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microscopic polyangiitis

Rosaria Talarico, Simone Barsotti, Elena Elefante, Chiara Baldini, Chiara Tani, Marta Mosca
PURPOSE OF REVIEW: The purpose of this review is to provide a critical analysis of the recent literature on this topic, with particular focus on the most relevant studies published over the last year. RECENT FINDINGS: Many studies are published every year on the diagnosis, pathogenesis and treatment of pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). The main subjects covered by this article are the pathogenesis, diagnosis and clinical aspects of lung involvement in ANCA-associated vasculitis and non-ANCA-associated vasculitis...
October 17, 2016: Current Opinion in Rheumatology
Cyril Garrouste, Dany Anglicheau, Nassim Kamar, Claire Bachelier, Joseph Rivalan, Bruno Pereira, Sophie Caillard, Julien Aniort, Philippe Gatault, Martin Soubrier, Johnny Sayegh, Charlotte Colosio, Anthony Buisson, Eric Thervet, Nicolas Bouvier, Anne Elisabeth Heng
Anti-tumor necrosis factor-α (TNFα) therapy has improved the prognosis of many chronic inflammatory diseases. It appears to be well-tolerated by liver-transplant patients. However, their use and their safety in kidney-transplant patients have yet to be determined.In this retrospective study, we identified 16 adult kidney-transplant patients aged 46.5 years (34-51.8) who received anti-TNFα therapy from 7 kidney transplantation centers. The indications for this treatment included: chronic inflammatory bowel disease (n = 8), inflammatory arthritis (n = 5), AA amyloidosis (n = 1), psoriasis (n = 1), and microscopic polyangiitis (n = 1)...
October 2016: Medicine (Baltimore)
Christian Pagnoux
Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Renal-limited ANCA-associated vasculitides can be considered the fourth entity. Despite their rarity and still unknown cause(s), research pertaining to ANCA-associated vasculitides has been very active over the past decades. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) has been supported using several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) has not been as strongly demonstrated...
September 2016: Eur J Rheumatol
Amandine Tubery, Françoise Fortenfant, Bernard Combe, Isabelle Abreu, Xavier Bossuyt, Pascale Chretien, Sophie Desplat-Jégo, Nicole Fabien, Sophie Hue, Catherine Johanet, Daniela Lakomy, Thierry Vincent, Claire I Daïen
We report here the case of a 50-years-old man treated for mixed connective tissue disease (MCTD) positive for anti-U1 ribonucleoprotein (U1RNP) antibodies who secondarily developed a granulomatosis with polyangiitis (GPA) associated with anti-proteinase 3 anti-neutrophil cytoplasmic antibodies (PR3-ANCA). We then evaluated the frequency of the association between anti-U1RNP and anti-PR3-ANCA antibodies by a systematic retrospective study in ten European hospitals. Overall, out of 11,921 samples analyzed for both auto-antibodies, 18 cases of anti-U1RNP and anti-PR3-ANCA double positivity were found and only one patient presented with both MCTD and GPA symptoms...
September 12, 2016: Immunologic Research
K Gleghorn, J Wilson, M Wilkerson
Rituximab is an anti-CD20 monoclonal antibody with considerable potential in dermatology due to an increase in off-label indications. Chronic graft-versus-host disease and pemphigus vulgaris are two of the most promising indications for off-label use of rituximab. It is a generally safe alternative that should be considered when traditional therapy with corticosteroids or immunosuppressants has failed or caused significant intolerance. Currently, rituximab is only FDA-approved for treatment of follicular and diffuse large B-cell non-Hodgkin's lymphoma, rheumatoid arthritis, chronic lymphocytic leukemia, granulomatosis with polyangiitis (formerly Wegener's granulomatosis) and microscopic polyangiitis...
September 2016: Skin Therapy Letter
Sophia Lionaki, John N Boletis
BACKGROUND: Pauci-immune glomerulonephritis is the most common cause of aggressive glomerulonephritis and occurs as a renal-limited disease or as a component of systemic necrotizing small-vessel vasculitis. It is characterized by paucity of staining for immunoglobulins, by immunofluorescence along with fibrinoid necrosis and crescent formation by light microscopy, while the vast majority of patients have anti-neutrophil cytoplasmic antibodies (ANCA) in their circulation, which also participate in the pathogenesis of the disease...
March 2016: Kidney Diseases
Zhi-Ying Li, Tian-Tian Ma, Min Chen, Ming-Hui Zhao
BACKGROUND: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Myeloperoxidase (MPO) and proteinase 3 (PR3) are the main antigens for ANCA. AAV is a common multisystem autoimmune disease and most of the studies on AAV have been conducted in Western countries. Nowadays in China many efforts are made to investigate this disease. SUMMARY: This review highlights the progress in the prevalence, management and outcomes of AAV in Chinese patients...
