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ANCA-associated vasculitis

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https://www.readbyqxmd.com/read/29682388/granulomatosis-with-polyangiitis-localized-in-the-greater-omentum
#1
Ryuji Komine, Takashi Kobayashi, Hiro Uehara, Keisuke Minamimura, Kazuhiko Mori, Toru Hirata, Atsushi Shimizu, Masaya Mori
Granulomatosis with polyangiitis (GPA) is known as anti-neutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis and typically manifests as pulmonary-renal syndrome, but the disease is not limited to pulmonary or renal systems. The inflammation can involve whole body organs. In addition, the ANCA titer does not always become positive. Here, we describe the case of a 91-year-old man who presented with umbilical pain and fever of unknown origin. Only the increased computed tomography value of the greater omentum suggested intra-abdominal inflammation; however, serological examinations, including the ANCA level, could not reveal the focus or cause of symptoms...
2018: Case Reports in Surgery
https://www.readbyqxmd.com/read/29679168/late-restoration-of-renal-function-in-patients-with-severe-anca-associated-glomerulonephritis-who-were-dialysis-dependent-at-presentation
#2
Tian-Tian Ma, Yi-Ran Liu, Min Chen, Ming-Hui Zhao
In antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), many patients with severe renal dysfunction are on dialysis at presentation. Among those who got rid of dialysis finally, renal restoration happened mostly within the first 3 months of treatment. However, in clinical practice, we occasionally encountered patients who got rid of dialysis after 3 months. The current study investigated clinical and pathological features of AAV patients with so-called delayed renal function restoration...
April 20, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29670798/anca-vasculitis-and-hemophagocytic-lymphohistiocytosis-following-a-fecal-microbiota-transplant
#3
Adam Amlani, Amy Bromley, Aurore Fifi-Mah
A 69-year-old female with antisynthetase syndrome, a history of multiple recurrent infections, and documented previous negative titres for anti-neutrophil cystoplasmic antibody (ANCA) suddenly developed a de novo MPO-ANCA-associated glomerulonephritis three weeks after a fecal microbiota transplantation (FMT) for recurrent Clostridium difficile infections. Six months following her FMT and less than two weeks following treatment for urosepsis, she developed severe cholestasis, a markedly elevated ferritin and hypertriglyceridemia...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29657868/outcomes-of-hydralazine-induced-renal-vasculitis
#4
Homa Timlin, Jason E Liebowitz, Khusleen Jaggi, Duvuru Geetha
Objective: Hydralazine has been implicated as an etiologic agent for lupus-like syndrome and vasculitis. Hydralazine-induced vasculitis frequently affects the kidney, but the long-term renal outcomes in these patients have not yet been studied. Methods: Patients who had a diagnosis of ANCA-associated vasculitis (AAV) and were on hydralazine at the time of AAV diagnosis were included in this retrospective cohort study. Clinical and laboratory data were obtained from the review of medical records...
March 2018: European Journal of Rheumatology
https://www.readbyqxmd.com/read/29648530/clinical-and-histological-features-of-antineutrophil-cytoplasmic-antibody-associated-vasculitis-related-to-antithyroid-drugs
#5
Jumpei Hasegawa, Junichi Hoshino, Akinari Sekine, Noriko Hayami, Tatsuya Suwabe, Keiichi Sumida, Koki Mise, Toshiharu Ueno, Masayuki Yamanouchi, Ryo Hazue, Naoki Sawa, Kenichi Ohashi, Takeshi Fujii, Kenmei Takaichi, Yoshifumi Ubara
BACKGROUND: Antithyroid drugs such as propylthiouracil and methimazole have been reported to cause antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), but little is known about long-term outcomes. MATERIALS AND METHODS: We identified AAV patients who underwent renal biopsy and retrospectively assessed their clinical and histological findings. Patients with AAV who had received propylthiouracil or methimazole were defined as having antithyroid drug-associated AAV (ATD-AAV), and the other patients were defined as having primary AAV...
