Perinuclear Antineutrophil Cytoplasmic Autoantibodies Research Articles

ANCA-positive IgA nephropathy without necrotising or crescentic glomerulonephritis: a clinical conundrum

IgA nephropathy, the most prevalent form of primary glomerular disease, usually portends a favourable outcome. Antineutrophil cytoplasmic autoantibodies (ANCAs) have been reported in association with IgA nephropathy in a small...

Autoantibodies in a Three-Year-Old Girl with Visceral Leishmaniasis: A Potential Diagnostic Pitfall.

Visceral leishmaniasis (VL), a life-threatening parasitic infection, is endemic in the Mediterranean region. Diagnosis of VL is based on epidemiologic, clinical, and laboratory findings. However, sometimes, clinical features and laboratory findings overlap with those of autoimmune diseases. In some cases, autoantibodies are detected in patients with VL and this could be a potential diagnostic pitfall. In this study, we have reported on a three-year-old girl from a VL-endemic area in Iran, who presented with prolonged fever and splenomegaly. Bone marrow examination, serologic tests, and the molecular PCR assay were performed; however, results were inconclusive. The levels of anti-double stranded DNA, cytoplasmic antineutrophil cytoplasmic autoantibody, and perinuclear antineutrophil cytoplasmic autoantibody were elevated and, at the end, splenic biopsy was performed. The splenic tissue PCR test detected the DNA of Leishmania infantum. The patient's condition improved with anti-Leishmania therapy, and the autoantibodies disappeared within the following four months. Clinical presentations and laboratory findings of VL and autoimmune diseases may overlap in some patients.

A Case of Different Organ Responses to Immunosuppressive Therapy In a Microscopic Polyangiitis Patient with Interstitial Lung Disease

Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis associated with antineutrophil cytoplasmic autoantibodies. Interstitial lung disease is a less recognized manifestation of MPA and has a poor prognosis. A 61-year-old man presented with persistent cough, sputum and dyspnea. Laboratory examination revealed microscopic hematuria and renal insufficiency. Perinuclear anti-neutrophil cytoplasmic autoantibodies were positive according to serological testing. Computed tomography scans showed bibasilar reticulation and honeycombing in a peripheral distribution. Therefore, renal biopsy was performed, and MPA was diagnosed. After treating with corticosteroids and immunosuppressive agents, the patient had a complete renal response but progressive interstitial lung disease. We report a case of MPA presenting with interstitial lung disease in which the patient experienced different responses in each organ. (Korean J Med 2014;86:84-88)

A new cutoff value for fecal calprotectin level in differentiating functional from organic causes of chronic diarrhea

Background The gold standard to establish inflammatory bowel disease diagnosis remains in the hands of endoscopists and pathologists. A challenge is thus to distinguish symptoms of inflammatory bowel disease from that of irritable bowel syndrome. Aim of this work The aim of this study was to evaluate the clinical usefulness of fecal calprotectin level as a noninvasive marker in order to distinguish patients with diarrhea in need of intensified follow-up from those who do not need further workup. Patients and methods From a total of 150 patients presented with chronic diarrhea with or without bleeding per-rectum in the outpatient clinic of Specialized Medical Hospital, only 60 were involved in this study. Stool analysis and culture were carried out. Measurement of fecal calprotectin was done using the ELISA kit. Inflammatory biomarkers, such as erythrocyte sedimentation rate and C-reactive protein and perinuclear anti-neutrophil cytoplasmic autoantibodies (P-ANCA) and cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA), were tested. Full colonoscopy with histopathological examination was performed. Results The frequencies of diseases on the basis of diagnostic colonoscopy and pathological examination were as follows: 19 patients with irritable bowel syndrome (31.67%), 41 patients with nonorganic gastrointestinal (GIT) disease versus organic GIT diseases (68.33%), 32 patients with ulcerative colitis (53.33%), two patients with Crohn's disease (3.33%), two patients with diverticulitis (3.33%), two patients with eosinophilic gastroenteritis (3.33%), and two patients with cancer colon (3.33%). There was a remarkable difference between organic GIT diseases versus nonorganic groups as regard fecal level of calprotectin (P Conclusion Calprotectin, a fecal marker, is helpful as an adjunctive tool in overall evaluation of patients with nonspecific symptoms and as a diagnostic tool in those with inflammatory disease. It is less invasive than colonoscopy and can help to guide management in a more cost-effective manner.

