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pauci-immune crescentic glomerulonephritis

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https://www.readbyqxmd.com/read/29043147/a-case-report-of-paraproteinemia-associated-pauci-immune-glomerulonephritis-a-new-form-of-monoclonal-gammopathy-of-renal-significance
#1
Robert Rope, Neeraja Kambham, Neiha Arora
BACKGROUND: Renal disease associated with paraproteinemias is classically predicated upon pathologic paraprotein deposition in the kidney. However, growing evidence suggests that paraproteins may be able to systemically activate complement or neutrophils to drive renal damage. This may provide an alternative pathologic mechanism for renal injury in rare cases. CASE REPORT: We report a case of a patient with crescentic pauci-immune glomerulonephritis presenting with rapidly progressive renal failure, polyarthropathy, and a purpuric rash in association with a monoclonal immunoglobulin G κ-light-chain producing multiple myeloma...
2017: Clin Nephrol Case Stud
https://www.readbyqxmd.com/read/29043141/antineutrophil-cytoplasmic-antibody-positive-pauci-immune-glomerulonephritis-associated-with-mantle-cell-lymphoma
#2
Kana N Miyata, Nazia A Siddiqi, Lawrence P Kiss, Nikolas B Harbord, James F Winchester
Renal involvement in non-Hodgkin lymphoma, especially mantle cell lymphoma (MCL) is rare. A 77-year-old man presented with acute kidney injury (AKI), which rapidly progressed to dialysis dependence. Kidney biopsy revealed patchy B-cell lymphocytic aggregates in the interstitium, which were positive for cyclin D1, consistent with atypical CD5-negative MCL as confirmed by the detection of translocation t(11;14) by FISH. Crescents were noted in 3 of 26 glomeruli; while PR-3 antineutrophil cytoplasmic antibody (ANCA) positivity and negative immunofluorescence suggested an additional pauci-immune (rapidly progressive) glomerulonephritis pattern of injury...
2017: Clin Nephrol Case Stud
https://www.readbyqxmd.com/read/29018146/anca-and-iga-glomerulonephritis-all-in-one-prognosis-and-complications
#3
Pitchaphon Nissaisorakarn, Vivette D'Agati, Kisra Anis, Belinda Jim
We present the case of a 75-year-old Hispanic woman with known stage 3 chronic kidney disease, long-standing hypertension and type 2 diabetes mellitus who presented with right-sided abdominal pain and acute kidney injury, nephrotic range proteinuria with positive antimyeloperoxidase antibody. A renal biopsy revealed IgA nephropathy with superimposed pauci-immune antineutrophilic cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis. The patient was treated with pulse intravenous methylprednisolone, cyclophosphamide and plasmapheresis...
October 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28912820/granulomatosis-with-polyangiitis-with-myocarditis-and-ventricular-tachycardia
#4
Ramy Magdy Hanna, Eduardo Lopez, James Wilson
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a pulmonary-renal syndrome affecting small and medium sized blood vessels. The disease has a prevalence in studies ranging from 3 to 15.7 cases per 100,000, with a noted increasing incidence and prevalence in more recent studies. Pulmonary manifestations include hemorrhage, lung cavitary lesions, and pulmonary fibrosis. Within the kidney, GPA is known to cause rapidly progressive pauci-immune crescentic glomerulonephritis...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28842398/anca-glomerulonephritis-and-vasculitis
#5
J Charles Jennette, Patrick H Nachman
ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). A diagnosis of ANCA vasculitis should always specify the serotype as MPO-ANCA positive, PR3-ANCA positive, or ANCA-negative. To fully characterize a patient, the serotype also should be accompanied by the clinicopathologic variant if this can be determined: microscopic polyangiitis, granulomatosis with polyangiitis (Wegener), eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or renal-limited vasculitis...
October 6, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28670084/medullary-angiitis-and-pauci-immune-crescentic-glomerulonephritis
#6
Jeffrey Klein, William Rodriguez, Michael Kuperman, Harold Szerlip
Although almost all pathological diagnoses made from a native kidney biopsy come from careful examination of the renal cortex, certain diseases have a characteristic medullary component. Medullary angiitis has histological features of interstitial hemorrhage in the medulla with an associated polymorphonuclear leukocyte infiltrate. These findings are primarily found in the setting of antineutrophil cytoplasmic antibody-associated vasculitis. Medullary angiitis identified in the setting of negative immunofluorescence is most suggestive of pauci-immune crescentic glomerulonephritis, as presented in this case...