April 12, 2018: Clinical Nephrology
https://www.readbyqxmd.com/read/29643885/anca-associated-vasculitis-in-patients-from-saudi-arabia
#6
Abdurhman Saud Al Arfaj, Najma Khalil
Objective: To explore clinical and laboratory features, therapy and outcome of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) patients from our tertiary care center. Methods: This study of AAV patients seen in Rheumatology clinics at King Khalid University hospital (KKUH), King Saud University, Riyadh during the period 1990-2014 was carried out retrospectively. Demographic, clinical, haematological and immunological parameters along with therapy, complications and outcome were retrieved from patients' medical charts...
January 2018: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/29621352/hypocomplementemia-is-associated-with-worse-renal-survival-in-anca-positive-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis
#7
Samuel Deshayes, Achille Aouba, Kathy Khoy, Delphine Mariotte, Thierry Lobbedez, Nicolas Martin Silva
Recent data suggest the existence of a complement alternative pathway activation in the pathogenesis of antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV), a condition that remains poorly understood. This study aims to assess the clinical characteristics and outcomes of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) patients with regard to their plasma complement levels at diagnosis. A retrospective monocentric study carried out at Caen University Hospital led to the identification of proteinase-3- or myeloperoxidase-ANCA-positive GPA and MPA patients from January 2000 to June 2016 and from September 2011 to June 2016, respectively...
2018: PloS One
https://www.readbyqxmd.com/read/29616045/complement-factor-h-inhibits-anti-neutrophil-cytoplasmic-autoantibody-induced-neutrophil-activation-by-interacting-with-neutrophils
#8
Su-Fang Chen, Feng-Mei Wang, Zhi-Ying Li, Feng Yu, Min Chen, Ming-Hui Zhao
Our previous study demonstrated that plasma levels of complement factor H (FH) were inversely associated with the disease activity of patients with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). In addition to serving as an inhibitor of the alternative complement pathway, there is increasing evidence demonstrating direct regulatory roles of FH on several cell types. Here, we investigated the role of FH in the process of ANCA-mediated activation of neutrophils and neutrophil-endothelium interaction...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29614541/-anca-associated-vasculitis-recent-methodological-advances-for-anca-detection
#9
Elena Csernok
Anti-neutrophil cytoplasmic antibodies (ANCA) play a central role in the diagnostic and pathogenesis of patients with small vessel vasculitis, so called ANCA-associated vasculitis (AAV). ANCA in these diseases are almost always directed against proteinase 3 (PR3-ANCA) and myeloperoxidase (MPO-ANCA). Most laboratories worldwide use as standard the indirect immunofluorescence technique (IFT), on human neutrophils, to screen for ANCA, and then confirm positive IFT results with antigen specific immunoassyas for PR3- and MPO-ANCA...
April 2018: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29611402/delta-neutrophil-index-is-associated-with-vasculitis-activity-and-risk-of-relapse-in-anca-associated-vasculitis
#10
Juyoung Yoo, Sung Soo Ahn, Seung Min Jung, Jason Jungsik Song, Yong Beom Park, Sang Won Lee
PURPOSE: Delta neutrophil index (DNI) represents the immature granulocytes count associated with neutrophil-consumption. We investigated whether DNI might be associated with Birmingham vasculitis activity score (BVAS) at diagnosis and could predict relapse during the follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). MATERIALS AND METHODS: We reviewed the medical records of 97 patients having DNI results. Twenty patients had granulomatosis with polyangiitis (GPA), 58 had microscopic polyangiitis (MPA), and 19 had eosinophilic GPA (EGPA)...