Perinuclear antineutrophil cytoplasmic autoantibodies in dogs infected with various vector-borne pathogens and in dogs with immune-mediated hemolytic anemia

To determine the prevalence of perinuclear antineutrophil cytoplasmic autoantibodies (pANCA) in dogs with confirmed or suspected immune-mediated hemolytic anemia (IMHA) or dogs infected with various vector-borne pathogens, including Rickettsia rickettsii, Bartonella henselae, Bartonella vinsonii subsp berkhoffii, Ehrlichia canis, Borrelia burgdorferi, and Leishmania infantum. 55 dogs with confirmed or suspected IMHA, 140 dogs seroreactive for vector-borne pathogens, and 62 healthy dogs and dogs seronegative for vector-borne pathogens. Samples were allocated to subgroups on the basis of the health status of the dogs and the degree of seroreactivity against various vector-borne pathogens. Serum samples were tested retrospectively via indirect immunofluorescence assay to determine pANCA status. 26 of 55 (47%) dogs with confirmed or suspected IMHA and 67 of 140 (48%) dogs seroreactive for vector-borne pathogens had positive results when tested for pANCA. Serum samples with the highest antibody concentrations against L infantum antigen had the highest proportion (28/43 [65%]) that were positive for pANCA. One of 20 (5%) dogs seronegative for tick-borne pathogens and 8 of 22 (36%) dogs seronegative for L infantum had positive results for pANCA. One of 20 (5%) healthy dogs had serum antibodies against pANCA. pANCA were detected in a high percentage of dogs with IMHA and vector-borne infectious diseases. Therefore, pANCA may be a relatively nonspecific marker for dogs with inflammatory bowel disease, although they could represent a biomarker for immune-mediated diseases and infections.

The Role of Anti- Cathapsin G among Patients with Rheumatiod Arthritis

Background: Perinuclear Antineutrophil Cytoplasmic Autoantibodies (pANCA) have been found in patients with rheumatiod arthritis. Cathepsin G was the major target antigen.The present study was to investigate the unknown target antigen of ANCA (Cathapsin G ) in patients with rheumatiod arthritis Objective: This study is to investigate the prevalence of anti-Cathapsin G and rheumatoid factor in Iraqi patients with rheumatiod arthritis.Patienties&Methods: From1st January until 30 June of 2011 fourty five rheumatiod arthritis patients referred to the immunological department in the teaching laboratory of medical city and twenty five apparently healthy individual used as a control group were investeged to rheumatic factor IgG, IgM ,IgA isotypes and anti-Cathapsin G were measured by using enzyme immunoassay technique.Result: This study showed that the age of patients with rheumatiod arthritis ranged between eigthen years and sixty six years with mean age of (36.08) , while the mean level for RF screen, RF IgG, RF IgM, RF IgA isotypes and anti-Cathapsin G were (160.71, 91.7, 136.64, 95.03 and > 9.2 ) respectively. There were 17(37.8%) males and 28(62.2%) females of RA patients. Immunologically, this study revealed that the serum RF screen was found more than 25 U/ml in all rheumatiod arthritis patients (100%, P: 0.0001 HS). On the other hand RF isotypes IgG ,RF IgM and IgA level were elevated more than 20 U/ml and it was detected in 31 (68.9%, P: 0.0001 HS), 31 (68.9%, P : 0.0001 HS) ,and 32 (71.1%, P: 0.0001 HS) patients respectively, while the rheumatiod factors (IgG,IgM, and IgA)were normal in healthy control group . Statistically RF screen has a highly significant association with RF IgG, IgM, and IgA (P= 0.0001). Anti-Cathapsin G was found in 12(26.7%, P value: 0.019) of patients with rheumatic arthritis patients. This study showed a association between the positive result of anti-Cathapsin G with RF IgG and RF IgM (35.5%, P: 0.047) the results were the same for both, while no association found with RF IgA (25%, P: 0.692).Conclusion: A significant correlation was found between anti-Cathapsin G and patients with rheumatiod arthritis and has a significant association with RF IgG and RF IgM while RF IgA had no relation with anti-Cathapsin G.

Auto-antibodies in patients with inflammatory bowel disease unclassified.