March 2016: Kidney Diseases
Hong Xiao, Peiqi Hu, Ronald J Falk, J Charles Jennette
BACKGROUND: Antineutrophil cytoplasmic autoantibodies (ANCA) are associated with a spectrum of necrotizing vasculitis including granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited necrotizing and crescentic glomerulonephritis. Clinical observations and in vitro and in vivo experimental evidence strongly indicate that ANCA are pathogenic. SUMMARY: The etiology and pathogenesis of ANCA-associated vasculitis (AAV) are multifactorial, with contributions from genetic factors, environmental exposures, infections, characteristics of the innate and adaptive immune system, and the intensity and duration of the injury...
March 2016: Kidney Diseases
Q Zhang, H Q Zhou, Y H Li, J Guo, P H Xu, M H Lu, X L Liu, L Wang, S G Li
OBJECTIVE: To investigate the clinical features of patients with antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), and to explore the disease activity of AAV patients and the relationship with prognosis. METHODS: The clinical data of 46 cases of AAV patients in the First Affiliated Hospital of PLA General Hospital were analyzed retrospectively.The clinical and laboratory features of each clinical subtype were compared.The disease activity of AAV and the relationship between disease activity and prognosis were evaluated...
July 19, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Divi Cornec, Emilie Cornec-Le Gall, Fernando C Fervenza, Ulrich Specks
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a heterogeneous group of rare syndromes characterized by necrotizing inflammation of small and medium-sized blood vessels and the presence of ANCAs. Several clinicopathological classification systems exist that aim to define homogeneous groups among patients with AAV, the main syndromes being microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Two main types of ANCA can be detected in patients with AAV...
October 2016: Nature Reviews. Rheumatology
Kian-Guan Lee, Su-Hooi Teo, Cynthia Lim, Alwin Loh, Viswanath Chidambaram, Jason Choo
Although the use of current immunosuppressive regimens has significantly improved the outcomes of autoimmune renal diseases, infectious complications remain an important clinical concern. Cytomegalovirus (CMV) infection has been shown to be one of the major causes of mortality in this group of patients. We report two cases of renal vasculitis (Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA)) that developed into severe gastrointestinal CMV disease and manifested with massive small bowel bleeding, resulting in an eventual fatal outcome for one of the patients...
September 2016: Clinical Nephrology
E M Miloslavsky, N Lu, S Unizony, H K Choi, P A Merkel, P Seo, R Spiera, C A Langford, G S Hoffman, C G M Kallenberg, E W St Clair, N K Tchao, F Fervenza, P A Monach, U Specks, J H Stone
Objective To examine the impact of antineutrophil cytoplasmic antibody (ANCA) type and ANCA-associated vasculitis (AAV) diagnosis on demographic features, disease manifestations, and clinical outcomes. We focused on patients that account for the differences between ANCA type and disease type classifications: anti-myeloperoxidase (MPO) ANCA-positive and ANCA-negative patients with granulomatosis with polyangiitis (GPA). Methods Pooled analysis of the Wegener's Granlomatosis Etanercept Trial (WGET) and the Rituximab in AAV (RAVE) trial comparing MPO-ANCA+ GPA and ANCA-negative GPA patients to proteinase 3 (PR3) ANCA+ GPA and MPO-ANCA+ microscopic polyangiitis (MPA) patients...
July 18, 2016: Arthritis & Rheumatology
Yoko Matsuda, Hideki Hamayasu, Atsuko Seki, Keisuke Nonaka, Tan Wang, Takumi Matsumoto, Yoshitomo Hamano, Hiroyuki Sumikura, Toshio Kumasaka, Shigeo Murayama, Akihiko Ishizu, Akira Shimizu, Takahiko Sugihara, Tomio Arai
A 76-year-old man was admitted with general fatigue, weight loss, fever, headache, renal failure, and a high serum level of myeloperoxidase-antineutrophil cytoplasmic antibody. Biopsy revealed citrullinated histone H3 (citH3)-positive neutrophils adherent to the temporal artery endothelium. Three days after completing pulse steroid therapy, he suffered from a sudden disturbance of consciousness and died. On autopsy, the kidneys showed the most severe vasculitis with dense infiltration of citH3-positive neutrophils...
