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Anti neutrophil cytoplasmic antibody

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https://www.readbyqxmd.com/read/29333079/type-1-autoimmune-hepatitis-presenting-with-severe-autoimmune-neutropenia
#1
Petros Doumtsis, Theodora Oikonomou, Ioannis Goulis, Kaliopi Zachou, George Dalekos, Evangelos Cholongitas
Autoimmune hepatitis (AIH) is a progressive, chronic liver disease characterized by unresolving hepatocellular inflammation of autoimmune origin. The clinical spectrum may vary from asymptomatic presentation, to non-specific symptoms such as fatigue, arthralgias, nausea and abdominal pain, to acute severe liver disease. AIH is characterized by the presence of interface hepatitis and portal plasma cell infiltration on histological examination, hypergammaglobulinemia, and positive autoantibodies. AIH is associated with other autoimmune diseases and its course is often accompanied by various non-specific hematological disorders...
January 2018: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
https://www.readbyqxmd.com/read/29322343/rituximab-for-anti-neutrophil-cytoplasmic-antibodies-associated-vasculitis-experience-of-a-single-center-and-systematic-review-of-non-randomized-studies
#2
Gizem Ayan, Sinem Nihal Esatoglu, Gulen Hatemi, Serdal Ugurlu, Emire Seyahi, Melike Melikoglu, Izzet Fresko, Huri Ozdogan, Sebahattin Yurdakul, Vedat Hamuryudan
Rituximab (RTX) is becoming a standard treatment for patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) but heterogeneity exists regarding its use. We present our uncontrolled experience with RTX in patients with refractory AAV and also the results of a systematic review of non-randomized studies on RTX in AAV patients. We retrospectively reviewed the records of AAV patients treated with RTX following an inadequate response to immunosuppressives between 2011 and 2015. The systematic review covered all English articles listed in PubMed until June 2017...
January 10, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29321928/lung-cancer-development-in-the-patient-with-granulomatosis-with-polyangiitis-during-long-term-treatment-with-cyclophosphamide-first-documented-case
#3
Midori Toriyama, Etsuko Tagaya, Tomoko Yamamoto, Mitsuko Kondo, Yoji Nagashima, Jun Tamaoki
A 65-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) at the age of 47, when cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) serology was positive, and he had multiple nodular shadows in both lungs. He had been treated with prednisolone, cyclophosphamide (CPA) and plasma exchange. At the age of 64, a nodular shadow was newly detected in the right lower lung field and serum tumour marker increased. Subsequent positron emission tomography/computed tomography scan demonstrated accumulations of fluorodexyglucose (FDG) in the same area, mediastinum lymph nodes, thoracic wall, right iliac bone, and right retroperitoneum...
February 2018: Respirology Case Reports
https://www.readbyqxmd.com/read/29315316/the-c3ar-promotes-macrophage-infiltration-and-regulates-anca-production-but-does-not-affect-glomerular-injury-in-experimental-anti-myeloperoxidase-glomerulonephritis
#4
Jonathan Dick, Poh-Yi Gan, A Richard Kitching, Stephen R Holdsworth
The anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides are autoimmune diseases associated with significant morbidity and mortality. They often affect the kidney causing rapidly progressive glomerulonephritis. While signalling by complement anaphylatoxin C5a though the C5a receptor is important in this disease, the role of the anaphylatoxin C3a signalling via the C3a receptor (C3aR) is not known. Using two different murine models of anti-myeloperoxidase (MPO) glomerulonephritis, one mediated by passive transfer of anti-MPO antibodies, the other by cell-mediated immunity, we found that the C3aR did not alter histological disease severity...
2018: PloS One
https://www.readbyqxmd.com/read/29287586/the-impact-of-intravenous-methylprednisolone-pulses-on-renal-survival-in-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-with-severe-renal-injury-patients-a-retrospective-study
#5
Yanhong Ma, Fei Han, Liangliang Chen, Hongya Wang, Haidongqing Han, Binfeng Yu, Ying Xu, Jianghua Chen
BACKGROUND: High-dose methylprednisolone pulses were one of the main treatments for anti-neutrophil cytoplasmic autoantibody (ANCA) associated vasculitides (AAV) but had obvious side effects. We aimed to know the impact on renal survival and identify the prognostic factors of this treatment in Chinese AAV patients with severe renal involvement. METHODS: One hundred and eleven AAV patients with an estimated glomerular filtration rate (eGFR) of 10ml/min/1.73 m2 or less at admission were included...
