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Takashi Ogura - One of the best experts on this subject based on the ideXlab platform.
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clinical features of usual interstitial pneumonia with anti neutrophil Cytoplasmic Antibody in comparison with idiopathic pulmonary fibrosis
Respirology, 2016Co-Authors: Chiaki Hosoda, Tamiko Takemura, Tomohisa Baba, Eri Hagiwara, Hiroyuki Ito, Norikazu Matsuo, Hideya Kitamura, Tae Iwasawa, Koji Okudela, Takashi OguraAbstract:Background and objective Myeloperoxidase Anti-Neutrophil Cytoplasmic Antibody (MPO-ANCA) is occasionally positive in patients with usual interstitial pneumonia (UIP). However, the differences from idiopathic pulmonary fibrosis (IPF/UIP) have not been well documented. We aimed to clarify the clinical, radiological and pathological features of UIP associated with MPO-ANCA (ANCA/UIP). Methods We retrospectively reviewed the medical records of 12 consecutive ANCA/UIP patients not manifesting microscopic polyangiitis and 108 IPF/UIP patients with no autoantibodies, both diagnosed by surgical lung biopsy. Results There was no significant difference in clinical background, laboratory results and pulmonary function tests between ANCA/UIP patients and IPF/UIP patients except for the percentage of bronchoalveolar lavage neutrophils. HRCT showed subpleural reticulation in both groups. Increased attenuation around honeycombing and cysts was significantly observed in ANCA/UIP. Pathologically, ANCA/UIP had more prominent inflammatory cell infiltration, lymphoid follicles with germinal centres and cellular bronchiolitis. During the disease course, three of 12 patients (25%) developed microscopic polyangiitis. Immunosuppressive treatment tended to be more effective in ANCA/UIP patients, and the survival time in ANCA/UIP patients tended to be longer than those with IPF/UIP. Conclusion ANCA/UIP may be distinguishable from IPF/UIP with a combination of HRCT findings of increased attenuation around honeycombing and cysts and some of the characteristic pathological findings. In contrast to IPF/UIP, immunosuppressive treatment could be a therapeutic option for ANCA/UIP.
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myeloperoxidase anti neutrophil Cytoplasmic Antibody positive interstitial pneumonia associated with granulomatosis with polyangiitis diagnosed by surgical lung biopsy
Respiration, 2016Co-Authors: Haruka Chino, Tamiko Takemura, Tomohisa Baba, Eri Hagiwara, Hideya Kitamura, Hideaki Yamakawa, Takashi OguraAbstract:Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis that often involves the lung. However, interstitial pneumonia (IP) is rarely seen in GPA patients. We herein report 3 cases of IP associated with GPA diagnosed by surgical lung biopsy. High-resolution CT showed uniform subpleural reticular opacity with traction bronchiectasis. Biopsies from all 3 patients revealed neutrophilic capillaritis, microabscesses with giant cells, and coexisting histological findings of usual IP pattern or fibrosing nonspecific IP pattern. All 3 patients had elevated levels of serum myeloperoxidase Anti-Neutrophil Cytoplasmic Antibody (MPO-ANCA), but not proteinase 3-ANCA. We diagnosed GPA and treated with corticosteroid and cyclophosphamide. Follow-up CT showed improvement of the lesions in all patients. Surgical lung biopsy specimens which revealed GPA enabled us to conduct the most suitable therapy. This report indicates the importance of surgical lung biopsy for differentiating idiopathic IPs from GPA-associated IP and suggests a relationship between MPO-ANCA and IP in GPA.
David W Mudge - One of the best experts on this subject based on the ideXlab platform.
