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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/28984299/medical-renal-diseases-are-frequent-but-often-unrecognized-in-adult-autopsies
#4
Marie E Perrone, Anthony Chang, Kammi J Henriksen
Kidney diseases affect many hospitalized patients and contribute to morbidity and mortality. Therefore, kidney disease should be prevalent, but the frequency and spectrum of medical renal pathology in autopsy specimens has not been well documented. We sought to determine the spectrum of medical renal pathology in adult autopsy specimens and the frequency of overlooked diagnoses. We reviewed the hematoxylin- and eosin-stained kidney sections from 140 adult autopsies performed at a large teaching hospital over a 2-year period...
October 6, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28937084/primary-sj%C3%A3-gren-s-syndrome-complicated-by-anti-neutrophil-cytoplasmic-antibody-mediated-crescentic-glomerulonephritis
#5
Imed Ben Ghorbel, Nabil Belfeki, Thouraya Ben Salem, Mohamed Habib Houman
Ocular and oral dryness are the hallmark of Sjögren's syndrome (SS). However, SS can be associated with a variety of complications, affecting organs such as the liver, kidneys, lungs, muscle, and nervous system. Renal involvement has been usually in the form of tubulointerstitial nephritis. However, glomerulonephritis is rare in primary SS. We report three clinical cases of SS with anti-neutrophil cytoplasmic antibody-mediated crescentic glomerulo-nephritis treated with prednisone and cyclophosphamide, with favorable outcome...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28937063/a-mini-epidemic-of-anti-glomerular-basement-membrane-disease-clinical-and-epidemiological-study
#6
Umesh Lingaraj, Shivaprasad Sasivehalli Mallappa, Rajiv Elkal Neminah, Satishkumar Madakala Mohan, Leelavathi Venkatesh, Sreedhara Chikkanayakanahalli Gurusiddaiah, Niranjan Manibally Rachaiah
Acute glomerulonephritis due to anti-glomerular basement membrane (anti-GBM) antibody disease is rare, estimated to occur in fewer than one case per million population and accounts for less than 20% of rapidly progressive glomerulonephritis. The prevalence among patients evaluated for potential glomerular disease is lower. It accounts for fewer than 3% of all kidney biopsies done with crescentic glomerulonephritis. Cases of anti-GBM disease occurring in a cluster have rarely been reported. All biopsy proven anti-GBM disease cases were collected from January 2015 to March 2015 at our Institute...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28918828/crescentic-glomerulonephritis-with-immunoglobulin-g4-related-disease
#7
Inbar Raber, Asiri Ediriwickrema, John Higgins, Neeraja Kambham, Alan C Pao
Immunoglobulin G4 (IgG4)-related disease is an uncommon autoimmune disease that affects multiple organ systems. Renal involvement typically presents as tubulointerstitial nephritis and less commonly as membranous glomerulonephritis. In this case report, we discuss a 68-year-old patient who presented with rapidly progressive glomerulonephritis. His renal biopsy revealed a membranoproliferative pattern of injury with fibrocellular crescents and extensive infiltration of the tubulointerstitium with IgG4-positive plasma cells...
September 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28912820/granulomatosis-with-polyangiitis-with-myocarditis-and-ventricular-tachycardia
#8
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/28891817/neutrophil-fc%C3%AE-riia-promotes-igg-mediated-glomerular-neutrophil-capture-via-abl-src-kinases
#9
Hiroshi Nishi, Kazuhiro Furuhashi, Xavier Cullere, Gurpanna Saggu, Mark J Miller, Yunfeng Chen, Florencia Rosetti, Samantha L Hamilton, Lihua Yang, Spencer P Pittman, Jiexi Liao, Jan M Herter, Jeffrey C Berry, Daniel J DeAngelo, Cheng Zhu, George C Tsokos, Tanya N Mayadas
The kidney glomerular capillaries are frequent sites of immune complex deposition and subsequent neutrophil accumulation in post-infectious and rapidly progressive glomerulonephritis. However, the mechanisms of neutrophil recruitment remain enigmatic, and there is no targeted therapeutic to avert this proximal event in glomerular inflammation. The uniquely human activating Fc receptor FcγRIIA promotes glomerular neutrophil accumulation and damage in anti-glomerular basement membrane-induced (anti-GBM-induced) glomerulonephritis when expressed on murine neutrophils...
