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pauci-immune crescentic glomerulonephritis

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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
#1
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/28391334/complement-activation-in-pauci-immune-necrotizing-and-crescentic-glomerulonephritis-results-of-a-proteomic-analysis
#2
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/28339709/clinicopathologic-correlations-of-renal-pathology-in-the-adult-population-of-poland
#3
Agnieszka Perkowska-Ptasinska, Artur Bartczak, Malgorzata Wagrowska-Danilewicz, Agnieszka Halon, Krzysztof Okon, Aldona Wozniak, Marian Danilewicz, Henryk Karkoszka, Andrzej Marszalek, Jolanta Kowalewska, Andrzej Mroz, Agnieszka Korolczuk, Andrzej Oko, Alicja Debska-Slizien, Beata Naumnik, Zbigniew Hruby, Marian Klinger, Kazimierz Ciechanowski, Marek Myslak, Wladyslaw Sulowicz, Andrzej Rydzewski, Andrzej Wiecek, Jacek Manitius, Tadeusz Gregorczyk, Stanislaw Niemczyk, Michal Nowicki, Ryszard Gellert, Tomasz Stompor, Monika Wieliczko, Krzysztof Marczewski, Leszek Paczek, Olga Rostkowska, Dominika Deborska-Materkowska, Grazyna Bogdanowicz, Andrzej Milkowski, Magdalena Durlik
Background: This is the first report on the epidemiology of biopsy-proven kidney diseases in Poland. Methods: The Polish Registry of Renal Biopsies has collected information on all (n = 9394) native renal biopsies performed in Poland from 2009 to 2014. Patients' clinical data collected at the time of biopsy, and histopathological diagnoses were used for epidemiological and clinicopathologic analysis. Results: There was a gradual increase in the number of native renal biopsies performed per million people (PMP) per year in Poland in 2009-14, starting from 36 PMP in 2009 to 44 PMP in 2014...
April 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28316335/complement-in-anca-associated-vasculitis-mechanisms-and-implications-for-management
#4
REVIEW
Min Chen, David R W Jayne, Ming-Hui Zhao
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of potentially life-threatening autoimmune diseases. The main histological feature in the kidneys of patients with AAV is pauci-immune necrotizing crescentic glomerulonephritis with little immunoglobulin and complement deposition in the glomerular capillary walls. The complement system was not, therefore, initially thought to be associated with the development of AAV. Accumulating evidence from animal models and clinical observations indicate, however, that activation of the complement system - and the alternative pathway in particular - is crucial for the development of AAV, and that the complement activation product C5a has a central role...
March 20, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28133935/post-transplant-immune-complex-nephritis-in-a-patient-with-systemic-lupus-erythematosus-associated-with-anca-vasculitis
#5
Carlos Sanchez, Alejandra Rebolledo, Junior Gahona, Mauricio Rojas, Raquel Jiménez, Aurora Bojórquez
Nearly 20% of SLE corresponds to the pediatric population, and 75% of them have kidney involvement representing an important etiology of chronic kidney disease. A correlation between SLE and ANCA-associated vasculitis has been identified as an overlapping syndrome. Kidney allograft recurrence is rare in SLE when disease control is achieved and with nowadays immunosuppression treatment. Histologic transformation is unusual, especially when there are negative serologic markers and no immune complex deposition reported in native kidneys...
May 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/27939215/the-functional-activities-of-complement-factor-h-are-impaired-in-patients-with-anca-positive-vasculitis
#6
Su-Fang Chen, Feng-Mei Wang, Zhi-Ying Li, Feng Yu, Min Chen, Ming-Hui Zhao
Increasing evidences have demonstrated that the activation of the alternative complement pathway is crucial for the pathogenesis of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). Our recent study found that circulating levels of complement factor H (FH), a key regulator of the alternative pathway, were associated with disease activity. In the current study, functional activities of FH were assessed to further explore the potential role of FH in the pathogenesis of AAV. We found that the two patients with ANCA-negative pauci-immune necrotizing crescentic glomerulonephritis exhibited relatively normal functional activities of FH...
