2,696 results match your criteria Glomerulonephritis Crescentic


Clinical and pathological characteristics of patients with acute kidney injury in Japan in whom kidney biopsy was performed: a cross-sectional analysis of the Japan Renal Biopsy Registry (J‑RBR).

Clin Exp Nephrol 2022 May 20. Epub 2022 May 20.

Department of Pathology, Keio University School of Medicine, Tokyo, Japan.

Introduction: Acute kidney injury (AKI) is a worldwide concern and it leads to a poor prognosis or end-stage kidney disease. The purpose of this study was to clarify the characteristics of patients with AKI in whom kidney biopsy was performed using data of the Japan Renal Biopsy Registry (J-RBR).

Methods: We screened 38,351 cases that were registered in the J-RBR from 2007 to 2018. Read More

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Analysis of Various Types of Glomerulonephritis with Crescents at a Single Center.

Int J Nephrol 2022 9;2022:1749548. Epub 2022 May 9.

Department of Nephrology, Tokyo Women's Medical University, Tokyo, Japan.

Background: The importance of crescent formation in glomerulonephritis has increased. However, detailed analysis of crescentic glomerulonephritis in Asia is scarce. In addition, advances in serological diagnostic techniques (antineutrophil cytoplasmic and antiglomerular basement membrane autoantibodies) and early diagnosis have reduced the number of cases meeting the strict definition of crescentic glomerulonephritis (>50% of glomeruli are crescentic). Read More

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ANCA-Negative Vasculitis in Eosinophilic Granulomatosis with Polyangiitis Complicated with Membranous Nephropathy: A Case Report and Brief Literature Review.

Case Rep Nephrol 2022 6;2022:8110940. Epub 2022 May 6.

Department of Nephrology, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjyuku-ku, Tokyo 162-0054, Japan.

Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA) typically occurs in anti-neutrophil cytoplasmic autoantibody (ANCA)-positive cases presenting with rapidly progressive renal insufficiency and urinary abnormalities induced by primarily necrotizing crescentic glomerulonephritis (NCGN). Recently, ANCA-negative EGPA has also been reported to manifest with renal involvement, such as NCGN or non-NCGN, including membranous nephropathy (MN). Herein, we report a 70-year-old female who presented with purpura on the lower legs, upper limb numbness, renal dysfunction (eGFR, 20. Read More

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A Rare Case of Nephrotic-Range Proteinuria in Antineutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis.

Cureus 2022 May 10;14(5):e24889. Epub 2022 May 10.

Internal Medicine, BronxCare Health System, New York City, USA.

Granulomatosis with polyangiitis (GPA), or Wegener's granulomatosis as it was formerly referred to, is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). GPA is characterized as a necrotizing vasculitis with few or no immune deposits termed pauci-immune deposits, predominantly affecting small and medium arterial vessels, involving the upper and lower respiratory tract as well as glomeruli. Renal manifestations are of critical importance because of the progression that may ensue following onset. Read More

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Bartonella endocarditis and diffuse crescentic proliferative glomerulonephritis with a full-house pattern of immune complex deposition.

BMC Nephrol 2022 May 12;23(1):181. Epub 2022 May 12.

Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA.

Background: Bartonella endocarditis is often a diagnostic challenge due to its variable clinical manifestations, especially when it is first presented with involvement of organs other than skin and lymph nodes, such as the kidney.

Case Presentation: This was a 13-year-old girl presenting with fever, chest and abdominal pain, acute kidney injury, nephrotic-range proteinuria and low complement levels. Her kidney biopsy showed diffuse crescentic proliferative glomerulonephritis with a full-house pattern of immune complex deposition shown by immunofluorescence, which was initially considered consistent with systemic lupus erythematous-associated glomerulonephritis (lupus nephritis). Read More

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Single-cell RNA Sequencing Identified Novel Nr4a1 Ear2 Anti-Inflammatory Macrophage Phenotype under Myeloid-TLR4 Dependent Regulation in Anti-Glomerular Basement Membrane (GBM) Crescentic Glomerulonephritis (cGN).

Adv Sci (Weinh) 2022 Apr 28:e2200668. Epub 2022 Apr 28.

Department of Medicine and Therapeutics, Li Ka Shing Institute of Health Sciences, and Lui Che Woo Institute of Innovative Medicine, The Chinese University of Hong Kong, Hong Kong, 999077, P. R. China.