July 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28646482/clinical-serological-and-histological-determinants-of-patient-and-renal-outcome-in-anca-associated-vasculitis-with-renal-involvement-an-analysis-from-a-referral-centre
#7
Matija Crnogorac, Ivica Horvatic, Luka Toric, Danica Galesic Ljubanovic, Miroslav Tisljar, Krešimir Galesic
PURPOSE: To evaluate significance of clinical and histopathological prognostic factors for renal and patient outcome in AAV patient cohort. METHODS: Retrospective study included consecutive patients diagnosed with pauci-immune crescentic glomerulonephritis from January 2003 to December 2013. Primary outcome was combined endpoint patient death or progression to end-stage renal disease (ESRD). Secondary outcomes were patient survival and progression to ESRD (renal survival) singularly and disease relapse...
August 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28584626/mmp2-and-mmp9-associate-with-crescentic-glomerulonephritis
#8
Tessa M Phillips, Mitali Fadia, Tom N Lea-Henry, Jonathan Smiles, Giles D Walters, Simon H Jiang
Background: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by multiple organ involvement. Lupus nephritis (LN) is a common manifestation with a wide variety of histological appearances. Matrix metalloproteinases (MMP) 2 and 9 are gelatinases capable of degrading glomerular basement membrane type IV collagen, which have been associated with LN. We examine the expression of MMP2 and MMP9 in different classes of LN. Methods: MMP2 and MMP9 expression was detected by immunohistochemistry in sections from renal biopsy specimens with class III, class IV and class V LN (total n = 31), crescentic immunoglobulin A nephropathy (n = 6), pauci-immune glomerulonephritis (n = 7), minimal change disease (n = 2), mesangiocapillary glomerulonephritis (n = 7), diabetic nephropathy (n = 12) and histologically normal controls (n = 8)...
April 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28580649/myeloperoxidase-antineutrophil-cytoplasmic-antibody-causes-different-renal-diseases-by-immune-complex-formation-and-pauci-immune-mechanism-a-case-report
#9
Shun Manabe, Michiyasu Hatano, Marie Nakano, Teruhiro Fujii, Kosaku Nitta, Michio Nagata
Antineutrophil cytoplasmic antibody (ANCA) has been known to cause pauci-immune crescentic glomerulonephritis. In addition, several reports described membranous glomerulonephritis (MN) concurrent with ANCA-associated glomerulonephritis. Because the two glomerular diseases simultaneously appear in an ANCA-positive patient, the mechanisms whereby ANCA causes the two different glomerular diseases remain ambiguous. Herein, we report a case of 19-year-old man who presented with hematuria, pre-nephrotic proteinuria, and high titer of myeloperoxidase (MPO)-ANCA...
June 4, 2017: Pathology International
https://www.readbyqxmd.com/read/28540906/an-overlap-of-granulomatosis-with-polyangiitis-and-eosinophilic-granulomatosis-with-polyangiitis
#10
Sujit Surendran, Chandramohan Gundappa, Arun Gandhi, Anila Abraham Kurien, Edwin Fernando
We present a case report of overlap of granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome). We report a 45-year-old female who presented with rapidly progressive renal failure associated with fever, polyarthralgia, and respiratory symptoms with cytoplasmic antineutrophilic cytoplasmic antibody (ANCA) and proteinase (PR-3) antigen positivity. Computerized tomography scan of the chest showed diffuse alveolar hemorrhage with renal biopsy revealing pauci-immune necrotizing crescentic glomerulonephritis with intense eosinophilic infiltration suggestive of eosinophilic GPA (EGPA)...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28540889/patterns-of-glomerulonephritis-with-crescents-experience-at-a-tertiary-medical-center-in-saudi-arabia
#11
Turki Al-Hussain, Shuaa Asiri, Sadiq Amer, Hadeel Al Mana, Mohammed Akhtar
A series of 78 cases of glomerulonephritis (GN), in which renal biopsy revealed changes of GN associated with crescent formation, were reviewed. Renal pathology findings were correlated with clinical features including patient's age, renal function, and serologic findings. In most of the cases (71.8%), the crescents were due to immune complex-mediated GN. This was followed by pauci-immune GN (20.5%) and anti-glomerular basement membrane antibody (GBM) GN (7.7%). The percentage of glomeruli with crescents was the highest in cases of anti-GBM disease (mean of 93...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28509135/renal-limited-vasculitis-with-elevated-levels-of-multiple-antibodies
#12
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/28508974/pauci-immune-crescentic-glomerulonephritis-associated-with-use-of-trimethoprim-sulfamethoxazole
#13
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/28432167/a-tale-of-two-vasculitides-biopsy-proven-giant-cell-arteritis-followed-by-the-independent-development-of-renal-limited-microscopic-polyangiitis
#14
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
#15
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
#16
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
#17
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...
June 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
#18
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
#19
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...
February 2017: 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
#20
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
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