December 6, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27891268/a-case-report-describing-a-rare-presentation-of-simultaneous-occurrence-of-mpo-anca-associated-vasculitis-and-rheumatoid-arthritis
#7
Nathalie Foray, Tamer Hudali, Muralidhar Papireddy, John Gao
Background. Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation. A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical history consisted of newly diagnosed but untreated rheumatoid arthritis, hypertension, and non-insulin-dependent diabetes mellitus...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27884116/kidney-disease-in-the-elderly-biopsy-based-data-from-14-renal-centers-in-poland
#8
Agnieszka Perkowska-Ptasinska, Dominika Deborska-Materkowska, Artur Bartczak, Tomasz Stompor, Tomasz Liberek, Barbara Bullo-Piontecka, Anna Wasinska, Agnieszka Serwacka, Marian Klinger, Jolanta Chyl, Michal Kuriga, Robert Malecki, Krzysztof Marczewski, Bogdan Hryniewicz, Tadeusz Gregorczyk, Monika Wieliczko, Stanislaw Niemczyk, Olga Rostkowska, Leszek Paczek, Magdalena Durlik
BACKGROUND: Longer life expectancy is associated with an increasing prevalence of kidney disease. Aging itself may cause renal damage, but the spectrum of kidney disorders that affect elderly patients is diverse. Few studies, mostly form US, Asia and West Europe found differences in the prevalence of some types of kidney diseases between elderly and younger patients based on renal biopsy findings, with varied proportion between glomerulopathies and arterionephrosclerosis as a dominant injury found...
November 25, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27872837/antineutrophil-cytoplasmic-antibodies-crescentic-allograft-glomerulonephritis-after-sofosbuvir-therapy
#9
Shilpa Gadde, Belinda Lee, Laura Kidd, Rubin Zhang
Antineutrophil cytoplasmic antibodies (ANCA) are well known to be associated with several types of vasculitis, including pauci-immune crescentic glomerulonephritis, a form of rapid progressive glomerular nephritis (RPGN). ANCA vasculitis has also been reported after administration of propylthiouracil, hydralazine, cocaine (adulterated with levimasole), allopurinol, penicillamine and few other drugs. All previously reported cases of drug-associated ANCA glomerulonephritis were in native kidneys. Sofosbuvir is a new and effective drug for hepatitis C virus infection...
November 6, 2016: World Journal of Nephrology
https://www.readbyqxmd.com/read/27801920/hydralazine-induced-anca-vasculitis-in-the-setting-of-acute-clostridium-difficile-infection
#10
Somwail Rasla, Amr El Meligy, Dragos F Cucu
We report a rare case of Hydralazine-induced ANCA associated glomerulonephritis with alveolar hemorrhage in the setting of acute Clostridium Difficile Infection. A 71-year-old Caucasian woman with hypertension, who was being treated with hydralazine 25 mg twice a day for six years, presented to the hospital with diarrhea, nausea, vomiting and anemia. She had acute kidney injury and urinalysis showed proteinuria, dysmorphic RBCs, and rare RBC cast. She was found to have Clostridium difficile colitis which was successfully treated...
November 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27772637/ajkd-atlas-of-renal-pathology-pauci-immune-necrotizing-crescentic-glomerulonephritis
#11
Agnes B Fogo, Mark A Lusco, Behzad Najafian, Charles E Alpers
No abstract text is available yet for this article.
November 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27536682/the-prevalence-and-management-of-pauci-immune-glomerulonephritis-and-vasculitis-in-western-countries
#12
REVIEW
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
https://www.readbyqxmd.com/read/27512296/clinical-spectrum-and-outcomes-of-crescentic-glomerulonephritis-a-single-center-experience
#13
S K Rampelli, N G Rajesh, B H Srinivas, K T Harichandra Kumar, R P Swaminathan, P S Priyamvada
There is limited data on the etiology, clinical and histopathological spectrum and outcomes of crescentic glomerulonephritis (CrGN) in adult Indian population. This prospective study was done to evaluate the etiology, clinicohistological patterns and predictors of outcome of CrGN in South Indian population. All the patients received standard protocol based immunosuppression in addition to supportive care. Immune-complex glomerulonephritis (ICGN) was the most common etiology (n = 31; 77.5%) followed by pauci-immune glomerulonephritis (PauciGN; n = 8; 20%) and anti-glomerular basement membrane disease (n = 1; 2...
July 2016: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/27506330/pauci-immune-glomerulonephritis-in-a-captive-chimpanzee-pan-troglodytes-and-a-review-of-spontaneous-cases-in-animals
#14
REVIEW
Lauren E Neidig, Michael A Owston, Erin Ball, Edward J Dick
BACKGROUND: Crescentic glomeruli are the hallmark finding in rapidly progressive glomerulonephritis (RPGN) and are characterized by disruption and proliferation of the glomerular capsule and an influx of cells into Bowman's space. Pauci-immune-type RPGN is identified by a lack of immunoglobulins and immune complexes in the glomerular basement membrane. METHODS: Complete necropsy and histology were performed on the affected chimpanzee. Electron microscopy was performed on kidney sections...
