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Anti neutrophil cytoplasmic antibody

Shinya Kawamoto, Ryo Koda, Atsunori Yoshino, Tetsuro Takeda, Yoshihiko Ueda
Medullary cystic kidney disease (MCKD) is a hereditary disease associated with bilateral medullary polycysts and interstitial fibrosis. MCKD is typically associated with slowly progressive renal dysfunction. We herein report two rare elderly cases with enlarged kidneys and rapidly progressive renal dysfunction without myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA), PR3-ANCA, or anti-glomerular basement membrane (GBM) antibodies. Renal biopsies revealed extensive tubular dilatation and atrophy with interstitial fibrosis consistent with MCKD...
2016: Internal Medicine
Haruka Chino, Eri Hagiwara, Hideya Kitamura, Tomohisa Baba, Hideaki Yamakawa, Tamiko Takemura, Takashi Ogura
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...
October 6, 2016: Respiration; International Review of Thoracic Diseases
Shinichiro Okauchi, Tomohiro Tamura, Katsunori Kagohashi, Mio Kawaguchi, Hiroaki Satoh
A 71-year-old woman with arthralgia and lung fibrosis was referred to Mito Kyodo General Hospital (Mito, Japan) for a mass, which was incidentally observed on a chest radiograph. The chest computed tomography scan demonstrated fibrotic lesions in the lower lobes of the lung and a nodule in the left upper lobe. The serum levels of myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) and proteinase 3 (PR3)-ANCA were 60.3 and 7.5 U/ml, respectively. A transbronchial biopsy obtained from the nodule in the left upper lobe of the lung revealed a lung adenocarcinoma and the patient underwent standard upper lobectomy of the left lung...
October 2016: Biomedical Reports
Wildéa Lice de Carvalho Jennings Pereira, Edna Maria Vissoci Reiche, Ana Paula Kallaur, Sayonara Rangel Oliveira, Andréa Name Colado Simão, Marcell Alysson Batisti Lozovoy, Lucas José Vaz Schiavão, Paula Raquel do Vale Pascoal Rodrigues, Daniela Frizon Alfieri, Tamires Flauzino, Damacio Ramón Kaimen-Maciel
OBJECTIVE: The aim of this study was to report the frequency of autoimmune disorders and autoantibodies in 22 patients with neuromyelitis optica (NMO), as well as whether the seropositivity for autoantibodies differs between anti-aquaporin 4 (AQP4) positive and AQP4 negative NMO patients. METHODS: Demographic, medical records, and a profile of autoantibodies were evaluated in 22 NMO patients, including AQP4, anti-thyroid-stimulating hormone receptor, antinuclear antibodies (ANA), anti-thyroperoxidase (anti-TPO), anti-thyroglobulin (anti-Tg), anti-double-stranded DNA, anti-neutrophil cytoplasmic, anti-cyclic citrullinate peptide, rheumatoid factor, anti-SSA/Ro, anti-SSB/La, anti-Smith antibodies (anti-Sm), anti-ribonucleoprotein, anti-nucleosome, and anti-Scl70...
October 3, 2016: Acta Neuropsychiatrica
Stavros Giaglis, Sinuhe Hahn, Paul Hasler
Neutrophil extracellular trap (NET) formation represents a form of cell death distinct from apoptosis or necrosis, by which invading pathogens are simultaneously entangled and potentially eliminated. Increased NET formation is observed in systemic lupus erythematosus (SLE), rheumatoid arthritis, antineutrophil cytoplasmic antibody-associated small vessel vasculitis, antiphospholipid antibody syndrome (APS), and psoriasis. NETs contribute to the pathogenesis of autoimmunity by exposing cryptic autoepitopes, which may facilitate the generation of autoantibodies, induce the production of interferons, and activate the complement cascade...
2016: Frontiers in Pediatrics
Anne Laure Bernat, Etienne Lefevre, Damien Sène, Philippe Herman, Homa Adle Biassette, Sebastien Froelich
Granulomatosis with polyangitis (Wegener's disease) is a rare ANCA (anti-neutrophil cytoplasmic antibody) positive vasculitis that seldom involves brain and meninges. We herein present a case of a 35-year-old woman presenting with granulomatosis with polyangitis involving the brain and the meninges. The histological diagnosis required a complete resection of the lesion. Despite the possibility of post operative wound infection surgery was mandatory to ascertain the diagnostic and to treat locally.