Previously, this study demonstrates the critical role of myeloid specific TLR4 in macrophage-mediated progressive renal injury in anti-glomerular basement membrane (anti-GBM) crescentic glomerulonephritis (cGN); however, the underlying mechanism remains largely unknown. In this study, single-cell RNA sequencing (scRNA-seq), pseudotime trajectories reconstruction, and motif enrichment analysis are used, and macrophage diversity in anti-GBM cGN under tight regulation of myeloid-TLR4 is uncovered. Most significantly, a myeloid-TLR4 deletion-induced novel reparative macrophage phenotype (Nr4a1 Ear2+) with significant upregulated anti-inflammatory and tissue repair-related signaling is discovered, thereby suppressing the M1 proinflammatory responses in anti-GBM cGN. Read More

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Epidermal growth factor deficiency predisposes to progressive renal disease.

FASEB J 2022 05;36(5):e22286

Department of Dermatology, Cutaneous Biology Research Center, Massachusetts General Hospital, Harvard Medical School, Charlestown, Massachusetts, USA.

Epidermal growth factor (EGF) is produced in the kidney by thick ascending limbs of the loop of Henle and by distal convoluted tubules (DCTs). Reduced urinary EGF levels have been associated with chronic kidney disease but it is not known whether physiological levels of EGF protect the kidney from progressive renal disease. Here, we show that EGF-deficient mice on a mixed genetic background had increased urinary microalbumin, and a subset of these mice developed severe progressive renal disease with azotemia that was not seen in WT or TGFα-deficient littermates with this mixed genetic background. Read More

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Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Complicated by Thrombotic Microangiopathy with Posterior Reversible Encephalopathy Syndrome Successfully Treated with Eculizumab: A Case Report.

Mod Rheumatol Case Rep 2022 Apr 15. Epub 2022 Apr 15.

Department of Nephrology and Rheumatology, Aichi Medical University, Nagakute, Aichi, Japan.

Thrombotic microangiopathy is characterised by endothelial cell injury, intravascular platelet-fibrin thrombi, and vascular damage, leading to acute kidney injury, thrombocytopenia, and microangiopathic haemolytic anaemia. Among the autoimmune diseases related to thrombotic microangiopathy, anti-neutrophil cytoplasmic antibody-associated vasculitis-related thrombotic microangiopathy cases have been rarely reported; therefore, the optimal treatment for associated vasculitis-related thrombotic microangiopathy remains unknown. An 84-year-old woman without significant medical history presented with a 1-month history of general fatigue, fever, and deteriorating bilateral leg numbness and was admitted to our hospital. Read More

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Emergence of proteinase 3-antineutrophil cytoplasmic antibody-associated glomerulonephritis with mesangial immune deposition during the clinical course of IgG λ monoclonal gammopathy of uncertain significance.

CEN Case Rep 2022 Apr 14. Epub 2022 Apr 14.

Department of Internal Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, Japan.

Patients with monoclonal gammopathy of uncertain significance (MGUS) is sometimes associated with renal diseases, usually due to the deposition of secreted monoclonal immunoglobulin or a fragment thereof, a condition which is defined as monoclonal gammopathy of renal significance. Patients with MGUS appear to be at increased risk for various autoimmune conditions. We report the case of a 68-year-old man developed nephritic syndrome and mild renal insufficiency during the course of IgG λ MGUS. Read More

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Epidemiology, Impact, and Management Strategies of Anti-Glomerular Basement Membrane Disease.

Int J Nephrol Renovasc Dis 2022 7;15:129-138. Epub 2022 Apr 7.

Department of Laboratory Medicine and Pathology, Hamad General Hospital, Hamad Medical Corporation, Doha, Qatar.

Anti-glomerular basement membrane (anti-GBM) disease is a rare but serious autoimmune disease, which is characterized by the development of pathogenic antibodies to type IV collagen antigens in the glomerular and alveolar basement membranes. This results in rapidly progressive glomerulonephritis (GN), alveolar hemorrhage, or both. A variety of environmental factors can trigger the disease in genetically predisposed patients. Read More

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CSF2-dependent monocyte education in the pathogenesis of ANCA-induced glomerulonephritis.

Ann Rheum Dis 2022 Apr 13. Epub 2022 Apr 13.