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patient was subsequently discharged from hospital after a rapid clinical improvement. JOURNAL OF MEDICAL
2016Co-Authors: Case Reports, Justin Keasberry, J Frazier, Nicole M Isbel, Carolyn Van Eps, Kimberley Oliver, David W MudgeAbstract:Full list of author information is available at the end of the articleConclusion: Hydralazine-induced Anti-Neutrophil Cytoplasmic Antibody-positive renal vasculitis is a rare adverse effect and can present with a severe vasculitic syndrome with multiple organ involvement. Features of this association include the presence of high titres of anti-myeloperoxidase-Anti-Neutrophil Cytoplasmic Antibody with multi-antigenicity, positive anti-histone antibodies and the lack of immunoglobulin and complement deposition histopathogically. A rash that is characteristic of Sweet’s syndrome has also been described as an association. Prompt cessation of hydralazine may be sufficient to reverse disease activity but immunosuppression may be needed for definite treatment
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hydralazine induced anti neutrophil Cytoplasmic Antibody positive renal vasculitis presenting with a vasculitic syndrome acute nephritis and a puzzling skin rash a case report
Journal of Medical Case Reports, 2013Co-Authors: Justin Keasberry, J Frazier, Nicole M Isbel, Carolyn Van Eps, Kimberley Oliver, David W MudgeAbstract:Anti-Neutrophil Cytoplasmic Antibody-associated vasculitis has been associated with many drugs and it is a relatively rare side effect of the antihypertensive drug hydralazine. The diagnosis and management of patients who have Anti-Neutrophil Cytoplasmic Antibody-associated vasculitis may be challenging because of its relative infrequency, variability of clinical expression and changing nomenclature. The spectrum of Anti-Neutrophil Cytoplasmic Antibody-associated vasculitis is wide and can be fatal. This case documents a 62-year-old woman who presented with hydralazine-induced Anti-Neutrophil Cytoplasmic Antibody-positive renal vasculitis with a puzzling cutaneous rash. We report a rare case of hydralazine-induced Anti-Neutrophil Cytoplasmic Antibody-associated vasculitis in a 62-year-old Caucasian woman who presented with a vasculitic syndrome with a sore throat, mouth ulcers and otalgia after several months of constitutional symptoms. She then proceeded to develop a rash over her right lower limb. Clinically, the rash had features to suggest Sweet’s syndrome, but also had some appearances consistent with embolic phenomena and did not have the appearance of palpable purpure usually associated with cutaneous vasculitis. Differential diagnoses were hydralazine-associated Sweet’s syndrome, streptococcal-induced cutaneous eruption or an unrelated contact dermatitis. A midstream urine sample detected glomerular blood cells in the setting of Anti-Neutrophil Cytoplasmic Antibody-positive renal vasculitis and Streptococcus pyogenes bacteremia. A renal biopsy revealed a pauci-immune, focally necrotizing glomerulonephritis with small crescents. Her skin biopsy revealed a heavy neutrophil infiltrate involving the full thickness of the dermis with no evidence of a leucocytoclastic vasculitis, but was non-specific. She was initially commenced on intravenous lincomycin for her bloodstream infection and subsequently commenced on immunosuppression after cessation of hydralazine. The patient was subsequently discharged from hospital after a rapid clinical improvement. Hydralazine-induced Anti-Neutrophil Cytoplasmic Antibody-positive renal vasculitis is a rare adverse effect and can present with a severe vasculitic syndrome with multiple organ involvement. Features of this association include the presence of high titres of anti-myeloperoxidase-Anti-Neutrophil Cytoplasmic Antibody with multi-antigenicity, positive anti-histone antibodies and the lack of immunoglobulin and complement deposition histopathogically. A rash that is characteristic of Sweet’s syndrome has also been described as an association. Prompt cessation of hydralazine may be sufficient to reverse disease activity but immunosuppression may be needed for definite treatment.
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hydralazine induced anti neutrophil Cytoplasmic Antibody positive renal vasculitis presenting with a vasculitic syndrome acute nephritis and a puzzling skin rash a case report
Journal of Medical Case Reports, 2013Co-Authors: Justin Keasberry, J Frazier, Nicole M Isbel, Kimberley Oliver, David W MudgeAbstract:Introduction Anti-Neutrophil Cytoplasmic Antibody-associated vasculitis has been associated with many drugs and it is a relatively rare side effect of the antihypertensive drug hydralazine. The diagnosis and management of patients who have Anti-Neutrophil Cytoplasmic Antibody-associated vasculitis may be challenging because of its relative infrequency, variability of clinical expression and changing nomenclature. The spectrum of Anti-Neutrophil Cytoplasmic Antibody-associated vasculitis is wide and can be fatal. This case documents a 62-year-old woman who presented with hydralazine-induced Anti-Neutrophil Cytoplasmic Antibody-positive renal vasculitis with a puzzling cutaneous rash.
Shigeto Kobayashi - One of the best experts on this subject based on the ideXlab platform.