Inflammatory bowel disease unclassified (IBDU) is considered to be an aberrant immune response with loss of tolerance to many antigens. This paper tries to address whether there is any value to test for auto-antibodies in such patients. 60 patients with inflammatory bowel disease unclassified participated in the study. Auto-antibodies to nuclear antigen, intestinal goblet cell, exocrine part of pancreatic acinar cells, perinuclear antineutrophil cytoplasmic, cytoplasmic antineutrophil cytoplasmic and Saccharomyces cerevisiae were tested and compared to 20 ulcerative colitis (UC) patients and 30 healthy controls matched for age and sex. There was a significant difference (p=0.000) between patients and control group in anti-exocrine part of pancreatic acinar cells, perinuclear antineutrophil cytoplasmic and Saccharomyces cerevisiae auto-antibodies. There was also a significant difference between IBDU and UC patients in the auto-antibodies directed against intestinal goblet cells, (p=0.000) exocrine part of pancreas (p=0.000) and anti Saccharomyces cerevisiae antibody (p=0.000). Due to the autoimmune nature of indeterminate colitis, involvement of some antigens from gastrointestinal tract or the bile system in the initiation of this disease is likely.

A case of a 6-year-old girl with anti-neutrophil cytoplasmic autoantibody-negative pauci-immune crescentic glomerulonephritis

A 6-year-old girl was admitted to our hospital with proteinuria, hematuria, skin rash and joint pain of the lower limbs. Due to rapid progression of renal insufficiency, hemodialysis and peritoneal dialysis were performed. She was diagnosed with rapidly progressive glomerulonephritis. Kidney biopsy showed severe crescent formation (50% of glomeruli) and no deposition of any immunoglobulins or complements. Serologically, anti-neutrophil cytoplasmic autoantibody (ANCA) was negative not only by ELISA against proteinase-3 and myeloperoxidase-ANCA but also by indirect immunofluorescent assay against cytoplasmic and perinuclear ANCA. Anti-glomerular basement membrane antibody was also negative. In the acute phase, proinflammatory cytokines such as soluble tumor necrosis factor receptor 1 (sTNFR1), soluble interleukin (IL)-2 receptor (sIL2R), IL-6 and chemokine IL-8 were elevated. The patient was diagnosed with ANCA-negative pauci-immune crescentic glomerulonephritis (CrGN). Intensive treatment with methylprednisolone pulse therapy, plasma exchange, and multiple drug therapy including prednisolone and cyclophosphamide resulted in histopathological improvement and complete remission of proteinuria. There was a possibility that sTNFR1, sIL2R, IL-6 and IL-8 might be involved in the initiation and progression of ANCA-negative pauci-immune CrGN, and to remove and suppress these cytokines might be an effective way to treat ANCA-negative pauci-immune CrGN.

Cutting Edge Issues in Goodpasture’s Disease

Goodpasture's disease, or anti-glomerular basement membrane (anti-GBM) disease, is a systemic autoimmune disorder defined by anti-GBM antibody-mediated damage (mainly immunoglobulin G-1) resulting in progressive crescentic glomerulonephritis and, frequently, diffuse pulmonary alveolar hemorrhage. It may be regarded as a "conformeropathy" where the quaternary structure of the α345NC1 hexamer that constitutes GBM undergoes a conformational change, exposing pathogenic epitopes on the α3 and α5 chains, eliciting a pathogenic autoantibody anti-GBM response. Goodpasture's disease accounts for 20% of all patients presenting with a pulmonary-renal syndrome and may be associated with detectable perinuclear antineutrophil cytoplasmic autoantibody positivity in up to a third of patients. Associated triggers may include tobacco smoking, hydrocarbon solvent exposure, and cocaine abuse. Cough, hemoptysis, and dyspnea with fatigue are the commonest presenting features. It is critical to rapidly distinguish Goodpasture's disease from other causes of pulmonary-renal syndromes such as Wegener's granulomatosis. Early and intensive treatment with plasmapheresis and immunosuppression with systemic corticosteroids pending results of diagnostic testing, and later cyclophosphamide, is often beneficial, with 90% of patients surviving the acute presentation of Goodpasture's disease. The need for hemodialysis on initial presentation, a serum creatinine >5mg/dL, and 50% to 100% crescents on renal biopsy, portend the necessity of long-term hemodialysis. Further elucidation of the molecular pathobiology of Goodpasture's disease, particularly the regulation of involved antigen-specific T cells, may improve early diagnosis, treatment, and outcomes in this rare but potentially lethal autoimmune disorder.

