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Clin Immunol 2020 May 29:108488. Epub 2020 May 29.

Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Laiko Hospital, Athens, Greece. Electronic address:

B-cell clonal expansion has been sporadically described in the blood and/or renal tissue of patients with glomerulonephritides, albeit with unclear pathogenetic role. Herein, using spectratyping analysis, we observed oligoclonal intrarenal B-cell populations in 59% of glomerulonephritis patients with podocyte injury (6/7 with focal segmental glomerulosclerosis, 1/3 minimal change disease, 1/3 idiopathic membranous nephropathy, 3/4 IgA nephropathy, 2/5 membranous lupus nephritis), 20% of glomerulonephritis patients without podocyte involvement (4/13 with mesangial or proliferative lupus nephritis, 0/3 idiopathic membranoproliferative glomerulonephritis, 0/4 pauci-immune vasculitis) and 17% of control patients with renal cancer. In multivariate analysis, oligoclonal B-cells were associated with podocyte injury and the grade of glomerulosclerosis (both p = . Read More

Am J Case Rep 2020 May 27;21:e921445. Epub 2020 May 27.

Department of Nephrology, Virgin Health Hospital, Toledo, Spain.

BACKGROUND Visceral leishmaniasis (VL) is an endemic systemic disease in the Mediterranean countries, including Spain. This vector-borne infection can present with several clinical presentations, from asymptomatic to severe forms. Renal impairment is frequently described in VL but is usually mild and related to interstitial nephritis, being that glomerular involvement is rarely found. Read More

Qatar Med J 2020 5;2020(1):16. Epub 2020 May 5.

Division of Nephrology, Department of Medicine, Hamad Medical Corporation, Doha, Qatar.

Anti-glomerular basement membrane (anti-GBM) disease occurs in fewer than two cases per million population. Patients usually present with features of rapidly progressive glomerulonephritis (RPGN) with or without pulmonary involvement. Anti-GBM disease is classically diagnosed by both demonstrating GBM linear immunofluorescence staining on kidney biopsy and detecting anti-GBM antibodies in serum. Read More

Kidney Int 2020 Jun 28;97(6):1260-1274. Epub 2020 Feb 28.

National Renal Complement Therapeutics Centre, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK; Complement Therapeutics Research Group, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK. Electronic address:

Recessive mutations in diacylglycerol kinase epsilon (DGKE) display genetic pleiotropy, with pathological features reported as either thrombotic microangiopathy or membranoproliferative glomerulonephritis (MPGN), and clinical features of atypical hemolytic uremic syndrome (aHUS), nephrotic syndrome or both. Pathophysiological mechanisms and optimal management strategies have not yet been defined. In prospective and retrospective studies of aHUS referred to the United Kingdom National aHUS service and prospective studies of MPGN referred to the National Registry of Rare Kidney Diseases for MPGN we defined the incidence of DGKE aHUS as 0. Read More

Ann Hematol 2020 May 4. Epub 2020 May 4.

Department of Internal Medicine III, Jena University Hospital, Am Klinikum 1, 07747, Jena, Germany.

Iran J Kidney Dis 2020 May;14(3):184-190

Kidney Transplantation Complications Research center,Mashhad University of Medical Sciences,Mashhad,Ira.

Introduction: Knowing the national statistics of glomerular diseases will help in the management and minimizing their burden in the community. The aim of this study was to assess the overall distribution of subtypes of glomerulonephritis (GN) and the prevalence of renal diseases in a subgroup of diabetic and hypertensive patients.

Methods: This cross-sectional study was conducted on 860 patients with different subtypes of GN diagnosed by percutaneous renal biopsy and histological examination. Read More

CEN Case Rep 2020 Apr 27. Epub 2020 Apr 27.

Department of Nephrology, Saitama Medical University, 38 Morohongo, Moroyama-machi, Iruma-gun, Saitama, 350-0495, Japan.

We report here two cases of membranoproliferative glomerulonephritis that developed during treatment of rheumatoid arthritis with tocilizumab. In both cases, the initial findings were proteinuria and haematuria, followed by development of bilateral lower leg oedema. One of the patients was weakly positive for anti-nuclear antibody; both had hypocomplementaemia. Read More

New Microbiol 2019 Dec 13;43(1). Epub 2019 Dec 13.

Microbiology and Virology Unit, IRCCS San Matteo Hospital Foundation, Pavia, Italy.

Cryptococcus species is still a very common opportunistic infection in AIDS patients. However, it is increasingly responsible for disease in otherwise immunocompromised individuals, such as transplant recipients and the heterogeneous group of patients with underlying immunologic diseases, hematologic disorders and organ failure syndromes. Clinical presentation, prognosis, and outcomes are difficult to define given these varied host groups, and tailoring treatments to fit the necessities of each patient is likewise challenging. Read More

Clin Kidney J 2020 Apr 21;13(2):225-234. Epub 2019 Jun 21.

Research Laboratory, 3rd Department of Internal Medicine, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary.

Background: A novel data-driven cluster analysis identified distinct pathogenic patterns in C3-glomerulopathies and immune complex-mediated membranoproliferative glomerulonephritis. Our aim was to replicate these observations in an independent cohort and elucidate disease pathophysiology with detailed analysis of functional complement markers.

Methods: A total of 92 patients with clinical, histological, complement and genetic data were involved in the study, and hierarchical cluster analysis was done by Ward method, where four clusters were generated. Read More

Saudi Med J 2020 Apr;41(4):369-375

Department of Pediatric, King Saud University, Riyadh, Kingdom of Saudi Arabia. E-mail.

Objectives: To identify the trends in the diagnostic frequency of glomerular disease subtypes by renal biopsy in children in Saudi Arabia over the last 20 years.