Experimental and Clinical Research Center, Max Delbrück Center for Molecular Medicine in the Helmholtz Association (MDC) and Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany

Objectives: Myeloid cell activation by antineutrophil cytoplasmic antibody (ANCA) is pivotal for necrotising vasculitis, including necrotising crescentic glomerulonephritis (NCGN). In contrast to neutrophils, the contribution of classical monocyte (CM) and non-classical monocyte (NCM) remains poorly defined. We tested the hypothesis that CMs contribute to antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and that colony-stimulating factor-2 (CSF2, granulocyte-macrophage colony-stimulating factor (GM-CSF)) is an important monocyte-directed disease modifier. Read More

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Acquired aplastic anemia complicated with anti-glomerular basement membrane disease successfully treated with immunosuppressive therapy: a case report.

BMC Nephrol 2022 04 7;23(1):136. Epub 2022 Apr 7.

Kidney Disease and Transplant Center, Shonan Kamakura General Hospital, 1370-1, Okamoto, Kamakura, Kanawaga, 247-8533, Japan.

Background: Aplastic anemia (AA) is a rare but fatal disorder characterized by pancytopenia due to bone marrow hypoplasia. Anti-glomerular basement membrane disease (anti-GBM disease) is an immune complex small-vessel vasculitis that presents as rapidly progressive glomerulonephritis and/or pulmonary hemorrhage. Although both involve autoreactive T cells that are partially triggered by human leukocyte antigen (HLA)-DR15, there have been no reports of their co-existence and the treatment strategy is not well understood. Read More

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Glomerular Disease in Temporal Association with SARS-CoV-2 Vaccination: A Series of 29 Cases.

Kidney360 2021 Nov 16;2(11):1770-1780. Epub 2021 Sep 16.

Kidney Specialists of Southern Nevada, Las Vegas, Nevada.

Background: Immune responses to vaccination are a known trigger for a new onset of glomerular disease or disease flare in susceptible individuals. Mass immunization against SARS-CoV-2 in the COVID-19 pandemic provides a unique opportunity to study vaccination-associated autoimmune kidney diseases. In the recent literature, there are several patient reports demonstrating a temporal association of SARS-CoV-2 immunization and kidney diseases. Read More

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November 2021

Excretion Patterns of Urinary Sediment and Supernatant Podocyte Biomarkers in Patients with CKD.

Kidney360 2022 Jan 5;3(1):63-73. Epub 2021 Nov 5.

Division of Nephrology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.

Background: Podocyte depletion causes glomerulosclerosis, and persistent podocyte loss drives progression to ESKD. Urinary sediment podocin (u-sed Pod) mRNA excretion and urinary supernatant podocalyxin (u-sup PCX) protein have been used to monitor disease activity in glomerular diseases. However, the differences in these markers among pathologies have not been investigated. Read More

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January 2022

A case of crescentic glomerulonephritis in a patient with COVID-19 infection: A case report and literature review.

Medicine (Baltimore) 2022 Feb;101(7):e28754

Department of Internal Medicine, Hamad Medical Corporation, Doha, Qatar.

Rationale: Kidney involvement with COVID-19 infection is a well-known complication, and the majority of kidney involvement is related to ischemic injury/acute tubular injury. However, there are some cases of glomerulonephritis, the etiology of which is not yet known, but an immune process is likely to be the trigger.

Patient Concerns: A 27-year-old man presented to our hospital with facial puffiness and lower-limb swelling. Read More

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February 2022

Atypical Neurological Manifestation in Childhood Microscopic Polyangiitis: A Case Report and Review of Literature.

Front Pediatr 2022 11;10:855338. Epub 2022 Mar 11.

Division of Rheumatology, Department of Pediatrics, Faculty of Medicine Vajira Hospital, Navamindradriraj University, Bangkok, Thailand.

Microscopic polyangiitis (MPA), a systemic necrotizing vasculitis of small vessels, is primarily associated with necrotizing and crescentic glomerulonephritis and pulmonary capillaritis. Neurological involvement, particularly of the central nervous system (CNS) is scarcely observed. The diversity of CNS symptoms could puzzle the diagnosis causing delays in treatment and potentially having a considerable effect on patient's quality of life or even death. Read More

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Atypical Conjunctival Lesion as the Initial Presentation of Granulomatosis With Polyangiitis in an Adolescent Male.

Cornea 2022 Mar 31. Epub 2022 Mar 31.

Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami, Miami, FL.

Purpose: The purpose of this study was to report the first case of a conjunctival granulomatous lesion as the presenting sign of granulomatous polyangiitis (GPA) in a pediatric patient.

Methods: This study is a case report.