August 10, 2016: Journal of Medical Primatology
https://www.readbyqxmd.com/read/27504208/pauci-immune-crescentic-glomerulonephritis-in-connective-tissue-disease
#15
Supraja Yeturi, Mary Cronin, Adam Robin, Campbell Lorna, Ann K Rosenthal
Pauci-immune crescentic glomerulonephritis is commonly seen in ANCA-associated vasculitis but it is rarely seen during the course of other connective tissue diseases like lupus or Sjogren's syndrome or MCTD. We report 3 cases of pauci-immune crescentic glomerulonephritis in patients with connective tissue disease other than vasculitis. We reviewed literature and made summary of previously reported cases of this rare entity. Clinical and laboratory features of these patients varied widely, but most of patients have met criteria for lupus...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27461086/long-term-outcomes-in-antineutrophil-cytoplasmic-autoantibody-positive-eosinophilic-granulomatosis-with-polyangiitis-patients-with-renal-involvement-a-retrospective-study-of-14-chinese-patients
#16
Yinghua Chen, Yuemei Ding, Zhengzhao Liu, Haitao Zhang, Zhihong Liu, Weixin Hu
BACKGROUND: The clinic-pathological features and outcomes of Chinese patients with antineutrophil cytoplasmic autoantibody (ANCA)-positive eosinophilic granulomatosis with polyangiitis (EGPA) and renal involvement have not been studied. METHODS: Fourteen EGPA patients with renal involvement were included. All patients underwent renal biopsy. Clinic-pathological features and outcomes were retrospectively analyzed. RESULTS: The most common initial symptom of EGPA was asthma (57...
July 26, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27424701/hilar-and-para-aortic-necrotizing-lymphadenopathy-associated-with-antineutrophil-cytoplasmic-antibody-negative-pauci-immune-crescentic-glomerulonephritis
#17
Kamel El-Reshaid, Jozsef Varro, John P Madda
Lymph node involvement as a part of systemic vasculitis is rare. We report a case of women with rapidly progressive renal disease associated with recurrent epigastric pain, weight loss, and massive hilar as well as para-aortic lymphadenopathy. Ultrasound-guided biopsy of her scarred kidneys revealed antineutrophil cytoplasmic antibody-negative crescentic glomerulonephritis and that of lymph nodes showed severe necrotizing vasculitis. Biopsy of the lymph nodes and the failing kidney established the diagnosis of this rare presentation and ruled out lymphoma and tuberculosis...
July 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27384449/dual-anti-neutrophil-cytoplasmic-antibody-related-pauci-immune-crescentic-glomerulonephritis-in-a-patient-with-sj%C3%A3-gren-s-syndrome
#18
REVIEW
In Hee Lee, Seong-Kyu Kim, Min-Kyung Kim
Sjögren's syndrome is an autoimmune disease that primarily affects exocrine glands. Renal involvement of Sjögren's syndrome may lead to tubulointerstitial disease, whereas secondary glomerulopathies such as anti-neutrophil cytoplasmic antibody (ANCA)-related pauci-immune crescentic glomerulonephritis are rarely observed. In addition, crescent glomerulonephritis that is simultaneously positive for both myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA has never been reported in Sjögren's syndrome. Here, we report a case of pauci-immune crescentic glomerulonephritis exhibiting positivity for both MPO- and PR3-ANCAs in a patient with primary Sjögren's syndrome...
September 2016: Rheumatology International
https://www.readbyqxmd.com/read/27293925/granulomatosis-with-polyangiitis-presenting-as-pauci-immune-crescentic-glomerulonephritis-in-pregnancy
#19
Ryan Kunjal, Raafat Makary, Andreea Poenariu
Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27257040/glomerular-c3d-as-a-novel-prognostic-marker-for-renal-vasculitis
#20
Javier Villacorta, Francisco Diaz-Crespo, Mercedes Acevedo, Carmen Guerrero, Yolanda Campos-Martin, Eugenio García-Díaz, Manuela Mollejo, Gema Fernandez-Juarez
Pauci-immune necrotizing crescentic glomerulonephritis is the histologic substrate of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Several studies in animal models have demonstrated the crucial role of complement activation in the pathogenesis of ANCA-associated vasculitis, but only small series have analyzed the prognostic implications of complement glomerular deposits. This study aimed to assess the clinical and prognostic implications of C3d- and C4d-positive glomerular staining in renal vasculitis...
October 2016: Human Pathology
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