December 29, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/29285157/serum-creatinine-level-and-esr-values-associated-to-clinical-pathology-types-and-prognosis-of-patients-with-renal-injury-caused-by-anca-associated-vasculitis
#6
Hongda Liang, Miaomiao Xin, Lei Zhao, Liqin Wang, Mingshu Sun, Jibo Wang
The correlation between serum creatinine and erythrocyte sedimentation rate (ESR) values and clinical pathology and prognosis in patients with renal injury caused by anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis were analyzed. Eighty-six patients with ANCA-associated vasculitis (AAV) treated in the Affiliated Hospital of Qingdao University were enrolled in the study. Patients were assigned into an elderly group (n=45) or a non-elderly group (n=41) according to age. The serum creatinine (Scr) level was measured via the sarcosine oxidase method, and the erythrocyte sedimentation rate (ESR) was measured using the full-automatic ESR analyzer; the relationship between Scr and ESR values and the pathology type of patients was statistically analyzed...
December 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29279515/anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-aav-restricted-to-the-limbs-a-case-report
#7
Yoshie Ojima, Kinya Sawada, Hiroshi Fujii, Tsuyoshi Shirai, Ayako Saito, Saeko Kagaya, Satoshi Aoki, Yoichi Takeuchi, Tomonori Ishii, Tasuku Nagasawa
A previously healthy 58-year-old man was admitted for muscle pain and weakness (manual muscle testing [MMT] of 4/4 for upper and lower limbs). We detected elevated levels of inflammatory makers and PR3-anti-neutrophil cytoplasmic antibody (ANCA). Subsequently, the muscle weakness rapidly progressed to an MMT of 2 for all limbs. Magnetic resonance imaging indicated muscle edema, and the CK level increased to 29,998 U/L. mPSL and cyclophosphamide pulse therapy improved the patient symptoms. MMT recovered to 4 for all limbs...
December 27, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29274703/clinical-significance-of-neutrophil-lymphocyte-ratio-in-patients-with-granulomatosis-with-polyangiitis
#8
Nouran M Abaza, Eiman M Abd El-Latif, Tamer A Gheita
OBJECTIVE: To determine to neutrophil-to-lymphocyte ratio (NLR) in granulomatosis with polyangiitis (GPA) patients and to study its relation to disease manifestations and activity. METHODS: The study included 44 GPA patients and 44 matched age and sex controls. Full history taking, thorough clinical examination with more attention to ocular examination, laboratory and radiological investigations were considered. Disease activity was assessed using the Birmingham Vasculitis Activity Score (BVAS)...
December 20, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/29274562/increased-platelet-activation-occurs-in-cystic-fibrosis-patients-and-correlates-to-clinical-status
#9
Ulrika Lindberg, Lisbeth Svensson, Thomas Hellmark, Mårten Segelmark, Oonagh Shannon
Cystic fibrosis (CF) is an inflammatory lung disease. Platelets have an emerging role in inflammation, however previous studies of platelet activation in CF have generated conflicting results. In this study, we determined platelet function in CF patients and correlated platelet activation to establish clinical and laboratory parameters. Twenty-two patients, aged 20.7 to 54.4 (mean 34.0, SD 9.45) years and with a mean FEV1%pred (forced expiratory volume in one second, % of predicted) of 72 (SD 21.4, range 32-110) were recruited...
December 19, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/29260039/intracerebral-hemorrhage-after-iv-tpa-for-stroke-as-early-symptom-of-anca-associated-vasculitis
#10
Neda Zarghami Esfahani, Daniel M Anderson, Connie Pieper, Harold P Adams
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare diseases characterized by a necrotizing small-vessel vasculitis and circulating ANCA that comprise granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Acute ischemic stroke (AIS) can be a manifestation of central nervous system (CNS) involvement in these diseases. Furthermore, intracerebral hemorrhage (ICH) is a potential complication of these necrotizing vasculitides...