Results: A 14-year-old Hispanic boy presented with a conjunctival lesion on the inferior bulbar conjunctiva of the right eye associated with diffuse conjunctival injection. Read More

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Biopsy-proven CKD etiology and outcomes: Chronic Kidney Disease Japan Cohort (CKD-JAC) study.

Nephrol Dial Transplant 2022 Mar 22. Epub 2022 Mar 22.

Division of Nephrology, Endocrinology and Metabolism, Tokai University School of Medicine, Isehara, Japan.

Background: The KDIGO guidelines advocate the cause-GFR-albuminuria (CGA) classification for predicting outcomes. However, there is a dearth of data supporting the use of the cause of chronic kidney disease (CKD). This study aimed to address how to incorporate a prior biopsy-proven diagnosis in outcome prediction. Read More

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Case Report: Clinical and Pathological Findings of a Recurrent C3 Glomerulopathy With Superimposed Membranoproliferative Glomerulonephritis Pattern and Cryoglobulinemia Associated With COVID-19.

Front Pediatr 2022 4;10:827466. Epub 2022 Mar 4.

Department of Pathology, Carver College of Medicine, University of Iowa, Iowa City, IA, United States.

Coronavirus disease 2019 (COVID-19) may cause a wide spectrum of kidney pathologies. The impact of COVID-19 is unclear in the context of the complement system abnormalities, including C3 glomerulopathy (C3G). In this report, we describe a young adult receiving a kidney transplant for C3 glomerulopathy (C3G), a disorder of the alternative complement pathway. Read More

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Rapidly progressive glomerulonephritis in children.

Pak J Med Sci 2022 Jan;38(2):417-425

Farhana Amanullah, MBBS, DABP, FAAP. Pediatric Nephrologist, The Indus Hospital and Health Network, and the Aga Khan University Hospital, Karachi, Pakistan.

Rapidly progressive glomerulonephritis (RPGN), characterized by a rapid development of nephritis with loss of kidney function in days or weeks, is typically associated histologically, with crescents in most glomeruli; and is a challenging problem, particularly in low resource settings. RPGN is a diagnostic and therapeutic emergency requiring prompt evaluation and treatment to prevent poor outcomes. Histopathologically, RPGN consists of four major categories, anti-glomerular basement membrane (GBM) disease, immune complex mediated, pauci-immune disorders and idiopathic /overlap disorders. Read More

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January 2022

Digital Spatial Profiling of Individual Glomeruli From Patients With Anti-Neutrophil Cytoplasmic Autoantibody-Associated Glomerulonephritis.

Front Immunol 2022 2;13:831253. Epub 2022 Mar 2.

Department of Nephrology, Hunan Key Laboratory of Kidney Disease and Blood Purification, Institute of Nephrology, The Second Xiangya Hospital at Central South University, Changsha, China.

We previously showed that the rupture of Bowman's capsule (BC) promotes the progression of crescentic glomerulonephritis by enhancing the entry of CD8 T cells into the glomeruli. In the present study, we utilized digital spatial profiling to simultaneously profile the altered abundances of the messenger RNA (mRNA) transcripts and proteins in the glomerular and periglomerular areas of four biopsy samples of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis (ANCA-GN) and two biopsy specimens of minimal change disease (MCD) controls. The paraffin-embedded biopsy samples were stained with collagen IV, CD45, and SYTO 13 to distinguish the glomeruli with periglomerular infiltration but intact BC, with focal BC rupture, and with extensive rupture of BC and glomeruli without crescent formation and leukocytic infiltration in ANCA-GN. Read More

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A Novel Association Between Coronavirus Disease 2019 and Normocomplementemic Rapidly Progressive Glomerulonephritis-Crescentic Immunoglobulin A Nephropathy: A Report of Two Pediatric Cases.

Cureus 2022 Feb 10;14(2):e22077. Epub 2022 Feb 10.

Nephrology, All India Institute of Medical Sciences, Rishikesh, Rishikesh, IND.

Coronavirus disease 2019 (COVID-19), caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has been predominately associated with respiratory illness. Acute kidney injury (AKI) is the most common reported kidney involvement. Kidney complications, including proteinuria, hematuria, and rarely collapsing glomerulopathy (CG), a form of focal segmental glomerulosclerosis (FSGS), are also well known now and are frequently documented in the literature published so far. Read More

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February 2022

Rapidly progressive crescentic diabetic nephropathy: two case reports.