October 2, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28883245/the-sequential-development-of-antiglomerular-basement-membrane-nephritis-and-myeloperoxidase-antineutrophil-cytoplasmic-antibody-associated-vasculitis
#10
Naro Ohashi, Akio Namikawa, Masafumi Ono, Takamasa Iwakura, Shinsuke Isobe, Takayuki Tsuji, Akihiko Kato, Hideo Yasuda
A 55-year-old woman presented with deafness, increased levels of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA), and renal insufficiency with proteinuria and hematuria. Renal biopsy revealed crescentic glomerulonephritis with the linear deposition of immunoglobulin G along the glomerular basement membrane (GBM) and peritubular capillaritis. The anti-GBM antibody levels on admission and 10 days after admission were 11.7 U/mL and 127 U/mL, respectively. These results indicated the sequential development of anti-GBM nephritis and MPO-ANCA-associated vasculitis...
October 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28881339/interstitial-immunostaining-and-renal-outcomes-in-antineutrophil-cytoplasmic-antibody-associated-glomerulonephritis
#11
Duvuru Geetha, Sanjeev Sethi, An S De Vriese, Ulrich Specks, Cees G M Kallenberg, Noha Lim, Robert Spiera, E William St Clair, Peter A Merkel, Philip Seo, Paul A Monach, Nicola Lepori, Barri J Fessler, Carol A Langford, Gary S Hoffman, Rishi Sharma, John H Stone, Fernando C Fervenza
BACKGROUND: Immunopathologic features predict renal function at baseline and follow-up in antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN). The interstitial infiltrate consists predominantly of T lymphocytes, but their pathophysiologic significance is unclear, especially in light of the success of B-cell-directed therapy. METHODS: Renal biopsies from 33 patients treated with cyclophosphamide (CYC; n = 17) or rituximab (RTX; n = 16) in the RTX in ANCA-associated vasculitis (RAVE) trial were classified according to the new ANCA GN classification...
2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28880680/the-effect-and-possible-clinical-efficacy-of-in-vivo-inhibition-of-neutrophil-extracellular-traps-by-blockade-of-pi3k-gamma-on-the-pathogenesis-of-microscopic-polyangiitis
#12
Hirotaka Kimura, Yasushi Matsuyama, Sachiko Araki, Atsushi Koizumi, Yumi Kariya, Shunsuke Takasuga, Satoshi Eguchi, Hiroki Nakanishi, Junko Sasaki, Takehiko Sasaki
OBJECTIVE: Neutrophil extracellular traps (NETs) are peculiar structures composed of the externalized chromatin with intracellular proteins and formed by activated neutrophils in a reactive oxygen species (ROS)-dependent manner. Aberrant NETs are considered to be autoantigens for anti-neutrophil cytoplasmic antibodies (ANCAs) underling the development of microscopic polyangiitis (MPA). However, little is known regarding the therapeutic efficacy of in vivo inhibition of NET formation (NETosis) on MPA pathogenesis...
September 7, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28842398/anca-glomerulonephritis-and-vasculitis
#13
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/28807596/the-role-of-angiotensin-ii-in-parietal-epithelial-cell-proliferation-and-crescent-formation-in-glomerular-diseases
#14
Paola Rizzo, Rubina Novelli, Cinzia Rota, Elena Gagliardini, Barbara Ruggiero, Daniela Rottoli, Ariela Benigni, Giuseppe Remuzzi
Crescentic glomerulonephritis (GN) is a devastating disease with rapidly progressive deterioration in kidney function, which, histologically, manifests as crescent formation in most glomeruli. We previously found that crescents derive from the aberrant proliferation and migration of parietal epithelial cells (PECs)/progenitor cells, and that the angiotensin (ang) II/ang II type-1 (AT1) receptor pathway may participate, together with the stromal cell-derived factor-1 (SDF-1)/C-X-C chemokine receptor 4 axis, in the development of those lesions...
August 12, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28756872/retinoic-acid-improves-nephrotoxic-serum-induced-glomerulonephritis-through-activation-of-podocyte-retinoic-acid-receptor-%C3%AE
#15
Yan Dai, Anqun Chen, Ruijie Liu, Leyi Gu, Shuchita Sharma, Weijing Cai, Fadi Salem, David J Salant, Jeffrey W Pippin, Stuart J Shankland, Marcus J Moeller, Norbert B Ghyselinck, Xiaoqiang Ding, Peter Y Chuang, Kyung Lee, John Cijiang He
Proliferation of glomerular epithelial cells, including podocytes, is a key histologic feature of crescentic glomerulonephritis. We previously found that retinoic acid (RA) inhibits proliferation and induces differentiation of podocytes by activating RA receptor-α (RARα) in a murine model of HIV-associated nephropathy. Here, we examined whether RA would similarly protect podocytes against nephrotoxic serum-induced crescentic glomerulonephritis and whether this effect was mediated by podocyte RARα. RA treatment markedly improved renal function and reduced the number of crescentic lesions in nephritic wild-type mice, while this protection was largely lost in mice with podocyte-specific ablation of Rara (Pod-Rara knockout)...