December 2017: ENeurologicalSci
https://www.readbyqxmd.com/read/29260006/high-pr3-anca-positivity-in-a-patient-with-chronic-inflammatory-demyelinating-polyneuropathy
#11
Masanori Kurihara, Yu Kurata, Izumi Sugimoto, Yuki Hatanaka, Yasuhisa Sakurai
Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) is reported to be highly specific to vasculitis compared to myeloperoxidase (MPO)-ANCA. We report a case of a 19-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP) with high PR3-ANCA positivity. The patient responded well to intravenous immunoglobulin plus oral steroid, and showed no signs of systemic vasculitis during the subsequent 10 months of follow-up. Our present case suggests that CIDP may accompany high PR3-ANCA levels, which should be differentiated from axonal neuropathy due to vasculitis...
March 2017: ENeurologicalSci
https://www.readbyqxmd.com/read/29243051/diagnostic-outcome-and-indications-for-testing-in-patients-with-positive-anca-at-a-canadian-tertiary-care-centre
#12
Cyrus Chehroudi, Ronald A Booth, Nataliya Milman
INTRODUCTION/OBJECTIVE: With widespread availability of anti-neutrophil cytoplasmic antibody (ANCA) testing, interpreting positive results has become increasingly challenging. Here, we conducted a retrospective study to evaluate indications for testing and diagnosis of patients with positive ANCA. METHODS: Positive ANCA tests (immunofluorescence or immunoassay) performed between April 2014 and March 2015 were identified using the Ottawa Hospital (TOH) laboratory information system...
December 14, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29241626/c5a-receptor-1-promotes-autoimmunity-neutrophil-dysfunction-and-injury-in-experimental-anti-myeloperoxidase-glomerulonephritis
#13
Jonathan Dick, Poh-Yi Gan, Sharon L Ford, Dragana Odobasic, Maliha A Alikhan, Sven H Loosen, Pam Hall, Clare L Westhorpe, Anqi Li, Joshua D Ooi, Trent M Woodruff, Charles R Mackay, A Richard Kitching, Michael J Hickey, Stephen R Holdsworth
The prospects for complement-targeted therapy in ANCA-associated vasculitis have been enhanced by a recent clinical trial in which C5a receptor 1 (C5aR1) inhibition safely replaced glucocorticoids in induction treatment. C5aR1 primes neutrophils for activation by anti-neutrophil cytoplasmic antibody (ANCA) and is therefore required in models of glomerulonephritis induced by anti-myeloperoxidase antibody. Although humoral and cellular autoimmunity play essential roles in ANCA-associated vasculitis, a role for C5aR1 in these responses has not been described...
December 11, 2017: Kidney International
https://www.readbyqxmd.com/read/29239918/anti-neutrophil-cytoplasmic-antibody-negative-central-nervous-system-granulomatosis-with-polyangiitis-and-its-clinical-characteristics
#14
Zhihua Chen, Yifeng Miao, Hui Wu, Ran Wang, Zhiyi Zhou, Shilei Zhang, Longtian Chen, Yongming Qiu
Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis occasionally affecting central nervous system (CNS), and GPA patients with initial CNS symptoms are even rarer, whose diagnosis is further confused by an absence of positive antineutrophil cytoplasmic antibody. The authors described the characteristics of antineutrophil cytoplasmic antibody -negative GPA with CNS onset in a patient and discussed on its management, which may contribute to future diagnosis and treatment of patients with similar conditions...
