2,093 results match your criteria Glomerulonephritis Rapidly Progressive

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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Kidney Disease in Ankylosing Spondylitis: a case series and review of the literature.

J Bras Nefrol 2022 May 13. Epub 2022 May 13.

Centro Hospitalar de Lisboa Central, Hospital Curry Cabral, Departamento de Nefrologia, Lisboa, Portugal.

Background: Kidney disease is a rare manifestation of ankylosing spondylitis (AS) and its pathological alterations remain poorly described. The aim of this study was to investigate the clinical presentation and pathological alterations on kidney biopsy of AS patients and review and discuss the current literature on the issue.

Methods: We retrospectively studied the clinical presentation and kidney pathological alterations of 15 Caucasian AS patients submitted to kidney biopsy between October 1985 and March 2021. 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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Successful Treatment of COVID-19-Related Immune- Complex Glomerulonephritis, Case Report.

Iran J Kidney Dis 2022 Mar;16(2):147-151

University of Health Sciences, Gazi Yasargil Training and Research Hospital, Nephrology Department , Diyarbakir, Turkey.

Acute kidney injury (AKI) , proteinuria in the nephrotic or subnephrotic range and hematuria might be seen in patients with coronavirus disease 2019 (COVID-19) infection. In this case study we present a 59 years old manwho was diagnosed with immune-complex glomerulonephritis after development of rapidly progressive kidney failure accompanied by pulmonary hemorrhage, 2 months after COVID-19 infection. The patient was hospitalised with the diagnosis of acute kidney injury and nephrotic syndrome. Read More

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Eosinophilic granulomatosis with polyangiitis complicated with rapidly progressive glomerulonephritis in a young man who is a healthy cyclist.

G Ital Nefrol 2022 Apr 21;39(2). Epub 2022 Apr 21.

Nephrology and Dialysis Unit, San Giovanni Evangelista Hospital, Tivoli, Italy.

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic vasculitis. Rapidly progressive glomerulonephritis (RPGN) is a rare complication of EGPA. We report a case of a 60-year-old man, who is also a skilled cyclist, who was hospitalized to investigate a symptomatology that had arisen over the previous months and worsened in the last few weeks, to the point of limiting normal everyday activities. Read More

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Acute glomerulonephritis.

Lancet 2022 04;399(10335):1646-1663

Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA. Electronic address:

Glomerulonephritis is a heterogeneous group of disorders that present with a combination of haematuria, proteinuria, hypertension, and reduction in kidney function to a variable degree. Acute presentation with full blown nephritic syndrome or rapidly progressive glomerulonephritis is uncommon and is mainly restricted to patients with post-infectious glomerulonephritis, anti-neutrophil cytoplasmic antibodies-associated vasculitis, and anti-glomerular basement membrane disease. Most frequently, patients present with asymptomatic haematuria and proteinuria with or without reduced kidney function. 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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Evaluation of a newly proposed renal risk score for Japanese patients with ANCA-associated glomerulonephritis.

Clin Exp Nephrol 2022 Apr 8. Epub 2022 Apr 8.

Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, Akita, 010-8543, Japan.

Background: We determined the usefulness and prognostic ability of the renal risk score (RRS), proposed in Europe, for Japanese patients with antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (AAGN) and high myeloperoxidase (MPO)-ANCA positivity; these aspects remain to be verified.

Methods: This retrospective study was conducted on 86 Japanese patients with new, biopsy-confirmed AAGN. We calculated the RRS and analyzed the relationship between this classification, and clinicopathological features and prognosis. 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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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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New Onset Biopsy-Proven Nephropathies after COVID Vaccination.

Am J Nephrol 2022 30;53(4):325-330. Epub 2022 Mar 30.

University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-net, ERN-Reconnect and RITA-ERN Member) including the Nephrology and Dialysis Unit, Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley (North-West Italy), Department of Clinical and Biological Sciences, San Giovanni Bosco Hub Hospital, University of Turin, Turin, Italy.

Introduction: To date, almost 7 billion doses of the different types of vaccine against SARS-CoV-2 have been administered worldwide. Although the severity of new cases of SARS-CoV-2 has progressively decreased, and the pressure on national health systems has declined, the development of de novo glomerular injuries has been suggested.

Methods: This study aimed to examine the patients who were hospitalized in our Unit between April and November 2021 and underwent renal biopsy for new-onset urinary abnormalities (UA) and/or renal impairment within 3 months of SARS-CoV-2 vaccination. Read More

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Is There Still a Role of Plasma Exchange in the Current Management of ANCA-Associated Vasculitides?