September 23, 2016: World Neurosurgery
Konstantinos Thomas, Dimitrios Vassilopoulos
PURPOSE OF REVIEW: To review recent evidence for infection rates in patients with systemic vasculitides, the role of specific infectious agents in the pathogenesis of vasculitis and recent breakthroughs in the treatment of virus-associated vasculitides. RECENT FINDINGS: In well designed recent studies, infections were found to be common during the first 6-12 months in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) and giant cell arteritis (GCA) and to contribute significantly to increased mortality during this period...
September 21, 2016: Current Opinion in Rheumatology
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
Huifang Liu, Jiachuan Xiong, Jun Zhang, Ying Zhang, Ling Nie, Yiqin Wang, Jinghong Zhao
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a life-threatening condition that causes renal failure. Multiple myeloma (MM) is a malignant proliferation of monoclonal plasma cells in the blood that can also cause renal failure. The two diseases have high morbidity and mortality rates in the elderly, with a poor prognosis. A 64-year-old female presented to Xinqiao Hospital (Chonqing, China) with fatigue and a poor appetite that had been apparent for 6 weeks. Laboratory tests revealed a serum creatinine level of 10...
September 2016: Oncology Letters
Yue Chen, Ping Li, Chunli Cui, Aihong Yuan, Kun Zhang, Chen Yu
OBJECTIVE: To explore the clinical characteristics, renal histopathological spectrum and prognostic factors of biopsy-proven kidney diseases in the elderly. METHODS: A retrospective observational study was conducted in elderly patients who had received renal biopsies. Demographic, clinical and pathological data at the time of the biopsy were collected from the medical records. Follow-up records and prognostic factors were studied. RESULTS: The elderly (≥60 years) accounted for 74 of 434 (17...
September 5, 2016: Journal of International Medical Research
Gabriel Horta-Baas, Esteban Meza-Zempoaltecatl, Mario Pérez-Cristóbal, Leonor Adriana Barile-Fabris
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a systemic necrotizing vasculitis, which affects small and medium sized blood vessels and is often associated with cytoplasmic anti-neutrophil cytoplasmic antibodies (ANCA). Inflammatory pseudotumor is a rare condition characterized by the appearance of a mass lesion that mimics a malignant tumor both clinically and on imaging studies, but that is thought to have an inflammatory/reactive pathogenesis. We report a patient with a GPA which was originally diagnosed as malignancy...
July 2016: Gaceta Médica de México
Koji Tamai, Harukazu Yoshimatsu, Toshiharu Saito, Hirofumi Matsuoka, Nobuhiko Okada, Yasuko Koma, Akiko Otsuka, Nao Oda, Sayaka Inoue, Sachie Kume, Yujiro Suzuki
BACKGROUND: Autoimmune involvement in the pathogenesis of asthma and chronic obstructive pulmonary disease (COPD) has been proposed, and autoantibodies are a hallmark of autoimmunity. This study aimed to compare the autoantibody profiles of asthma and COPD, and the relationship between autoantibodies and features of these diseases. METHODS: We recruited 110 asthma patients and 92 COPD patients for a prospective study. Six autoantibody types were evaluated: antinuclear antibody, anti-cytoplasmic antibodies, rheumatoid factor, anti-cyclic citrullinated peptide antibody, myeloperoxidase-anti-neutrophil cytoplasmic autoantibody (MPO-ANCA) and proteinase 3-ANCA...
August 31, 2016: Allergology International: Official Journal of the Japanese Society of Allergology
Bárbara Nery Porto, Renato Tetelbom Stein
Neutrophil extracellular traps (NETs) arise from the release of granular and nuclear contents of neutrophils in the extracellular space in response to different classes of microorganisms, soluble factors, and host molecules. NETs are composed by decondensed chromatin fibers coated with antimicrobial granular and cytoplasmic proteins, such as myeloperoxidase, neutrophil elastase (NE), and α-defensins. Besides being expressed on NET fibers, NE and MPO also regulate NET formation. Furthermore, histone deimination by peptidylarginine deiminase 4 (PAD4) is a central step to NET formation...