December 12, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29238021/-granulomatosis-with-polyangiitis-complicated-with-gastrointestinal-perforation-a-case-report-and-review-of-literature
#15
Takao Kiboshi, Kentaro Isoda, Koichi Furukawa, Tomoyuki Wakahara, Kenichiro Otani, Kayo Ueda, Junichi Konma, Kazuhiro Teramura, Nozomi Ueno, Hiroshi Fujiwara, Takeshi Shoda
  A 51-year-old man was detected nasal bleeding, multiple pulmonary nodule and mass, urinalysis abnormality, renal involvement and high titer of proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and was suspected of granulomatosis with polyangiitis and initiated with steroid pulse therapy. On the day after the start of steroid pulse therapy, generalized peritonitis due to ileal perforation occurred, and emergency ileectomy and peritonitis surgery were performed. Induction therapy with steroid pulse therapy, plasma exchange and intravenous cyclophosphamide therapy (IVCY) and maintenance therapy with glucocorticoid and azathioprine led to good therapeutic outcomes...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29221579/biomarkers-in-connective-tissue-diseases
#16
REVIEW
Neelakshi R Jog, Judith A James
Autoimmune connective tissue diseases are clinically variable, making biomarkers desirable for assessing future disease risk, supporting early and accurate diagnosis, monitoring disease activity and progression, selecting therapeutics, and assessing treatment response. Because of their correlations with specific clinical characteristics and often with disease progression, autoantibodies and other soluble mediators are considered potential biomarkers. Additional biomarkers might reflect downstream pathologic processes or appear because of ongoing inflammation and damage...
December 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29213125/the-role-of-semaphorins-in-immune-responses-and-autoimmune-rheumatic-diseases
#17
REVIEW
Masayuki Nishide, Atsushi Kumanogoh
Semaphorins have a well-characterized role in guiding axon repulsion during development; however, the important contribution of these proteins in immunity is becoming increasingly clear. Immunoregulatory semaphorins, termed 'immune semaphorins', have roles in regulating immune cell activation, differentiation, mobility and migration. These proteins are also intimately associated with the pathogenesis of autoimmune diseases including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)...
December 7, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/29204761/clinical-value-of-systemic-symptoms-in-iga-nephropathy-with-anca-positivity
#18
Lijiao Xie, Jianghua He, Xing Liu, Sha Tang, Weili Wang, Furong Li, Ying Zhang, Jun Zhang, Yunjian Huang, Jinghong Zhao, Yafei Li, Jingbo Zhang
Our aim was to evaluate the pathogenic role of anti-neutrophil cytoplasmic antibodies (ANCAs) in patients with IgA nephropathy (IgAN). A total of 2390 patients with biopsy-confirmed IgAN were analyzed retrospectively. Thirty-five IgAN patients with ANCA and 40 IgAN patients without ANCA were enrolled. According to the Birmingham Vasculitis Activity Score (BVAS) items, the ANCA-positive patients were further divided into two subgroups which with or without systemic symptoms. The cumulative renal survival rate was calculated using Kaplan-Meier analysis...
December 4, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29201630/avoidance-of-harm-from-treatment-for-anca-associated-vasculitis
#19
REVIEW
Catherine King, Lorraine Harper
Purpose of review With established immunosuppressant treatment regimens for anti-neutrophil cytoplasm antibody-associated vasculitides (AAV), prognosis has significantly improved. The mainstay of treatment still comprises high-dose corticosteroids and cyclophosphamide for severe forms, although rituximab is being increasingly utilised instead of cyclophosphamide as induction therapy. AAV patients experience an excess of infections, malignancies and cardiovascular events as compared to the general population, which is a combination of the systemic inflammatory process associated with vasculitis and the adverse events from treatment...
2017: Current Treatment Options in Rheumatology
https://www.readbyqxmd.com/read/29167552/association-of-ets1-polymorphism-with-granulomatosis-with-polyangiitis-and-proteinase-3-anti-neutrophil-cytoplasmic-antibody-positive-vasculitis-in-a-japanese-population
#20
Aya Kawasaki, Keita Yamashita, Fumio Hirano, Ken-Ei Sada, Daisuke Tsukui, Yuya Kondo, Yoshitaka Kimura, Kurumi Asako, Shigeto Kobayashi, Hidehiro Yamada, Hiroshi Furukawa, Kenji Nagasaka, Takahiko Sugihara, Kunihiro Yamagata, Takayuki Sumida, Shigeto Tohma, Hajime Kono, Shoichi Ozaki, Seiichi Matsuo, Hiroshi Hashimoto, Hirofumi Makino, Yoshihiro Arimura, Masayoshi Harigai, Naoyuki Tsuchiya
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)...
October 5, 2017: Journal of Human Genetics
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