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renal vasculitis anca

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
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
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
H Marco, J Draibe, J Villacorta, L F Quintana, N Martin, R Garcia-Osuna, C Cabre, M A Martín-Gómez, A Balius, A Saurina, M Picazo, I Gich-Saladich, M Navarro-Díaz, M Praga, T Cavero, J Ballarin, M M Díaz-Encarnación
The classification of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) remains controversial. The main objective of this study was to define the respective values of ANCA serotype-based classification, clinicopathological classification, and histopathological classification in predicting patient and renal outcomes in a Spanish cohort of patients with ANCA with specificity for myeloperoxidase, MPO-ANCA, versus ANCA with specificity for proteinase 3, PR3-ANCA. Two hundred and forty-five patients with ANCA-AAV and biopsy-proven renal involvement diagnosed between 2000 and 2104 were recruited in 12 nephrology services...
March 9, 2018: Clinical Rheumatology
Z W Liu, W Qiu, J M Peng, Z W Wang, J L Zhao, Q J Wu
A 56-year-old female was admitted to the Department of Rheumatology, Peking Union Medical College Hospital with complaint of recurrent fever and acute lumbar pain. Fever was complicated with malaise, cough and occasional blood-streaked sputum. Lab tests showed elevated white blood cell count, increased serum creatinine, erythrocyte sedimentation rate and C-reactive protein. Other lab findings included severe anemia, hematuria, and proteinuria. Immunological examinations were positive for antinuclear antibodies, antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibody...
March 1, 2018: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Gerjan J Dekkema, Wayel H Abdulahad, Theo Bijma, Sarah M Moran, Louise Ryan, Mark A Little, Coen A Stegeman, Peter Heeringa, Jan-Stephan F Sanders
Background: Early detection of renal involvement in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is of major clinical importance to allow prompt initiation of treatment and limit renal damage. Urinary soluble cluster of differentiation 163 (usCD163) has recently been identified as a potential biomarker for active renal vasculitis. However, a significant number of patients with active renal vasculitis test negative using usCD163. We therefore studied whether soluble CD25 (sCD25), a T cell activation marker, could improve the detection of renal flares in AAV...
March 1, 2018: Nephrology, Dialysis, Transplantation
Suheyla Apaydin
Therapeutic plasma aphresis (plasmapheresis) is one form of treatment that is frequently used in practice of Nephrology. Plasmapheresis is the most important part of the therapies for Goodpasture's syndrome and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis which are causes of rapidly progressive glomerulonephritis. The reason why the effectiveness of plasmapheresis therapy cannot be clearly demonstrated in renal involvement in these diseases is that it does not appear to be possible to recruit an adequate number of patients and plasmapheresis is not effective in advanced disease if early treatment is not initiated...
February 20, 2018: Transfusion and Apheresis Science
Vineeta Shobha, Saba Fathima, Ravi Prakash
Granulomatosis with polyangiitis (GPA) previously known as Wegener's granulomatosis is one of the forms of idiopathic systemic vasculitis. There is very scanty data available on GPA in Asian and Indian population. We studied data of 60 patients from southern India, diagnosed with GPA to describe the physical characteristics, the treatment, and outcome. Patients who fulfilled any two of the four criteria proposed by the American College of Rheumatology, and those with clinical features of GPA with ANCA positivity and histopathological confirmation, were included in the study...
February 28, 2018: Clinical and Experimental Medicine
Zdenka Hruskova, Vladimir Tesar
BACKGROUND: Rapidly progressive glomerulonephritis (RPGN) is characterized by a rapid deterioration of renal function and by extracapillary proliferation in >50% of glomeruli. The most common type of RPGN is "pauci-immune" glomerulonephritis caused by anti-neutrophil cytoplasmic antibodies-associated vasculitis (AAV). SUMMARY: The incidence of AAV increases with age and pauci-immune glomerulonephritis is the most common diagnosis found in renal biopsies in the elderly population...
