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Crescentic glomerulonephritis

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https://www.readbyqxmd.com/read/28514287/concurrent-igg4-related-tubulointerstitial-nephritis-and-igg4-myeloperoxidase-anti-neutrophil-cytoplasmic-antibody-positive-crescentic-glomerulonephritis-a-case-report
#1
Tao Su, Li Yang, Zhao Cui, Su-Xia Wang, Ming-Hui Zhao
RATIONALE: IgG4-related disease (IgG4-RD) is a newly recognized systemic disease. The typical pathological finding in the kidney is abundant IgG4-positive plasma cell infiltration with characteristic storiform fibrosis in the interstitium. Antibodies of the IgG4 subclass have been linked to certain autoimmune diseases including antiproteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) of the IgG4 subclass. Here, we report a rare case of kidney injury with concurrent typical IgG4-related tubulointerstitial nephritis and IgG4 subclass of myeloperoxidase (MPO) ANCA-positive necrotizing crescentic glomerulonephritis...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28509135/renal-limited-vasculitis-with-elevated-levels-of-multiple-antibodies
#2
Noriaki Sato, Hideki Yokoi, Hirotaka Imamaki, Eiichiro Uchino, Kaoru Sakai, Takeshi Matsubara, Tatsuo Tsukamoto, Sachiko Minamiguchi, Motoko Yanagita
Renal-limited vasculitis (RLV) is a type of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis that presents with crescentic glomerulonephritis with no other organ involvement. Although several studies reported patients with crescentic glomerulonephritis who were dual positive for proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA or ANCA and anti-glomerular basement membrane (GBM) antibody, patients positive for all three antibodies, i.e., triple-positive patients, were rarely reported. We herein report the case of a male with pauci-immune type crescentic glomerulonephritis positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibody...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28509125/a-case-of-pr3-anca-positive-anti-gbm-disease-associated-with-intrarenal-arteritis-and-thrombotic-microangiopathy
#3
Shun Manabe, Mayuko Banno, Marie Nakano, Teruhiro Fujii, Yukio Kakuta, Kosaku Nitta, Michiyasu Hatano
Coexistence of anti-glomerular basement membrane (anti-GBM) disease with anti-neutrophil cytoplasmic antibody (ANCA) is occasionally reported and termed "double positive" disease. Interestingly, the majority of "double positive" ANCA is myeloperoxidase (MPO)-ANCA, and some of the MPO-ANCA-positive cases reveal intrarenal arteritis indicating an ANCA-associated renal lesion. In contrast, proteinase 3 (PR3)-ANCA-positive "double positive" disease had rarely been reported, and as far as we know, none of the cases showed intrarenal arteritis...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28509121/antithyroid-drug-associated-mpo-anca-positive-tubulointerstitial-nephritis-in-a-type-2-diabetes-patient-a-case-report
#4
Shinsuke Nishimura, Kazushi Nakao, Masaya Takeda, Ikuko Matsuura, Yoshihisa Nomura, Sonei Shojima, Yuriko Yamamura, Kazuyuki Fujita, Noriya Momoki, Keisuke Maruyama, Masahiro Yamamura, Makoto Hiramatsu
A 54-year-old man diagnosed with type 2 diabetes and hyperthyroidism was prescribed propylthiouracil (PTU) after the patient developed hepatic dysfunction on thiamazole. At 50 mg/day of PTU, he was stable with thyroid-stimulating hormone receptor and thyrotropic antibody titers remaining stable. After four years of taking PTU, he was referred to the Department of Nephrology due to a rapid increase in his serum creatinine (Cr) level. He showed impaired renal function (Cr 2.26 mg/dL; estimated glomerular filtration rate (eGFR), 25 mL/min)...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508974/pauci-immune-crescentic-glomerulonephritis-associated-with-use-of-trimethoprim-sulfamethoxazole
#5
Shruti S Hegde, Vanesa Bijol, Bertrand L Jaber
Drug-induced pauci-immune crescentic glomerulonephritis has been described with several agents, including propylthiouracil, minocycline, D-penicillamine, and hydralazine. We present the case of a 60-year-old man who presented with rapidly progressive glomerulonephritis in the setting of recent use of trimethoprim-sulfamethoxazole complicated by the development of the Stevens-Johnson syndrome, and was found to have biopsy-proven pauci-immune crescentic glomerulonephritis and undetectable anti-neutrophilic cytoplasmic antibodies...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28491860/differences-in-the-frequency-of-macrophage-and-t-cell-markers-between-focal-and-crescentic-classes-of-anti-neutrophil-cytoplasmic-antibody-anca-associated-glomerulonephritis
#6
Dana Kidder, Susan E Bray, Stewart Fleming
BACKGROUND: Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (AAGN) can be classified into; focal, crescentic, mixed and sclerotic classes. Macrophages and T lymphocytes are key players in mediating renal injury. The frequency of macrophage and T lymphocytes in different histological classes is unclear. OBJECTIVES: We examined the frequency of macrophage and T lymphocyte markers in AAGN and assessed their correlation with renal function at presentation...