May 2018: Yonsei Medical Journal
https://www.readbyqxmd.com/read/29606398/excessive-neutrophil-extracellular-trap-formation-in-anca-associated-vasculitis-is-independent-of-anca
#11
Tineke Kraaij, Sylvia W A Kamerling, Laura S van Dam, Jaap A Bakker, Ingeborg M Bajema, Theresa Page, Francesca Brunini, Charles D Pusey, Rene E M Toes, Hans U Scherer, Ton J Rabelink, Cees van Kooten, Y K Onno Teng
Neutrophil extracellular traps (NETs) are auto-antigenic strands of extracellular DNA covered with myeloperoxidase (MPO) and proteinase3 (PR3) that can be a source for the formation of anti-neutrophil cytoplasmic autoantibodies (ANCAs). The presence of NETs was recently demonstrated in renal tissue of patients with ANCA-associated vasculitis (AAV). NET formation was enhanced in AAV, suggesting that MPO-ANCA could trigger NET formation, supporting a vicious circle placing NETs in the center of AAV pathogenesis...
March 30, 2018: Kidney International
https://www.readbyqxmd.com/read/29594143/a-case-of-crescentic-glomerulonephritis-complicated-with-hypocomplementemic-urticarial-vasculitis-syndrome-and-anca-associated-vasculitis
#12
Marenao Tanaka, Norihito Moniwa, Tomohiro Mita, Toshiyuki Tobisawa, Tamaki Matsumoto, Atsushi Mochizuki, Tomohisa Yamashita, Toshiyuki Yano, Masato Furuhashi, Tetsuji Miura
Systemic urticaria in a 64-year-old woman was diagnosed as leukocytoclastic vasculitis by a punch biopsy of the skin. Her physical findings improved after prescription of prednisolone at a dose of 20 mg/day, but the skin rash relapsed with renal dysfunction, proteinuria, and hematuria when the dose of prednisolone was reduced over a period of 9 months to 1 mg/day. She was admitted to our institute for further examination, when urinary protein and plasma creatinine levels were 0.8 g/day and 1.7 mg/dL, respectively...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29592975/fulminant-hepatic-failure-in-the-setting-of-progressive-anca-associated-vasculitis-associated-with-a-rare-alpha-1-antitrypsin-phenotype-piee
#13
Ronald Reilkoff, Laurel Stephenson
Abnormalities in alpha-1 antitrypsin (AAT) proteins are risk factors for human disease. While the most common is AAT deficiency, a genetic disorder associated with chronic obstructive pulmonary disease, additional disorders associated with AAT abnormalities are increasingly recognised. We describe a middle-aged woman who presented with fulminant hepatic and multiorgan failure. Evaluation revealed the patient to have a rare AAT phenotype PiEE. Her clinical presentation was consistent with antineutrophilic cytoplasmic antibody-associated vasculitis, and her history suggested features of panniculitis...
March 28, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29588079/risk-of-cardiovascular-disease-and-venous-thromboembolism-among-patients-with-incident-anca-associated-vasculitis-a-20-year-population-based-cohort-study
#14
Alvise Berti, Eric L Matteson, Cynthia S Crowson, Ulrich Specks, Divi Cornec
OBJECTIVE: To assess the cardiovascular disease (CVD) and venous thromboembolism (VTE) risks among patients with newly diagnosed antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). PATIENTS AND METHODS: A population-based incident AAV cohort of 58 patients diagnosed between 1996 and 2015 in Olmsted County, MN, was identified by medical record review. For each patient, 3 age- and sex-matched non-AAV comparators were randomly selected from the same population and assigned an index date corresponding to the AAV incidence date...
March 24, 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29564565/imatinib-mesylate-use-in-refractory-eosinophilic-granulomatosis-with-polyangiitis-a-literature-review-and-a-case-report
#15
REVIEW
Tatiana V Beketova, Mikhail Y Volkov, Evgeniy A Naryshkin, Tatiana M Novoselova, Evgeniy L Nasonov
Recent advances in pharmacology have greatly expanded the drug repertoire for treatment of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic disorder, a type of the ANCA-associated vasculitis. Important features of this disease are eosinophilia and anti-myeloperoxidase ANCA presence in around 30-70% of patients. Primary therapy of EGPA includes steroids and cytotoxic drugs, e.g., cyclophosphamide, azathioprine, or methotrexate...