Curr Rheumatol Rep 2022 Apr 22;24(4):111-117. Epub 2022 Mar 22.

Norwich Medical School, University of East Anglia, Norwich, Norfolk, NR4 7TJ, UK.

Purpose Of Review: Plasma exchange (PLEX) is often recommended as an adjunctive therapy for patients with ANCA-associated vasculitis (AAV) in the setting of rapidly progressive glomerulonephritis or diffuse alveolar haemorrhage. Since ANCAs are pathogenic, it seems a reasonable and justified approach to remove them through therapeutic PLEX, as despite advances in immunosuppressive therapy regimens, AAV is associated with significant morbidity and death. However, the association between ANCA levels and mortality or disease activity is uncertain. 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

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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Concomitant rapidly progressive glomerulonephritis and acute rheumatic fever after streptococcus infection: a case report.

Paediatr Int Child Health 2022 Mar 17:1-5. Epub 2022 Mar 17.

Divisions of Paediatric Nephrology, Faculty of Medicine, Vajira Hospital, Navamindradhiraj University, Bangkok, Thailand.

Acute post-streptococcal glomerulonephritis (APSGN) and acute rheumatic fever (ARF) are common immune-mediated complications after group A streptococcus (GAS) infection. The causative antigenic epitopes on GAS are different for APSGN and ARF, and their simultaneous occurrence is uncommon. A 12-year-old boy presented with fever and gross haematuria. 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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A Case of Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis Superimposed on Post-streptococcal Acute Glomerulonephritis.

Intern Med 2022 Mar 12. Epub 2022 Mar 12.

Department of Nephrology, Nippon Medical School, Japan.

A 44-year-old woman was admitted due to gross hematuria and progressive renal dysfunction. Poststreptococcal acute glomerulonephritis (PSAGN) was suspected due to her elevated anti-streptolysin O and anti-streptokinase titers and hypocomplementemia. A renal biopsy showed crescent formation and endocapillary hypercellularity with neutrophil infiltrate. Read More

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ANCA-associated vasculitis in idiopathic pulmonary fibrosis: A case report and brief review of the literature.

Medicine (Baltimore) 2022 Mar;101(9):e29008

XIIIth Department of Pulmonology, Center for Research and Innovation in Precision Medicine of Respiratory Diseases, "Victor Babes" University of Medicine and Pharmacy Timisoara, Timişoara, Romania.

Rationale: Idiopathic pulmonary fibrosis (IPF) is a progressive disease with poor prognosis. Patients with IPF represent a heterogeneous population with several described clinical phenotypes. More recently, the development of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in IPF patients, with an incidence higher than that in the general population, has drawn attention. Read More

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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

IgA Vasculitis Without Typical Skin Rash Concomitated With c-ANCA Positivity.

Clin Med Insights Case Rep 2022 25;15:11795476221077790. Epub 2022 Feb 25.

Pediatrics, Alhada Armed Forces Hospital, Taif, Kingdom of Saudi Arabia.

We describe a case of IgA vasculitis without typical skin rash concomitated with c-ANCA positivity in a 6-year-old boy who presented with persistent severe generalized colicky abdominal pain, recurrent episodes of vomiting, non-pitting edema of both hands and feet, both knees and ankles arthritis with no associated skin rash following a history of an upper respiratory tract infection 2 weeks before presentation. Initially, he had normal laboratory findings apart from sub-nephrotic range proteinuria and microscopic hematuria in his urine analysis. Two weeks later, he started to have hypertension, gross hematuria, nephrotic range proteinuria, marked elevation of serum urea and creatinine associated with positive serum C-ANCA. Read More

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February 2022

Rapidly Progressive Glomerulonephritis in Human Brucellosis.

Saudi J Kidney Dis Transpl 2021 Jul-Aug;32(4):1171-1175

Department of Internal Medicine, Jordan Medical Services, Amman, Jordan.

Human brucellosis is a complex multisystem disease that can affect many organs. Renal involvement in human brucellosis is rarely found. Here, we present a case report of a patient who presented to us with acute kidney injury, complicating brucellosis. Read More

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Use of Immunosuppressive Therapy in the Treatment of IgA-dominant Infection-related Glomerulonephritis.

Intern Med 2022 1;61(5):697-701. Epub 2022 Mar 1.

Department of Nephrology, Kameda Medical Center, Japan.

A 51-year-old Japanese man who experienced colon cancer recurrence following primary and metastatic lesion resection was hospitalized due to facial cellulitis with febrile neutropenia and purpura on his lower extremities after chemotherapy. It was complicated by rapidly progressive glomerulonephritis. He was diagnosed with immunoglobulin A (IgA)-dominant endocapillary proliferative glomerulonephritis based on kidney histology. Read More

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Not Lupus Nephritis but a Rare Case of Drug-Induced Pauci-Immune Glomerulonephritis.