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association of ets1 polymorphism with granulomatosis with polyangiitis and proteinase 3 anti neutrophil Cytoplasmic Antibody positive vasculitis in a japanese population
Journal of Human Genetics, 2018Co-Authors: Aya Kawasaki, Keita Yamashita, Fumio Hirano, Ken Ei Sada, Daisuke Tsukui, Yuya Kondo, Yoshitaka Kimura, Kurumi Asako, Shigeto Kobayashi, Hidehiro YamadaAbstract:ETS proto-oncogene 1, transcription factor (ETS1) is involved in various immune responses. Genome-wide association studies on systemic lupus erythematosus in Chinese populations identified the association of ETS1 polymorphism in 3' untranslated region, rs1128334A, which was associated with lower ETS1 expression. In view of substantial sharing of susceptibility genes across multiple autoimmune diseases, we examined whether ETS1 is associated with a rare autoimmune rheumatic disease, Anti-Neutrophil Cytoplasmic Antibody (ANCA)-associated vasculitis (AAV). Association of rs1128334 was tested in 466 Japanese patients with AAV and 1099 healthy controls by logistic regression analysis under the additive model. AAV patients were classified into 285 microscopic polyangiitis (MPA), 92 granulomatosis with polyangiitis (GPA), 56 eosinophilic GPA, and 33 unclassifiable AAV, according to the European Medicines Agency (EMEA) algorithm. Among the patients, 376 were positive for MPO-ANCA and 62 for PR3-ANCA. When the patients were classified according to the EMEA classification, rs1128334A allele was significantly increased in GPA (P = 0.0060, P c = 0.030, odds ratio (OR), 1.54; 95% confidence interval (CI), 1.13-2.10). With respect to the ANCA specificity, significant association was observed in PR3-ANCA positive AAV (P = 0.0042, P c = 0.021, OR, 1.72; 95% CI, 1.19-2.49). In conclusion, ETS1 polymorphism was suggested to be associated with GPA and PR3-ANCA positive AAV in a Japanese population.
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comparison of the epidemiology of anti neutrophil Cytoplasmic Antibody associated vasculitis between japan and the uk
Rheumatology, 2011Co-Authors: Shouichi Fujimoto, Shigeto Kobayashi, Richard A Watts, Kazuo Suzuki, David Jayne, David G I Scott, Hiroshi Hashimoto, Hiroyuki NunoiAbstract:Objectives. The epidemiological manifestations of ANCA-associated vasculitis (AAV) differ geographically. However, there have been no prospective studies comparing the incidence of AAV between Japan and Europe over the same time period using the same case definitions. Methods. The incidence of AAV was determined by a population-based method in Miyazaki prefecture, Japan, and Norfolk, UK, between 2005 and 2009. Patients with AAV were defined and classified according to the European Medicines Agency (EMEA) algorithm. Results. The number of incident cases of AAV in Japan and the UK were 86 and 50, respectively, and the average annual incidence over the 5-year period was 22.6/million (95% CI 19.1, 26.2) and 21.8/million (95% CI 12.6, 30.9) in Japan and the UK, respectively. The average age was higher in patients in Japan than in patients in the UK [mean (median), 69.7 (72) vs 60.5 (61) years]. Microscopic polyangiitis (MPA) was the predominant subtype in Japan (83%), while granulomatosis with polyangiitis (Wegener’s) was more frequent in the UK (66%). As for the pattern of ANCA positivity, >80% of Japanese patients were pANCA/ MPO positive, whereas two-thirds of UK patients were cANCA/PR3 positive. Renal involvement in MPA was very common in both countries, but was much less common in granulomatosis with polyangiitis in Japan compared with the UK. Conclusion. There was no major difference in AAV incidence between Japan and the UK, but this prospective study found MPA and MPO-ANCA to be more common in Japan and granulomatosis with polyangiitis and PR3-ANCA to be more common in the UK, in line with earlier reports.