March 21, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29560002/a-rare-case-of-eosinophilic-granulomatosis-with-polyangiitis-associated-with-cryoglobulinemia-presenting-with-a-bullous-skin-eruption-of-the-lower-limbs
#16
D D K Abeyaratne, C Liyanapathirana, C L Fonseka, P W M C S B Wijekoon
Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis with multisystem involvement. It is characterized with asthma, eosinophilia, and renal and peripheral nervous system involvement. However, EGPA presenting with bullous skin eruption is an uncommon dermatological manifestation. We report a rare case of EGPA overlapped with mixed essential cryoglobulinemia presenting with a bullous skin eruption...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29557539/birmingham-vasculitis-activity-and-chest-manifestation-at-diagnosis-can-predict-hospitalised-infection-in-anca-associated-vasculitis
#17
Juyoung Yoo, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
We investigated the development rate and time, risk factors, predictors, and aetiologies of hospitalised infection in Korean patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We retrospectively reviewed the medical records of 154 patients with AAV. Hospitalised infection was considered only when patients were admitted for serious infection related to AAV or AAV treatment. The gap-time was defined as the period from diagnosis to the first hospitalised infection or to the last visit for uninfected patients...
March 20, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29548309/acantholytic-squamous-cell-carcinoma-of-the-lung-with-marked-lymphogenous-metastases-and-high-titers-of-myeloperoxidase-antineutrophil-cytoplasmic-antibodies-a-case-report
#18
Kenji Yorita, Kazuya Tsuji, Yoko Takano, Naoto Kuroda, Kei Sakamoto, Kaoru Arii, Yukio Yoshimoto, Kimiko Nakatani, Satoshi Ito
BACKGROUND: Acantholytic squamous cell carcinoma (ASQCC), histologically characterized by intercellular bridge loosening, is recognized as a rare variant of squamous cell carcinoma (SQCC). ASQCC may demonstrate a worse prognosis than conventional SQCC. Pulmonary ASQCC is particularly rare; its biological behavior and prognostic data have not been reported. CASE PRESENTATION: We report the clinical and autopsy findings of a 71-year-old Japanese man with pulmonary ASQCC...
March 16, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29545453/increased-prevalence-of-thyroid-disease-in-patients-with-antineutrophil-cytoplasmic-antibodies-associated-vasculitis
#19
Maria Prendecki, Leyre Martin, Anisha Tanna, Marilina Antonelou, Charles D Pusey
OBJECTIVE: Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) has been linked with thyroid disease as a result of antithyroid medications. We assessed the prevalence of thyroid disease in our patients with AAV. METHODS: Clinical records of 279 patients with AAV diagnosed between 1991 and 2014 were analyzed. RESULTS: Thyroid disease was identified in 21.5% of patients, but only 2 had previously received propylthiouracil...
March 15, 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/29532268/monoclonal-gammopathy-in-rheumatic-diseases
#20
Yue Yang, Long Chen, Yuan Jia, Yang Liu, Lei Wen, Yaoxian Liang, Yuan An, Shi Chen, Yin Su, Zhanguo Li
To analyze the clinical spectrum, laboratory characteristics, and outcomes of monoclonal gammopathy (MG) in patients with rheumatic diseases. Screening for the presence of MG was performed in 872 inpatients with rheumatic diseases from January 2010 to July 2017. A total of 41 patients were enrolled. Their clinical and biological features in addition to outcomes were described. For each patient with primary Sjögren syndrome (pSS), 2 age- and sex-matched pSS patients without MG were selected as controls. Risk factors for the presence of MG and malignant hematological neoplasias were assessed...
March 13, 2018: Clinical Rheumatology
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