P-ANCA pachymeningitis presenting with isolated “optic neuropathy”

Isolated bilateral optic neuropathy is an exceedingly rare presentation of perinuclear-antineutrophil cytoplasmic autoantibody (p-ANCA) vasculitis. We report one such case with MRI documentation of a compressive mechanism mediated by pachymeningitis. A 69-year-old woman had a 6-month history of progressive visual failure caused by bilateral optic neuropathy. Cranial MRI showed diffuse contrast enhancement of the dura mater extending to the perioptic sheaths bilaterally with encasement of the optic nerves. Extensive laboratory study showed increased levels of p-ANCA titres and autoimmune markers. Corticosteroid treatment improved both visual acuity and the neuroradiological signs of active pachymeningitis. In conclusion, our case demonstrates that bilateral optic neuropathy may be the presenting symptom of p-ANCA-associated pachymeningitis, whose causative role may be demonstrated by MRI.

Severe Pulmonary Hemorrhage in Patients With End-Stage Renal Disease in Antineutrophil Cytoplasmic Autoantibody–Associated Vasculitis

BackgroundIt has been reported that after patients with antineutrophil cytoplasmic autoantibody (ANCA)–associated vasculitis (AAV) progress to end-stage renal disease (ESRD), they are less likely to experience relapse of vasculitis. However, we encountered a few patients with ESRD suffering severe pulmonary hemorrhage caused by relapse of AAV. The current study presents our observation on these patients. MethodsOf 198 consecutive patients with AAV with follow-up data in our center, 66 progressed to ESRD during follow-up. Clinical and laboratory data were collected and retrospectively analyzed. ResultsAmong the 66 patients with ESRD, 5 experienced severe pulmonary hemorrhage. They had positive serum perinuclear ANCA and myeloperoxidase ANCA and were diagnosed as microscopic polyangiitis. All 5 patients achieved remission after initial induction therapy. The average duration of follow-up was 47.0 (range 8.0–98.0) months. After progressing to ESRD and starting hemodialysis, these patients experienced severe pulmonary hemorrhage within 9.0 (range 2.0–23.0) months. After immunosuppressive therapy, pulmonary hemorrhage ceased in 4 patients, and the other died of respiratory failure. ConclusionsSevere pulmonary hemorrhage can occur in ESRD patients with AAV. Disease activity and relapses of AAV should be monitored even after patients progress to ESRD.

M1116 Evaluation of Perinuclear Antineutrophil Cytoplasmic Autoantibodies (PANCA) and Anti-Saccharomyces Cerevisiae Antibodies (ASCA) in Inflammatory Bowel Disease in a Reference Center in Brazil

M1116 Evaluation of Perinuclear Antineutrophil Cytoplasmic Autoantibodies (PANCA) and Anti-Saccharomyces Cerevisiae Antibodies (ASCA) in Inflammatory Bowel Disease in a Reference Center in Brazil

Potential Interference by Antineutrophil Cytoplasmic Autoantibodies in Myeloperoxidase Immunoassays

Myeloperoxidase (MPO)1 has been identified as a potential marker of cardiovascular disease (1). As the clinical utility of new biomarkers is identified and commercial assays are developed, limitations on their use need to be identified and reported. Antineutrophil cytoplasmic autoantibodies (ANCAs) are known to exist in patients with diseases characterized by primary systematic vasculitis, such as Wegener granulomatosis, microscopic polyangiitis, and Churg– Strauss syndrome, and in those with idiopathic pauci-immune necrotizing crescentic glomerulonephritis, systemic lupus erythematosus, and rheumatoid arthritis (2). In particular, the immunofluorescence- staining pattern for perinuclear ANCAs (pANCAs) is associated with anti-MPO autoantibodies. pANCAs are most prevalent in microscopic polyangiitis (40%–80%), followed by Churg–Strauss syndrome (20%–30%) and Wegener granulomatosis (5%–20%). Autoantibodies can interfere with assays when the autoantibodies bind to analyte epitopes that are similar to those of the monoclonal antibodies used in the immunoassay. Anti-MPO autoantibodies are likely to bind to MPO in the sample and block or partially mask the epitopes necessary for binding to the antibodies used to capture and detect MPO. We evaluated the effect of MPO autoantibodies on the performance of the Cardio MPO™ immunoassay (PrognostiX), which has been cleared by the US Food and Drug Administration. We similarly evaluated an immunoassay under development for the Abbott ARCHITECT® instrument (Abbott …