July 27, 2017: Kidney International
https://www.readbyqxmd.com/read/28731865/-joining-the-spots-in-adults-and-young-tots-a-clinicopathological-study-of-henoch-sch%C3%A3-nlein-purpura-iga-vasculitis
#16
Priyanka P Kedia, Rajalakshmi Tirumalae, Divya Puttegowda, Meryl Antony
INTRODUCTION: Henoch-Schönlein Purpura (HSP; IgA vasculitis), the most common vasculitis of childhood, has a wide spectrum of clinical manifestations ranging from palpable purpura with abdominal pain and arthritis to the more morbid renal involvement. AIMS AND OBJECTIVES: To study and correlate the clinical presentation, laboratory values, skin and renal histopathology and immunofluoroscence findings in HSP. MATERIAL AND METHODS: A total of 44 cases of HSP from March 2011 to February 2014 were studied for the above features along with their clinical outcomes...
August 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28699638/bcat1-controls-metabolic-reprogramming-in-activated-human-macrophages-and-is-associated-with-inflammatory-diseases
#17
Adonia E Papathanassiu, Jeong-Hun Ko, Martha Imprialou, Marta Bagnati, Prashant K Srivastava, Hong A Vu, Danilo Cucchi, Stephen P McAdoo, Elitsa A Ananieva, Claudio Mauro, Jacques Behmoaras
Branched-chain aminotransferases (BCAT) are enzymes that initiate the catabolism of branched-chain amino acids (BCAA), such as leucine, thereby providing macromolecule precursors; however, the function of BCATs in macrophages is unknown. Here we show that BCAT1 is the predominant BCAT isoform in human primary macrophages. We identify ERG240 as a leucine analogue that blocks BCAT1 activity. Selective inhibition of BCAT1 activity results in decreased oxygen consumption and glycolysis. This decrease is associated with reduced IRG1 levels and itaconate synthesis, suggesting involvement of BCAA catabolism through the IRG1/itaconate axis within the tricarboxylic acid cycle in activated macrophages...
July 12, 2017: Nature Communications
https://www.readbyqxmd.com/read/28676642/label-free-detection-of-sensitive-mid-infrared-biomarkers-of-glomerulonephritis-in-urine-using-fourier-transform-infrared-spectroscopy
#18
Mei-Ching Yu, Peter Rich, Liberty Foreman, Jennifer Smith, Mei-Shiuan Yu, Anisha Tanna, Vinod Dibbur, Robert Unwin, Frederick W K Tam
More reliable biomarkers using near-patient technologies are needed to improve early diagnosis and intervention for patients with renal disease. Infrared (IR) vibrational spectroscopy/microspectroscopy is an established analytical method that was first used in biomedical research over 20 years ago. With the advances in instrumentation, computational and mathematical techniques, this technology has now been applied to a variety of diseases; however, applications in nephrology are just beginning to emerge. In the present study, we used attenuated total reflectance Fourier transform infrared (ATR-FTIR) spectroscopy to analyze urine samples collected from rodent models of inflammatory glomerulonephritis (GN) as well as from patients with crescentic GN, with the aim of identifying potential renal biomarkers; several characteristic mid-IR spectral markers were identified in urine samples...
July 4, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28670084/medullary-angiitis-and-pauci-immune-crescentic-glomerulonephritis
#19
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/28667835/monocytes-promote-crescent-formation-in-anti-myeloperoxidase-antibody-induced-glomerulonephritis
#20
Anthony Rousselle, Ralph Kettritz, Adrian Schreiber
Neutrophils and monocytes express anti-neutrophil cytoplasmic antibody (ANCA) antigens, and activation of these cells by ANCA is central to ANCA-associated vasculitis and necrotizing crescentic glomerulonephritis (NCGN). The importance of neutrophils is established; however, any role of monocytes is less clear. We tested the hypothesis that depletion of CCR2(+) inflammatory monocytes and their derivatives would abrogate anti-myeloperoxidase (MPO) antibody-induced NCGN in a mouse model. We used passive anti-MPO antibody transfer for NCGN induction in wild-type mice or mice expressing the CCR2 promoter-controlled diphtheria toxin receptor...
September 2017: American Journal of Pathology
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