August 2016: Pathology International
Yoshiyuki Abe, Naoto Tamura, Kwang-Seok Yang, Joe Matsuoka, Takayuki Kon, Ken Yamaji, Hiroshi Hashimoto, Hiroshi Tsuda, Yoshinari Takasaki
PURPOSE: To determine mortality and its predictive factors in elderly Japanese patients with severe microscopic polyangiitis (MPA). METHOD: This retrospective single-center study determined the mortality of 52 patients with MPA who were admitted to our geriatric medical center from 2002 to 2014. The variables at baseline, including patient demographics, clinical characteristics, and treatment, were analyzed for their association with mortality. RESULT: Mean age at onset of MPA was 73...
July 11, 2016: Modern Rheumatology
Jan H Schirmer, Marvin N Wright, Kristine Herrmann, Martin Laudien, Bernhard Nölle, Eva Reinhold-Keller, Jan P Bremer, Frank Moosig, Julia U Holle
Objectives Comparing phenotype, clinical course and outcome of Myeloperoxidase(MPO)-ANCA associated Granulomatosis with polyangiitis (GPA) with GPA associated with Proteinase 3(PR3)-ANCA and with Microscopic Polyangiitis (MPA) associated with MPO-ANCA. Methods We characterized all MPO-ANCA-positive patients classified as GPA by the European Medicines Agency algorithm who attended our center in a retrospective chart review. A second cohort with GPA(PR3-ANCA) patients matched for age and gender was characterized...
June 22, 2016: Arthritis & Rheumatology
Tomohiko Shibata, Kumiko Tonooka, Kosei Tsuchida, Hirofumi Mitomi, Toshiko Shibata, Naooki Katsuyama
  Trimethoprim-sulfamethoxazole (TMP/SMX) treatment for pneumocystis pneumonia (PCP) in patients with autoimmune diseases who developed PCP was conducted in a retrospective study of the following: dosage, frequency of side effects and persistence rate of TMP/SMX and prognosis of patients. Seven patients (two males and five females, mean age: 72 years) were hospitalized between April 1, 2013 and August 31, 2015, and their underlying diseases were rheumatoid arthritis (six patients) and microscopic polyangiitis (one patient)...
2016: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
Rafael Bonamichi-Santos, Mariana Castells
Due to the increase in utilization of chemotherapies and antibodies, drug hypersensitivity reactions have increased dramatically worldwide, preventing the use of first-line therapies and impacting patients' survival and quality of life. Some of the more frequently used medications in cancer include taxanes for ovarian, lung, breast, and prostate cancers. Monoclonal antibodies are used in the treatment of neoplastic, autoimmune, and inflammatory diseases, and their clinical applications are becoming broader...
June 8, 2016: Clinical Reviews in Allergy & Immunology
Manish Rathi, Benzeeta Pinto, Aadhaar Dhooria, Vinay Sagar, Tarun Mittal, Roopa Rajan, Varun Dhir, Susheel Kumar, Kusum Sharma, Ritambhra Nada, Surjit Singh, Ranjana Walker Minz, Aman Sharma
BACKGROUND: Renal involvement is a serious complication of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). We describe the pattern of renal involvement and its correlation with outcomes. PATIENTS AND METHODS: Medical records of 92 patients seen in rheumatology clinic and diagnosed as AAV between January 2007 and June 2014 were analysed. Patients were classified as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA) and undifferentiated AAV...
September 2016: International Urology and Nephrology
Hiroshi Maruyama, Kouichi Hirayama, Miho Nagai, Itaru Ebihara, Homare Shimohata, Masaki Kobayashi
Type 17 T-helper (Th17) cells have been suggested to be involved in the pathogenesis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Th17 cell proliferation is promoted by tumor necrosis factor (TNF)-like ligand 1A (TL1A), which binds to death receptor 3 (DR3) expressed on Th17 cells. Decoy receptor 3 (DcR3) is known to block the TL1A-DR3 pathway by binding TL1A. To evaluate the Th17-TL1A systems as disease activity markers in AAV, we investigated the serum levels of TL1A and DcR3 in AAV patients...
October 2016: Clinical Rheumatology
Shuzo Sato, Makiko Yashiro, Naoki Matsuoka, Manabu Uematsu, Tomoyuki Asano, Hiroko Kobayashi, Hiroshi Watanabe, Hiromasa Ohira
Mixed connective tissue disease (MCTD) is characterized by a combination of clinical features of systemic lupus erythematosus, systemic sclerosis, and polymyositis with elevated antibodies to U1 small nuclear ribonucleoprotein (U1-RNP). MCTD is often accompanied by interstitial lung disease as pulmonary involvement. On the other hand, microscopic polyangiitis (MPA) is a systemic autoimmune disease characterized by the inflammation of small vessels (arterioles, capillaries, and venules) mainly affecting the lung and kidney...
2016: Tohoku Journal of Experimental Medicine
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