Les marqueurs sérologiques des maladies inflammatoires chroniques intestinales (MICI)

To date, anti-Saccharomyces cerevisiae antibodies (ASCA) and atypical perinuclear antineutrophil cytoplasmic autoantibodies known as NANA are still the paramount serological markers of IBD. They are relevant for their predictive value to discriminate indeterminate colitis, but also for their prognostic value and more recently observed, for their correlation with disease phenotypes especially in Crohn's disease. This article also gives an overall picture of the others IBD serological markers such as antibodies to exocrine pancreas, and more recently discovered antimicrobial antigens antibodies (such as anti-Escherichia coli outer membrane porin or OmpC, anti-Pseudomonas fluorescens or I2, anti-CBir1 flagellin-derived protein, anti-Bacteroides caccae OmpW, anti-Mycobacterium avium subspecies paratuberculosis or MAP) and antiglycan antibodies (such as antilaminaribioside carbohydrate antibody or ALCA, antichitobioside carbohydrate antibody or ACCA and antimannobioside carbohydrate antibody or AMCA). Les marqueurs de référence de dépistage des MICI restent à ce jour les anticorps anti-Saccharomyces cerevisiae (ASCA) et les ANCA atypiques de type NANA. Leur intérêt réside également dans leur valeur prédictive dans les colites indéterminées, dans leur valeur pronostique au cours des MICI et plus récemment dans leur association à des phénotypes particuliers, notamment dans la maladie de Crohn. Cet article fait également le point sur la place des autres marqueurs des MICI dont les anticorps antipancréas exocrine et des marqueurs plus récents comme les anticorps anti-antigènes microbiens (antiporine OmpC de l’Escherichia coli, antiséquence I2 du Pseudomonas fluorescens, antiprotéine CBir1 dérivée de la flagelline, anti-OmpW du Bacteroides caccae, anti-MAP pour Mycobacterium avium subspecies paratuberculosis) et les anticorps antiglycans (antimannobioside, antilaminaribioside et antichitobioside).

Interference of Wegener's granulomatosis autoantibodies with neutrophil Proteinase 3 activity.

Classic anti-neutrophil cytoplasmic autoantibodies (C-ANCA) are disease-specific markers of Wegener's granulomatosis (WG). The possible pathogenetic role of these autoantibodies, which are directed against Proteinase 3 (PR3), is not yet clear. We studied the effect of C-ANCA on PR3 proteolytic activity and on the complexation of PR3 with alpha 1-antitrypsin (alpha 1AT). C-ANCA IgG from eight patients with active WG significantly inhibited PR3 proteolytic activity, particularly towards elastin (median 84.2% inhibition). C-ANCA IgG significantly inhibited the complexation of PR3 with alpha 1AT (median 58.8% inhibition). Moreover, addition of purified PR3 to C-ANCA-positive sera from WG patients yielded less complexes with alpha 1AT (median 44.8%) compared with sera containing perinuclear anti-neutrophil cytoplasmic autoantibodies (P-ANCA) or ANCA-negative sera. These findings indicate the existence of a hitherto unknown property of C-ANCA, which may be of importance in the pathogenesis of WG.

Perinuclear Antineutrophil Cytoplasmic Autoantibodies and Anti-Saccharomyces CerevisiaeAntibodies as Serological Markers Are Not Specific in the Identification of Crohn’s Disease and Ulcerative Colitis