January 26, 2018: Blood Purification
Guangjian Liu, Jinhai Chen
This study investigated the risk factors of peritonitis during early peritoneal dialysis for renal failure in patients with antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis (AASV). This study enrolled hospitalized AASV patients with renal failure who underwent peritoneal dialysis at the Department of Nephrology of our hospital between 2008 and 2015. The primary aim of this study was to perform a retrospective analysis to characterize peritonitis during the early period (≤ 8 weeks) of dialysis, and to compare the differences in initial clinical manifestations, vasculitis activity, immunosuppressive therapies prior to the renal replacement therapy, and prognosis between patients with or without peritonitis...
February 21, 2018: Clinical Nephrology
Stephen P McAdoo, Nicholas Medjeral-Thomas, Seerapani Gopaluni, Anisha Tanna, Nicholas Mansfield, Jack Galliford, Megan Griffith, Jeremy Levy, Thomas D Cairns, David Jayne, Alan D Salama, Charles D Pusey
Background: Current guidelines advise that rituximab or cyclophosphamide should be used for the treatment of organ-threatening disease in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV), although few studies have examined the efficacy and safety of these agents in combination. Methods: We conducted a single-centre cohort study of 66 patients treated with a combination of oral corticosteroids, rituximab and low-dose pulsed intravenous cyclophosphamide followed by a maintenance regimen of azathioprine and tapered steroid for the treatment of biopsy-proven renal involvement in AAV...
February 14, 2018: Nephrology, Dialysis, Transplantation
Mara Felicetti, Diego Cazzador, Roberto Padoan, Alfonso Luca Pendolino, Chiara Faccioli, Ennio Nardello, Alvise Berti, Marina Silvestrini, Giuseppe Paolazzi, Giuliano Brunori, Elisabetta Zanoletti, Enzo Emanuelli, Alessandro Martini, Franco Schiavon
Ear, nose and throat (ENT) manifestations in granulomatosis with polyangiitis (GPA) represent the most frequent symptoms at disease onset. The aim of the study was to analyse ENT involvement at diagnosis, as well as how it could influence relapse rate, mortality and disease severity. A retrospective non-controlled cohort study was performed including all consecutive diagnosed GPA from 1996 to 2016 in two rheumatology centres of Northern Italy, focusing particularly on ENT presenting signs and symptoms at baseline...
February 20, 2018: Clinical Rheumatology
Silke R Brix, Mercedes Noriega, Elisabeth M Herden, Birgit Goldmann, Ulrike Langbehn, Martin Busch, Wolfram J Jabs, Oliver M Steinmetz, Ulf Panzer, Tobias B Huber, Rolf A K Stahl, Thorsten Wiech
AIMS: Renal involvement in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis contributes to significant morbidity and mortality in patients. In chronic inflammation, B cells are recruited to the inflamed tissue and organised lymphoid structures have been described in several autoimmune diseases. Aim of this study was to correlate the lymphoid organisation in renal biopsies with renal outcome in ANCA-associated glomerulonephritis (GN). METHODS AND RESULTS: We investigated 112 renal biopsies from patients with newly diagnosed ANCA-associated necrotizing GN...
February 17, 2018: Histopathology
Duvuru Geetha, Sam Kant
Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) has the propensity to acquire a devastating disease course. Despite the advances in therapeutics, a significant proportion of patients progress to end-stage renal disease (ESRD). Renal transplantation is being increasingly employed in this population, with gradual improvement in outcomes over the years, however, recurrence of disease requires constant surveillance and is associated with graft failure. Areas covered: A structured literature search in PubMed and Medline and abstracts of international conferences was performed to identify cases and cohorts of AAV patients who had undergone renal transplantation for ESRD...