Cureus 2022 Jan 24;14(1):e21549. Epub 2022 Jan 24.

Nephrology, St. Joseph Mercy Oakland Hospital, Pontiac, USA.

Hydralazine-induced pauci-immune glomerulonephritis is a rare cause of glomerulonephritis. It is an anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis that can be rapidly progressive and potentially life-threatening. However, most cases are found to be asymptomatic, and patients often present with acute renal failure and painless hematuria. Read More

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January 2022

Infective Endocarditis-Associated Glomerulonephritis: A Comprehensive Review of the Clinical Presentation, Histopathology, and Management.

Yonago Acta Med 2022 Feb 28;65(1):1-7. Epub 2022 Jan 28.

Division of Gastroenterology and Nephrology, Department of Multidisciplinary Internal Medicine, School of Medicine, Faculty of Medicine, Tottori University, Yonago 683-8504, Japan.

A significant proportion of patients with infective endocarditis presents with acute renal failure related to infective endocarditis-associated glomerulonephritis (IEAGN). However, the clinical presentation of IEAGN differs from that of other infection-related glomerulonephritis (IRGN) with anti-neutrophil cytoplasmic antibody (ANCA) positivity occurring in almost one-third of cases; therefore, it may be difficult to establish a definitive diagnosis and provide appropriate treatment. This review article provides a comprehensive understanding of the clinical presentation, investigations, histopathology, and treatment/management of IEAGN so that clinicians can keep this differential in mind for patients with fever of unknown origin accompanied by signs and symptoms of acute renal failure. Read More

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February 2022

The effects of plasma exchange in patients with ANCA-associated vasculitis: an updated systematic review and meta-analysis.

BMJ 2022 02 25;376:e064604. Epub 2022 Feb 25.

Department of Medicine, University of Cambridge, United Kingdom.

Objective: To assess the effects of plasma exchange on important outcomes in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV).

Design: Systematic review and meta-analysis of randomised controlled trials.

Eligibility Criteria: Randomised controlled trials investigating effects of plasma exchange in patients with AAV or pauci-immune rapidly progressive glomerulonephritis and at least 12 months' follow-up. Read More

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February 2022

Epigenetic Modulation of Gremlin-1/NOTCH Pathway in Experimental Crescentic Immune-Mediated Glomerulonephritis.

Pharmaceuticals (Basel) 2022 Jan 20;15(2). Epub 2022 Jan 20.

Cellular Biology in Renal Diseases Laboratory, IIS-Fundación Jiménez Díaz, Universidad Autónoma Madrid, 28040 Madrid, Spain.

Crescentic glomerulonephritis is a devastating autoimmune disease that without early and properly treatment may rapidly progress to end-stage renal disease and death. Current immunosuppressive treatment provides limited efficacy and an important burden of adverse events. Epigenetic drugs are a source of novel therapeutic tools. Read More

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January 2022

Pulmonary-Renal Syndrome: A Real-World Experience From a Tertiary Care Pulmonary Center in North India.

Cureus 2022 Jan 17;14(1):e21327. Epub 2022 Jan 17.

Pulmonary and Critical Care Medicine, Metro Centre for Respiratory Disease, Noida, IND.

Background: Pulmonary renal syndrome (PRS) is a simultaneous occurrence of diffuse alveolar hemorrhage (DAH) and glomerulonephritis (GN). The diagnosis of PRS not only requires a high index of clinical suspicion and prompt management, but it is often fatal due to rapidly progressive clinical deterioration despite aggressive treatment. The authors, therefore, share the real-world experience of PRS presenting to tertiary care pulmonary center in north India. Read More

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January 2022

Chimeric Fusion between IgA Protease and IgG Fc Provides Long-Lasting Clearance of IgA Deposits in Mouse Models of IgA Nephropathy.

J Am Soc Nephrol 2022 05 16;33(5):918-935. Epub 2022 Feb 16.

Department of Medicine, Division of Nephrology and Hypertension, Feinberg Cardiovascular and Renal Research Institute, Northwestern University Feinberg School of Medicine, Chicago, Illinois

Background: IgA nephropathy is a common primary glomerulonephritis caused by mesangial deposition of poly-IgA complexes. The disease follows a variable course of clinical progression, with a high risk of kidney failure. Although no specific therapy is available, enzymatic strategies to clear IgA deposits are being considered for the treatment of rapidly progressive IgA nephropathy. Read More

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