To evaluate the diagnostic accuracy of perinuclear antineutrophil cytoplasmic autoantibodies (pANCAs) and anti-Saccharomyces cerevisiae antibodies (ASCAs), as single agents and in combination, for the diagnosis of Crohn's disease (CD) and ulcerative colitis (UC), including in cases of indeterminate colitis (IC). The sera from a total of 98 patients were studied: 77 with CD, 16 with UC and five with IC. The medical records of these patients were reviewed for disease diagnosis, demographic data, and patient symptoms and medications. ELISAs were utilized to detect the presence of ASCAs and deoxyribonuclease-sensitive pANCAs, and these results were then compared with the patients' clinical data. For UC, a positive pANCA test alone provided a sensitivity of 50% and a specificity of 82%. For CD, a positive ASCA test alone provided a sensitivity of 40% and a specificity of 100%. A combination of pANCA-positive and ASCA-negative results showed a sensitivity of 50% and specificity of 90% for the diagnosis of UC. Similarly, the combination of ASCA-positive and pANCA-negative results provided a sensitivity and specificity of 32% and 100% for the diagnosis of CD, respectively. Interestingly, 80% of IC patients showed serology results consistent with UC. Although this combination of serological markers provides a diagnostic tool with generally high specificities, the low sensitivities of these serological markers, most notably in terms of CD, preclude the possibility that they can replace the tools currently used for inflammatory bowel disease diagnosis and management. It is possible, however, that these serological markers may prove beneficial in the management of IC.

Occupational exposure in ANCA-positive patients: A case-control study

Antineutrophil cytoplasmic autoantibodies (ANCA) are valuable biomarkers for the diagnosis and follow-up of small vessel vasculitis. The role of ANCA has not yet been fully established, but genetic, infectious, and/or environmental factors may increase susceptibility to these diseases. We performed an epidemiologic study to investigate whether the presence of ANCA was associated with silica or any other form of occupational exposure, regardless of the underlying disease. All consecutive ANCA-positive patients recorded at the institution's Laboratory of Immunology between 1990 and 2000 were included. Patients hospitalized in a unit of internal medicine matched for age and gender were selected as controls (two controls/case). Qualitative and semiquantitative professional exposure and smoking habits were analyzed by five experts blind to the diagnosis. Univariate analysis showed that patients who reported dust exposure had a 2.6 greater risk of being ANCA-positive (P= 0.007) (odds ratio 2.6; 95% CI 1.3 to 5.3) and individuals with professional exposure to silica had a 3.4 higher risk of being ANCA-positive (P= 0.03) (odds ratio 3.4; 95% CI 1.1 to 9.9). None of the other environmental factors or smoking habits were different between ANCA-positive patients and controls. There was no difference in silica exposure between patients with cytoplasmic ANCA (c-ANCA), perinuclear ANCA (p-ANCA), or atypical ANCA. Semiquantitative analysis showed a dose effect of silica exposure with a nearly sevenfold greater risk of being ANCA-positive compared to controls (P= 0.02) (odds ratio 6.9; 95% CI 1.3 to 35.1). These results support the hypothesis that the presence of ANCA in plasma might at least partially be related to occupational exposure.

Anti‐Saccharomyces cerevisiae and perinuclear anti‐neutrophil cytoplasmic antibodies in coeliac disease before and after gluten‐free diet

Anti-Saccharomyces cerevisiae and perinuclear anti-neutrophil cytoplasmic autoantibodies are markers of Crohn's disease and ulcerative colitis respectively. To determine the prevalence of anti-S. cerevisiae and perinuclear anti-neutrophil cytoplasmic autoantibodies in a large series of coeliac disease patients before and after gluten free diet, and to correlate anti-S. cerevisiae-positivity with intestinal mucosal damage. One hundred and five consecutive coeliac disease patients and 141 controls (22 ulcerative colitis, 24 Crohn's disease, 30 primary sclerosing cholangitis, 15 postenteritis syndrome, 50 blood donors) were tested for anti-S. cerevisiae by enzyme-linked immunosorbent assay and for perinuclear anti-neutrophil cytoplasmic autoantibodies by indirect immunofluorescence. In coeliac disease anti-S. cerevisiae (immunoglobulin G and/or immunoglobulin A) were slightly less frequent (59%) than in Crohn's disease (75%, P = 0.16) and significantly more frequent than in ulcerative colitis (27%), primary sclerosing cholangitis (30%), postenteritis syndrome (26%) and blood donors (4%) (P = 0.009, P = 0.0002, P = 0.025, P < 0.0001). No correlation was found between anti-S. cerevisiae and degree of mucosal damage. Perinuclear anti-neutrophil cytoplasmic autoantibodies were detected only in one coeliac. After gluten free diet the disappearance of anti-S. cerevisiae-immunoglobulin A (93%) was more frequent than that of immunoglobulin G (17%, P = 0.0001); perinuclear anti-neutrophil cytoplasmic autoantibodies disappeared in the only coeliac positive at diagnosis. More than half of untreated coeliacs are anti-S. cerevisiae-positive irrespective of the severity of mucosal damage. Differently from immunoglobulin A, anti-S. cerevisiae-immunoglobulin G persisted in more than 80% after gluten free diet. The high prevalence of anti-S. cerevisiae in coeliac disease suggests that they may be the effect of a non-specific immune response in course of chronic small bowel disease.