February 13, 2018: Expert Review of Clinical Immunology
Akinori Hara, Takashi Wada, Ken-Ei Sada, Koichi Amano, Hiroaki Dobashi, Masayoshi Harigai, Yoshinari Takasaki, Hidehiro Yamada, Hitoshi Hasegawa, Taichi Hayashi, Shouichi Fujimoto, Eri Muso, Tamihiro Kawakami, Sakae Homma, Masaharu Yoshida, Junichi Hirahashi, Noriyoshi Ogawa, Satoshi Ito, Hirofumi Makino, Yoshihiro Arimura
OBJECTIVE: The aim was to elucidate the prognosis and risk factors associated with relapse during longterm remission maintenance therapy for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: Patients with newly diagnosed AAV (n = 156) were registered in the Remission Induction Therapy in Japanese patients with ANCA-associated Vasculitides (RemIT-JAV) study, and among them, 83 patients who achieved remission were enrolled and followed up for 24 additional months in our nationwide, prospective cohort study (Co-RemIT-JAV; registration number UMIN 000006373)...
February 1, 2018: Journal of Rheumatology
James Goya Heaf, Alastair Hansen, Gunnar Hellmund Laier
BACKGROUND: The association of increased cancer risk with glomerulonephritis (GN) is well known, but controversy exists concerning which types of GN are involved, and the size of the association. A national registry survey was performed to assess the size of this association, and the temporal relationship of cancer diagnosis to GN diagnosis. METHODS: All patients with biopsy-proven GN between 1985 and 2015 in Denmark were extracted from The Danish Renal Biopsy Registry and the National Pathology Data Bank...
February 2, 2018: BMC Nephrology
Xiao-Li Li, Peng-Cheng Xu, Tong Chen, Tie-Kun Yan, Jian-Qing Jiang, Jun-Ya Jia, Li Wei, Wen-Ya Shang, Shui-Yi Hu
RATIONALE: The relationship between antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) and ANCA-negative vasculitis has not been elucidated. PATIENT CONCERNS: A 64-year-old female with edema and proteinuria was admitted. A kidney biopsy indicated focal proliferative nephritis with crescents in 25% of glomeruli. Serum ANCA was negative. Eighteen months later, systemic symptoms emerged and acute kidney injury occurred. Serum ANCA against myeloperoxidase (MPO) turned positive...
December 2017: Medicine (Baltimore)
Rennie L Rhee, John C Davis, Linna Ding, Fernando C Fervenza, Gary S Hoffman, Cees G M Kallenberg, Carol A Langford, W Joseph McCune, Paul A Monach, Philip Seo, Robert Spiera, E William St Clair, Ulrich Specks, John H Stone, Peter A Merkel
BACKGROUND AND OBJECTIVES: The significance of persistent hematuria or proteinuria in patients with ANCA-associated vasculitis who are otherwise in clinical remission is unclear. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: A post hoc analysis was conducted using participants enrolled in two randomized, placebo-controlled clinical trials who had active GN due to ANCA-associated vasculitis, had positive ANCA, and achieved remission by month 6. Dipstick and microscopic urinalyses were performed at each visit...
January 25, 2018: Clinical Journal of the American Society of Nephrology: CJASN
Marion Haubitz
In patients with ANCA-associated vasculitis renal involvement is frequently seen and the severity of renal manifestation is very important for therapeutic strategies and prognosis. Clinically rapid loss of renal function, nephritic sediment and proteinuria in a non-nephrotic range are characterizing a focal segmental necrotizing pauci-immune glomerulonephritis with extrarenal proliferations. Induction treatment depends on the severity of manifestations. With a normal renal function methotrexate can be used in combination with steroids...
January 2018: Deutsche Medizinische Wochenschrift
Peng-Cheng Xu, Zhi-Ying Tong, Tong Chen, Shan Gao, Shui-Yi Hu, Xiao-Wei Yang, Tie-Kun Yan, Shan Lin
OBJECTIVES: Hypoalbuminaemia has been proved to be a biomarker of poor prognosis in many diseases. The objective of this study was to investigate the significance of hypoalbuminaemia in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: Data of 117 AAV patients were analysed retrospectively. The relationship between hypoalbuminaemia and disease severity were studied. The influence of albumin on the pathogenetic role of ANCA was investigated in vitro...
January 15, 2018: Clinical and Experimental Rheumatology
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