dsDNA), anti-histone antibodies (AHA), anti-neutrophil cytoplasmic antibodies (ANCA), anti-myeloperoxidase (anti-MPO), anti-proteinase3 (anti-PR3) and anti-lactoferrin (anti-LF) antibodies were studied in 173 acute hospitalised patients suffering from malaria of which 160 patients had P. falciparum and remaining 13 had P. vivax infection. Standard methods like indirect immunofluorescence (IIF) microscopy along with Confocal microscopy and ELISA were used for identifying and quantifying the autoantibodies and IIF patterns on PMN and HL-60 cells were studied for ANCA classification. Also HEp-2 cells were used for ANA detection, while estimation of anti-dsDNA, AHA, anti-MPO, anti-PR3 and anti-LF were tested using ELISA. Sera from malaria patients showed prominent immunofluorescence staining patterns where 23.8% cases had ANA in P. falciparum group as compared to 15.4% in P. vivax group and ANCA was found to be present in 20% in P. falciparum and 15.4% in P. vivax group. An interesting observation was that, of the total ANCA positives, 59% had p-ANCA, 5.9% had c-ANCA and 44.1% of the cases showed the 'atypical' or X-ANCA pattern. When p-ANCA positivity was compared with c-ANCA positivity among these patients, a good statistical correlation was noted with OR = 16, chi 2 = 16.43, EF = 0.46 and p-value = 5.037E 0.5. ELISA showed 31.2% anti-MPO and 6.2% anti-PR3 in P. falciparum cases while the two ANCA positive cases in P. vivax had anti-MPO. Anti-LF was found to be present in 40.6% cases. Neither the P. falciparum nor P. vivax contained autoantibodies with specificities similar to the characteristic lupus autoantibodies such as double stranded DNA (dsDNA). ANCA positivity develops in some types of malarial infection also with the presence of various autoantibodies which is important from a clinical point of view and should be carefully evaluated in those geographic areas where malaria is endemic. It also alerts us to the fact, whether in cases of repeated malarial infections in susceptible individuals, vasculitic disorders, which through ANCA pathways develop, could lead to renal and other complications.     

Parvovirus B19 infection associated with the production of anti-neutrophil cytoplasmic antibody (ANCA) and anticardiolipin antibody (aCL)

We described four patients who had clinical diagnosis of erythema infectiosum and presented with skin rash, polyarthralgia, polyarthritis, and mild fever. Anti-parvovirus B19 IgM and IgG antibodies were found in all four patients and parvovirus B19 DNA was detected in three of the four patients by polymerase chain reaction (PCR) in sera using standard methods. Anticardiolipin antibody (aCL) was positive in three of the four patients included three with anti-beta2 glycoprotein I (beta2GPI). The immunoglobulin isotype of aCL was found to be IgM. Anti-neutrophil cytoplasmic antibody (ANCA) included three p-ANCA and one c-ANCA was detected in all four patients by indirect immunofluoresence (IIF). Both anti-proteinase 3 (PR3) and anti-myeloperoxidase (MPO) antibodies were found in two patients whom had polyarthritis for more than 6 months. These data indicate parvovirus B19 may be linked to the induction of an autoimmune response.     

65例系统性红斑狼疮患者抗中性粒细胞胞浆抗体的检测分析

目的探讨抗中性粒细胞胞浆抗体(ANCA)与系统性红斑狼疮(SLE)的关系及临床意义.方法回顾性分析65例SLE患者的ANCA检测结果; ANCA与SLE主要临床表现、实验室检查结果的关系; SLE患者病情活动组与非活动组ANCA阳性率的比较.结果 IIF法检测ANCA在SLE中的阳性率是61.5 %,ANCA阳性组中有血管炎皮损(67.5 %)及浆膜炎(55.0 %)者明显高于阴性组(P<0.05);同时ANCA阳性组与阴性组比较,在24 h尿蛋白大于0.5 g/L,血红蛋白低于90 g/L,抗ds-DNA抗体阳性,低补体血症方面差别有统计学意义(P<0.05).结论提示ANCA可能是判断SLE病情复发与缓解的一个有用指标,推测ANCA与活动性狼疮肾炎有关.     

Pulmonary hemorrhage associated with Henoch-Schönlein purpura in a child

Swelling of the right ankle and purpura on the bilateral lower extremities of a 6-year-old boy indicated a diagnosis of Henoch-Schönlein purpura (HSP). The patient was referred to our hospital because of severe abdominal pain that was unresponsive to prednisolone. Respiratory symptoms and pulmonary infiltrates on chest X-ray were absent upon admission. Methylprednisolone pulse therapy gradually improved the abdominal symptoms, but tachypnea developed, and insufficient aeration of the right lung was accompanied by a decline in percutaneous oxygen saturation from 99% to 90% and a rapid decrease in hemoglobin levels from 11.7 to 7.6 g/dL. Chest X-rays and high-resolution computed tomography scans showed widespread consolidation and patchy opacities predominantly in the right lung fields, suggesting acute pulmonary hemorrhage. Intravenous cyclosporin A (CyA) gradually resolved the pulmonary hemorrhage and respiratory insufficiency. Pulmonary hemorrhage, as a complication of HSP, is extremely rare and sometimes fatal. Aggressive steroid and immunosuppressive treatment is required to address severe complications of HSP.