The clinical and pathological characteristics of Chinese elderly patients with anti-neutrophil cytoplasmic autoantibodies associated small vessel vasculitis

Background. Anti-neutrophil cytoplasmic autoantibodies (ANCA) are serological markers of ANCA-associated small vessel vasculitis (AASV) which is one of common autoimmune diseases in Caucasian elderly population. Objective. To analyze the clinical and pathological characteristics of Chinese elderly patients with AASV. Methods. One-hundred forty one Chinese patients with AASV over 65 years old, diagnosed between 1997 and 2001 in the Institute of Nephrology of Peking University First Hospital, were retrospectively studied and their clinical and pathological data were analyzed. Results. Patients diagnosed with AASV patient increased chronologically with the yearly ratio in 2000 and 2001 significantly ( P<0.05) higher than that in 1998 and before. Of the 141 patients, 72 were male and 69 were female with an average age of 68.2 years. 13 of the 141 were cytoplasmic ANCA (cANCA) positive and all recognized proteinase 3 (PR3). The other 128 were perinuclear ANCA (pANCA) positive and 120/128 recognized MPO, 8/128 recognized both PR3 and MPO. Less than 50% of the patients were correctly diagnosed within 3 months. Clinically, 78% of the patients had fever and fatigue, 52.5% had body weight loss, 96.4% had kidney involvement, of which 75% had elevated serum creatinine and 30.8% had acute renal failure. 76.6% had lung involvement, over half of them had hemoptysis or lung infiltrates. Other clinical manifestations included arthralgia (48.2%), muscle pain (39.7%), gastrointestinal symptoms (39.7%), eye involvement (28.3%) and ENT involvement (31.2%). In laboratory examinations, 94.4% of the patients had anemia, 62.4% had increased WBC count, 93.6% had increased ESR and 55.1% had increased CRP. Conclusions. More and more patients with AASV were diagnosed in Chinese elderly. Kidney was the most vulnerable organ to be involved and lung was the most important extra-renal organ to be affected. For elderly patients with multi-organ damage, an ANCA test should be performed in order to make an early diagnosis and start therapy in time.

A Case of Microscopic Polyangiitis with Diffuse Alveolar Hemorrhage

Diffuse alveolar hemorrhage is a rare but serious and frequently life-threatening complication of a variety of conditions. The first goal in the management of patients with diffuse alveolar hemorrhage is to achieve or preserve stability of the respiratory status. Subsequently, the differential diagnosis is aimed at the identification of a remediable cause of the alveolar hemorrhage. The most common causes of diffuse alveolar hemorrhage with glomerulonephritis are microscopic polyangiitis and Wegener's granulomatosis, followed by Goodpasture syndrome and systemic lupus erythematosus. Microscopic polyangiitis (MPA) is a distinct systemic small vessle vasculitis affecting small sized vessels with few or no immune deposits and with no granulomatosus inflammation. The disease may involve multiple organs such as kidney, lung, skin, joint, muscle, gastrointestinal tract, eye, and nervous system. MPA is strongly associated with antineutrophil cytoplasmic autoantibody (ANCA) that is a useful serological diagnostic marker for the most common form of necrotizing vasculitis. Our report concerns a case of microscopic polyangiitis with diffuse alveolar hemorrhage in a 54-year-old man. He was admitted to our hospital due to dyspnea upon exertion and recurrent hemoptysis. Laboratory findings showed hematuria, proteinuria and deterioration of renal function. In the chest CT scan, diffuse ground glass appearance was seen in both lower lungs. A lung biopsy revealed small vessel vasculitis with intraalveolar hemorrhage and showed a positive reaction to against perinuclear ANCA. The patient was treated with prednisolone and cyclophosphamide. Chest infiltration decreased and hemoptysis and hypoxia improved. He is still being followed up in our hospital with a low dose of prednisolone.