March 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28491331/mpo-c-anca-associated-necrotising-and-crescentic-glomerulonephritis
#7
Abhilash Koratala, Dara N Wakefield, Kawther F Alquadan, A Ahsan Ejaz
The patterns of ANCA staining usually relate closely to antibodies against myeloperoxidase and proteinase-3. C-ANCA is mainly antibodies to proteinase-3 and P-ANCA is antibodies to myeloperoxidase. C-ANCA with antibodies to MPO with clinical sequelae is unusual.
April 2017: JRSM Open
https://www.readbyqxmd.com/read/28469165/the-regulatory-t-cell-transcription-factor-foxp3-protects-against-crescentic-glomerulonephritis
#8
Chen Yang, Xiao-Ru Huang, Erik Fung, Hua-Feng Liu, Hui-Yao Lan
Regulatory T cells (Tregs) have been shown to play a protective role in glomerulonephritis (GN) and Foxp3 is a master transcription factor in Treg development. In this study, we examined the functional role and mechanisms of Foxp3 in a mouse model of accelerated anti-glomerular basement membrane (anti-GBM) GN induced in antigen-primed Foxp3 transgenic (Tg) mice. Compared with littermate of wildtype (WT) mice in which induced severe crescentic GN developed with progressive renal dysfunction, Foxp3 Tg mice had reduced crescent formation, urinary protein excretion, plasma creatinine and decline in creatinine clearance...
May 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28459999/diagnostic-and-management-challenges-in-goodpasture-s-anti-glomerular-basement-membrane-disease
#9
Scott R Henderson, Alan D Salama
Goodpasture's or anti-glomerular basement membrane (GBM) disease is classically characterized by the presence of circulating autoantibodies directed against the non-collagenous domain of the α3 chain of type IV collagen, targeting glomerular and alveolar basement membranes, and associated with rapidly progressive crescentic glomerulonephritis, with alveolar haemorrhage in over half the patients. However, there are increasing examples of variants or atypical presentations of this disease, and novel therapeutic options have been proposed, which nephrologists should be aware of...
April 28, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28450461/identification-of-ceruloplasmin-as-a-gene-that-affects-susceptibility-to-glomerulonephritis-through-macrophage-function
#10
Tai-Di Chen, Maxime Rotival, Ling-Yin Chiu, Marta Bagnati, Jeong-Hun Ko, Prashant K Srivastava, Enrico Petretto, Charles D Pusey, Ping-Chin Lai, Timothy J Aitman, H Terence Cook, Jacques Behmoaras
Crescentic glomerulonephritis (Crgn) is a complex disorder where macrophage activity and infiltration are significant effector causes. In previous linkage studies using the uniquely susceptible Wistar Kyoto (WKY) rat strain, we have identified multiple crescentic glomerulonephritis QTLs (Crgn) and positionally cloned genes underlying Crgn1 and Crgn2, which accounted for 40% of total variance in glomerular inflammation. Here, we have generated a back-cross (BC) population (n=166) where Crgn1 and Crgn2 were genetically fixed, and found significant linkage to glomerular crescents on chromosome 2 (Crgn8, LOD=3...