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anti neutrophil Cytoplasmic Antibody associated vasculitis large vessel vasculitis and kawasaki disease in japan
Kidney & Blood Pressure Research, 2010Co-Authors: Shigeto Kobayashi, Shouichi Fujimoto, Kei Takahashi, Kazuo SuzukiAbstract:Based on studies comparing the prevalence of Anti-Neutrophil Cytoplasmic Antibody (ANCA)-associated vasculitis (AAV) between Japan and Europe, we have learned that the difference may be due to genetic background and environmental factors, but not to diagnosis or ELISA system for myeloperoxidase and proteinase-3 ANCA. In Japan, microscopic polyangiitis is the most common among AAV, but Wegener's granulomatosis was present in less than 2 per million patients. Also, one study from Hokkaido reported only 16 patients in a 27-year time frame. A recent retrospective study of renal vasculitis between 2000 and 2004 from Miyazaki prefecture in Japan reported an incidence of microscopic polyangiitis of 14.8 per million, but no patients with Wegener's granulomatosis or Churg-Strauss syndrome. In the present review, we focus on ANCA-related vasculitis in Japan: (1) AAV and large vessel vasculitis - Takayasu's arteritis and giant cell arteritis; (2) primary renal vasculitis; (3) epitopes of myeloperoxidase-ANCA in vasculitis in the Japanese population and comparison of ANCA-ELISA systems in Japan and Europe, and finally (4) children with vasculitis in Japan involving Kawasaki disease - a systemic vasculitis.
L Wilson - One of the best experts on this subject based on the ideXlab platform.
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coexistent anti neutrophil Cytoplasmic Antibody and antiglomerular basement membrane Antibody associated disease report of six cases
Modern Pathology, 1993Co-Authors: S M Bonsib, James A Goeken, John D Kemp, P Chandran, C Shadur, L WilsonAbstract:From a series of 95 patients biopsied for rapidly progressive glomerulonephritis, twelve patients were identified with anti-glomerular basement membrane-mediated renal disease who were also tested for antineutrophil Cytoplasmic Antibody (ANCA). Six patients had both anti-glomerular basement membrane and ANCA antibodies. Three of the latter six patients had significant extrarenal disease, including severe hemoptysis, while the remaining three patients had only renal disease. The three patients with extrarenal disease had either a myeloperoxidase-positive perinuclear-ANCA (two patients) or a proteinase-3-positive Cytoplasmic-ANCA (one patient). Two patients with renal disease alone had a myeloperoxidase-negative and proteinase-3-negative perinuclear-ANCA, and one patient had a proteinase-positive Cytoplasmic-ANCA. Renal biopsy in all six patients showed a severe necrotizing and crescentic glomerulonephritis involving 94 to 100% of glomeruli. Renal arteritis was also noted in one perinuclear-ANCA patient. Despite aggressive therapy with steroids, cyclophosphamide, and plasma exchange, two of the six double-Antibody patients died and four are on dialysis. We conclude that ANCA is commonly present in anti-glomerular basement membrane-associated disease and believe that this observation may have implications in the serologic evaluation of ANCA- and anti-glomerular basement membrane-positive patients.
Xavier Bosch - One of the best experts on this subject based on the ideXlab platform.
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anti neutrophil Cytoplasmic Antibody pathogenesis in small vessel vasculitis an update
American Journal of Pathology, 2009Co-Authors: Jose A Gomezpuerta, Xavier BoschAbstract:Vasculitides associated with serum positivity for Anti-Neutrophil Cytoplasmic antibodies (ANCAs) that affect small- to medium-sized vessels are commonly known as ANCA-associated vasculitis (AAV) and include Wegener’s granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Evidence derived from both in vitro studies and recent animal models points to a pathogenic role of ANCAs in AAV. In 2002, the first in vivo breakthrough in the pathogenesis of ANCAs showed that mouse ANCAs against myeloperoxidase (MPO) led to intrinsic pauci-immune renal vasculitis in mice. In 2004, a report using both in vitro and in vivo studies proposed that proteinase 3 (PR3)-directed autoimmunity involved the complementary peptide of PR3 (cPR3), which is encoded by the antisense strand of the PR3 gene. The last breakthrough came in October 2008 with a previously undescribed molecular explanation for the origin and development of injury in pauci-immune renal vasculitis, with potential clinical implications. This report showed that infection by fimbriated bacteria may trigger cross-reactive autoimmunity to a previously characterized ANCA antigen, lysosomal membrane protein-2, which is contained in the same vesicles that harbor MPO and PR3. Infection by fimbriated bacteria resulted in the production of autoantibodies, which activated neutrophils and killed human microvascular endothelium in vitro and caused renal vasculitis in rats. Although the evidence for a pathogenic role of ANCAs, mainly MPO-ANCAs, is striking, various questions remain unanswered. Understanding the key pathogenic mechanisms of AAV may provide a safer, more rational therapeutic approach than the traditional (ie, corticosteroids and immunosuppressants) treatment strategy.