2016: Frontiers in Immunology
Manfred Relle, Bernd Föhr, Federica Fasola, Andreas Schwarting
Granulomatosis with polyangiitis (GPA) is a severe autoimmune disease and one of the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although its etiology and pathophysiology are still widely unknown, it is accepted that infections, environmental factors, epigenetic modifications, and a genetic predisposition provide the basis for this systemic disorder. GPA typically evolves into two phases: an initial phase characterized by ear, nose and throat (ENT) manifestations, such as chronic sinusitis and otitis, ulceration of the oral cavity and pharynx, as well as pulmonary nodules and a severe generalized phase, defined by the occurrence of rapidly progressive glomerulonephritis, pulmonary hemorrhage, and arthritis...
August 21, 2016: Molecular and Cellular Probes
Johannes R Hov, Kirsten M Boberg, Eli Taraldsrud, Mette Vesterhus, Maria Boyadzhieva, Inger Camilla Solberg, Erik Schrumpf, Morten H Vatn, Benedicte A Lie, Øyvind Molberg, Tom H Karlsen
BACKGROUND&AIMS: The strongest genetic risk factors in primary sclerosing cholangitis (PSC) are encoded in the HLA complex. Anti-neutrophil cytoplasmic antibodies (ANCA) have been reported in up to 94% of PSC patients, but their clinical significance and immunogenetic basis are ill-defined. We aimed to characterize clinical and genetic associations of ANCA in PSC. METHODS: ANCA were analyzed with indirect immunofluorescence in 241 Norwegian PSC patients. HLA-B and HLA-DRB1 genotyping was performed in the patients and in 368 healthy controls...
August 25, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Giovanni M Rossi, Giacomo Emmi, Domenico Corradi, Maria L Urban, Federica Maritati, Federica Landini, Paola Galli, Alessandra Palmisano, Augusto Vaglio
Mediastinal fibrosis is a rare disease characterised by fibrous proliferation in the mediastinum. It can be idiopathic or secondary to several conditions such as infections and malignancies. Anecdotal reports have described idiopathic mediastinal fibrosis (IMF) in association with other fibro-inflammatory or autoimmune diseases. We report nine new IMF cases recently seen at our Fibro-Inflammatory Disease Clinic and reviewed the IMF cases reported in the English language literature throughout 2006-2016. The purposes of our literature search were to assess the frequency of the association between IMF and other immune-mediated disorders and to analyse which disorders most often coexist with IMF...
August 23, 2016: Clinical Reviews in Allergy & Immunology
Jian Hao, Tie-Gang Lv, Chen Wang, Li-Ping Xu, Jian-Rong Zhao
BACKGROUND: Macrophage migration inhibitory factor (MIF) is an inflammatory mediator released by macrophages that is central to the innate immune system, with an upstream role in the inflammatory cascade. MIF is one of the most important pathogenic factors in the development of the autoimmune diseases. In the current study, we investigated the role of MIF in anti-neutrophil cytoplasmic antibody (ANCA)-induced neutrophil activation. METHODS: Plasma levels of MIF from 31 patients with active ANCA-associated vasculitis (AAV) were analyzed by ELISA...
August 17, 2016: Human Immunology
Marco Cattalini, Jessica Galli, Laura Andreoli, Ivana Olivieri, Giada Ariaudo, Micaela Fredi, Simona Orcesi, Angela Tincani, Elisa Fazzi
PURPOSE: The purpose of this study was to explore the presence of autoimmune manifestations and characterize the autoantibody production in a cohort of patients with Aicardi-Goutières syndrome (AGS). METHODS: Seventeen patients with a genetically-confirmed diagnosis of AGS were recruited. At the time of enrollment, past medical and family history was reviewed, looking for possible signs or symptoms of autoimmune disorders. Blood samples were taken, for the detection of a panel of autoantibodies: anti-nuclear, anti-double-stranded-DNA, anti-nucleosome, anti-extractable nuclear antigens, anti-cardiolipin IgG/IgM, anti-β2glycoprotein I IgG/IgM, and anti-neutrophil cytoplasmic...
October 2016: Journal of Clinical Immunology
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
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