April 26, 2017: Genetics
https://www.readbyqxmd.com/read/28432167/a-tale-of-two-vasculitides-biopsy-proven-giant-cell-arteritis-followed-by-the-independent-development-of-renal-limited-microscopic-polyangiitis
#11
Roman Zuckerman, Mayur Patel, Deborah R Alpert
We describe an 80-year-old woman who presented with headaches, bilateral jaw claudication and visual symptoms. She was diagnosed with giant cell arteritis, which was confirmed by temporal artery biopsy. She was treated with high-dose prednisone followed by a slow taper, with complete resolution of symptoms. Approximately 4 years later, she developed progressively worsening renal function associated with haematuria. Serological workup revealed a high-titre perinuclear antinuclear cytoplasmic antibody and antibodies to myeloperoxidase...
April 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28405538/henoch-sch%C3%A3-nlein-purpura-presenting-as-severe-gastrointestinal-and-renal-involvement-with-mixed-outcomes-in-an-adult-patient
#12
Raj Shah, Madhuri Ramakrishnan, Alexis Vollmar, Amanda Harrell, Richard Van Trump, Amgad Masoud
Henoch-Schönlein purpura (HSP) is typically seen as a self-limiting disease in children, but can present more severely in adults, especially when there is renal involvement. Management of HSP in adults also remains a controversial topic with very few studies evaluating available therapies. In this case, HSP presenting as a combination of severe gastrointestinal involvement and a rapid decline in renal function in an adult patient directed our therapy. The patient was a 48-year-old Caucasian male with no known past medical history, who presented with a combination of purpuric rash over the lower extremities, severe abdominal pain with upper gastrointestinal bleeding and a rapidly increasing serum creatinine, with hematuria...
March 9, 2017: Curēus
https://www.readbyqxmd.com/read/28403058/multiorgan-involvement-confounding-the-diagnosis-of-bartonella-henselae-infective-endocarditis-in-children-with-congenital-heart-disease
#13
Christopher P Ouellette, Sarita Joshi, Karen Texter, Preeti Jaggi
Two children with congenital heart disease status post surgical correction presented with prolonged constitutional symptoms, hepatosplenomegaly and pancytopenia. Concern for malignancy prompted bone marrow biopsies that were without evidence thereof. In case 1, echocardiography identified a multilobulated vegetation on the conduit valve. In case 2, transthoracic, transesophageal and intracardiac echocardiography were performed and were without evidence of cardiac vegetations; however, pulmonic emboli raised concern for infective endocarditis...
May 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28391334/complement-activation-in-pauci-immune-necrotizing-and-crescentic-glomerulonephritis-results-of-a-proteomic-analysis
#14
Sanjeev Sethi, Ladan Zand, An S De Vriese, Ulrich Specks, Julie A Vrana, Siddak Kanwar, Paul Kurtin, Jason D Theis, Andrea Angioi, Lynn Cornell, Fernando C Fervenza
Background: Complement activation plays an important role in the pathophysiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), although it remains unclear which pathway is activated. Whether pauci-immune necrotizing crescentic glomerulonephritis (pauci-immune GN) with negative ANCA serology is part of the spectrum of AAV or a different disease entity is essentially unknown. Methods: We used proteomic analysis to delineate the complement profile in a series of 13 kidney biopsies of patients with pauci-immune GN, with either proteinase 3 (PR3) (five patients) or myeloperoxidase (MPO) antibodies (four patients) or with consistently negative ANCA serology (four patients)...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28381748/post-infectious-proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-g-deposits-associated-with-complement-factor-h-mutation
#15
Eriko Takehara, Shintaro Mandai, Satomi Shikuma, Wataru Akita, Motoko Chiga, Takayasu Mori, Takashi Oda, Michio Kuwahara, Shinichi Uchida
A 55-year-old man developed rapidly progressive glomerulonephritis and nephrotic syndrome. A kidney biopsy specimen showed diffuse proliferative and crescentic glomerulonephritis with monoclonal IgG1κ, humps, and nephritis-associated plasmin receptor, indicating infection-associated proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID). Despite dialysis-dependent renal failure, symptomatic therapy resulted in spontaneous recovery of the renal function, mimicking post-infectious glomerulonephritis (PIGN)...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28356674/fibrillary-glomerulonephritis-presenting-as-crescentic-glomerulonephritis