Intern Med J 2022 Mar;52(3):479-484

Kidney Centre, Royal Prince Alfred Hospital, Kidney Node Charles Perkins Centre, University of Sydney, Sydney, New South Wales, Australia.

Crescents are not a well recognised feature of diabetic nephropathy. We present two cases of patients presenting with a rapid decline in renal function and subacute onset of nephrotic syndrome. Glomerulonephritis screening was negative, and renal biopsy revealed non-necrotising cellular crescents and typical features of late-stage diabetic nephropathy. Read More

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Targeting Cathepsin C in PR3-ANCA Vasculitis.

J Am Soc Nephrol 2022 05 15;33(5):936-947. Epub 2022 Mar 15.

Experimental and Clinical Research Center, Max Delbrueck Center for Molecular Medicine and Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health, Berlin, Germany

Background: The ANCA autoantigens proteinase 3 (PR3) and myeloperoxidase (MPO) are exclusively expressed by neutrophils and monocytes. ANCA-mediated activation of these cells is the key driver of the vascular injury process in ANCA-associated vasculitis (AAV), and neutrophil serine proteases (NSPs) are disease mediators. Cathepsin C (CatC) from zymogens activates the proteolytic function of NSPs, including PR3. Read More

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Real-life experience with inotersen in hereditary transthyretin amyloidosis with late-onset phenotype: Data from an early-access program in Italy.

Eur J Neurol 2022 Mar 15. Epub 2022 Mar 15.

Department of Neuroscience, Reproductive and Odontostomatological Science, University of Naples "Federico II", Naples, Italy.

Background And Purpose: Hereditary transthyretin (TTR) amyloidosis (ATTRv) is a dominantly inherited, adult-onset, progressive, and fatal disease caused by mutations in the transthyretin gene. Therapeutic agents approved for this disease include the TTR stabilizer tafamidis and the gene-silencing drugs patisiran and inotersen. Inotersen is an antisense oligonucleotide that suppresses the hepatic production of transthyretin. Read More

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Glomerulonephritis with crescents in childhood; etiologies and significance of M2 macrophages.

Turk J Pediatr 2022 ;64(1):59-68

Divisions of Pediatric Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Background: Crescentic glomerulonephritis (CGN) is a rapidly progressive and rare cause of glomerulonephritis in childhood. The aim of this study is to evaluate demographic data of children with crescentic glomerulonephritis, to classify the etiologies and to investigate the correlation between the severity of kidney disease and the expression of CD163+ macrophages.

Methods: Between the years 2000 and 2016 in a single center, patients under 18 years of age with kidney biopsies containing crescents were included in the study. Read More

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The Critical Role of Therapeutic Plasma Exchange in the Treatment of MPO-ANCA Associated Crescentic IgA Nephropathy.

Iran J Kidney Dis 2022 Jan;1(1):63-65

Department of Nephrology, Gulhane Training and Research Hospital, University of Health Sciences, Ankara, Turkey.

Crescentic IgA nephropathy (IgAN) with the positivity for antineutrophilic cytoplasmic antibody (ANCA) is a novel and uncommon entity. The optimal management of this condition is not well-defined. We report a 49-years-old woman with complaints of skin rash and swelling of lower limbs. Read More

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January 2022

Endocarditis-associated rapidly progressive glomerulonephritis mimicking vasculitis: a diagnostic and treatment challenge.

Ann Med 2022 Dec;54(1):754-763

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.

Background: Infective endocarditis (IE)-associated rapidly progressive glomerulonephritis (RPGN) is rarely reported. Sporadic case reports have noted the diagnostic and therapeutic challenge in IE-associated glomerulonephritis because it may masquerade as idiopathic vasculitis.

Methods: Patients with clinical diagnosis of IE-related RPGN in a tertiary hospital in China between January 2004 and May 2021 were identified and retrospectively reviewed. Read More

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December 2022

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Developing Pancreatic Lesion and Diabetes Mellitus: A Case Report and Review of the Literature.

Tohoku J Exp Med 2022 02;256(2):161-168

Department of Nephrology, Shinshu University Hospital.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) affects small blood vessels and causes severe systemic organ injury commonly affecting the lungs and kidney. However, gastrointestinal, especially pancreatic, lesions are rare. We report the case of a 67-year-old Japanese man diagnosed with myeloperoxidase (MPO) AAV who developed pancreatic lesions and diabetes mellitus. Read More

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February 2022