#16
H H Shah, J Thakkar, J M Pullman, A T Mathew
Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that commonly presents clinically with hypertension, proteinuria, microscopic hematuria, and varying degree of renal insufficiency. Histologically, FGN can present with different patterns of glomerular injury, more commonly mesangioproliferative, membranoproliferative, and membranous nephropathy. While crescent formation has been described in some kidney biopsy series of FGN, crescentic glomerulonephritis pattern of glomerular injury has been rarely described...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28356661/anti-glomerular-basement-membrane-disease-case-series-from-a-tertiary-center-in-north-india
#17
D Prabhakar, M Rathi, R Nada, R W Minz, V Kumar, H S Kohli, V Jha, K L Gupta
Anti-glomerular basement (anti-GBM) disease is an uncommon disorder with a bimodal age of presentation. Patients presenting with dialysis-dependent renal failure have poor renal outcomes. There is limited data regarding the clinical presentation and outcomes of anti-GBM disease from India. We conducted this prospective study to analyze the clinical presentation and outcomes of anti-GBM disease at a large tertiary care hospital in North India over 1½ years. Subjects with a biopsy proven anti-GBM disease (light microscopic examination showing crescents and immunofluorescence examination showing linear deposition of IgG) with or without positive anti-GBM antibodies in serum were included in the study and followed-up for at least 12 months...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28356384/il-26-confers-proinflammatory-properties-to-extracellular-dna
#18
Caroline Poli, Jean François Augusto, Jonathan Dauvé, Clément Adam, Laurence Preisser, Vincent Larochette, Pascale Pignon, Ariel Savina, Simon Blanchard, Jean François Subra, Alain Chevailler, Vincent Procaccio, Anne Croué, Christophe Créminon, Alain Morel, Yves Delneste, Helmut Fickenscher, Pascale Jeannin
In physiological conditions, self-DNA released by dying cells is not detected by intracellular DNA sensors. In chronic inflammatory disorders, unabated inflammation has been associated with a break in innate immune tolerance to self-DNA. However, extracellular DNA has to complex with DNA-binding molecules to gain access to intracellular DNA sensors. IL-26 is a member of the IL-10 cytokine family, overexpressed in numerous chronic inflammatory diseases, in which biological activity remains unclear. We demonstrate in this study that IL-26 binds to genomic DNA, mitochondrial DNA, and neutrophil extracellular traps, and shuttles them in the cytosol of human myeloid cells...
May 1, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28356090/anca-vasculitis-in-a-patient-with-alport-syndrome-a-difficult-diagnosis-but-a-treatable-disease
#19
Valentine Gillion, Michel Jadoul, Selda Aydin, Nathalie Godefroid
BACKGROUND: Alport syndrome and ANCA-associated vasculitis are both rare diseases. The co-existence of these two conditions has never been reported. There is no obvious pathogenic link between these two glomerular diseases. The management of this case highlights the importance of a systematic approach when investigating the unexpected unfavourable evolution of a known glomerulopathy. CASE PRESENTATION: A-17 year old caucasian boy with a genetically proven X-linked Alport syndrome presented with progressive dyspnea, fatigue and pallor...
March 29, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28352022/analysis-of-renal-diseases-detected-in-renal-biopsies-of-adult-patients-a-single-center-experience
#20
Salman Imtiaz, Murtaza F Drohlia, Kiran Nasir, Beena Salman, Aasim Ahmad
Renal biopsy is crucial while evaluating for the diagnosis of glomerular, vascular, tubulointerstitial, and genetic diseases. It gives vital information which helps in estimating the disease prognosis, progression, and management. This is the retrospective analysis of all adult patients aged above 18 years, who underwent percutaneous renal biopsy at The Kidney Center Post Graduate Training Institute, Karachi, over a duration of 18 years, i.e., January 1, 1996, to December 2013. Renal graft biopsies and those which were inadequate were excluded from analysis...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
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