Methods: In this retrospective observational study, we identified all patients aged less than 18 years for whom native kidney biopsy was performed between 1998 and 2017. The period during which biopsy was performed (1998-2004, 2005-2011, and 2012-2017) and the demographic information and their association with the prevalence of various glomerular disease subtypes were our primary outcomes. Read More

Case Rep Nephrol 2020 24;2020:2638283. Epub 2020 Feb 24.

Department of Internal Medicine, Division of Nephrology, University of California at Davis, Sacramento, CA, USA.

Nivolumab is an immune checkpoint inhibitor that targets programmed death-1 on T cells and is designed to amplify an immunologic reaction against cancer cells. However, upregulation of the immune system with checkpoint inhibition is nonspecific, and it can be associated with certain renal side effects, the best documented of which is acute tubulointerstitial nephritis. We present a unique case of a patient with acute kidney injury associated with nephrotic syndrome shortly after starting nivolumab therapy for metastatic anal carcinoma. Read More

Vet Clin Pathol 2020 Mar 22;49(1):11-16. Epub 2020 Mar 22.

Department of Veterinary Biosciences, The Ohio State University College of Veterinary Medicine, Columbus, OH, USA.

A 4-year-old male neutered domestic shorthair cat was presented to The Ohio State University College of Veterinary Medicine for a 2-month history of severe weight loss, lethargy, anemia, and bilaterally hyperechoic kidneys with loss of corticomedullary distinction as reported by the referring veterinarian. Relevant initial laboratory results included severe non-regenerative normocytic hypochromic anemia, increased blood urea nitrogen, minimally concentrated urine, proteinuria, and an increased urine protein:creatinine ratio. Cytologic evaluation of a bone marrow aspirate revealed a markedly hypocellular marrow with abundant mucinous material. Read More

BMJ Case Rep 2020 Mar 12;13(3). Epub 2020 Mar 12.

Internal Medicine, King George's Medical University, Lucknow, Uttar Pradesh, India.

Chicken pox caused by varicella zoster virus is usually a self-limiting disease causing rare life-threatening complications. Involvement of the kidneys is infrequent during the course of the illness. Literature shows rare reports of acute glomerulonephritis following varicella infection. Read More

Nephron 2020 10;144(5):228-235. Epub 2020 Mar 10.

Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.

Background: Complement component 3 glomerulopathy (C3G) is a disease diagnosed based on the predominance of C3 immunostaining in glomeruli. The popular electron microscopic subtyping of C3G into dense deposit disease and C3 glomerulonephritis (GN) is not without limitations. We aimed to study the light microscopic (LM) patterns of C3G along with their clinicopathological correlation and treatment outcome. Read More

Cureus 2020 Feb 28;12(2):e7127. Epub 2020 Feb 28.

Nephrology, Medical College of Wisconsin, Milwaukee, USA.

Infection-related glomerulonephritis (IRGN) is an immune complex-mediated glomerulonephritis (GN), often preceded by infection with subsequent recovery of renal function after the resolution of the infection. C3 deposition in the absence of immune complex deposits can be seen in patients with IRGN, but with the emergence of C3 glomerulonephritis (C3GN), the distinction is difficult as the clinical and pathological presentation may be similar. However, their treatment and clinical course vary significantly. Read More

Saudi J Kidney Dis Transpl 2020 Jan-Feb;31(1):79-89

Department of Pediatrics, Division of Pediatric Nephrology, Erciyes University Faculty of Medicine, Kayseri, Turkey.

C3 glomerulopathy (C3G) is a clinical spectrum that presents with a variety of symptoms, ranging from a mild disease with asymptomatic microhematuria and/or proteinuria to severe disease with nephritic or nephrotic syndrome and renal impairment. Herein, we aim to document the clinical and laboratory findings, response to immunosuppressive and supportive treatment and prognosis of the children with C3G. We retrospectively reviewed the medical records of patients diagnosed with membranoproliferative glomerulonephritis (MPGN). Read More

Nefrologia 2020 Feb 26. Epub 2020 Feb 26.

University Medical Centre Ljubljana, University Children's Hospital, Institute for Special Laboratory Diagnostics, Ljubljana, Slovenia; University of Ljubljana, Faculty of Medicine, Institute of Biochemistry, Ljubljana, Slovenia. Electronic address:

Background: Hereditary spherocytosis is clinically and genetically heterogeneous disorder and its clinical characteristics are spherocytosis, anaemia, jaundice and splenomegaly. The aetiology is associated to the genes encoding proteins involved in the interaction between the erythrocyte membrane and the lipid bilayer. Causative variants in βI-spectrin (SPTB) gene presenting as mild to moderately severe disease are responsible for approximately 25% cases in the USA and Europe. Read More

BMC Nephrol 2020 Feb 27;21(1):68. Epub 2020 Feb 27.

Department of Pediatrics, Kindai University Faculty of Medicine, 377-2 Ohno-higashi, Osaka-sayama, Osaka, 589-8511, Japan.

Background: IgA deficiency associated with glomerulonephritis is rare. In particular, there is no prior report regarding the association between IgA deficiency and membranoproliferative glomerulonephritis (MPGN) in children. Herein, we describe the case of a 5-year-old girl with selective IgA deficiency and MPGN. Read More

BMC Nephrol 2020 Feb 24;21(1):56. Epub 2020 Feb 24.

Nephrology Division, University of São Paulo School of Medicine, Av. Dr. Enéas de Carvalho Aguiar, 255, 7° andar, Cerqueira Cesar, São Paulo, Brazil.

Background: Sickle cell disease (SCD) is a highly prevalent genetic disease worldwide. In the natural evolution of SCD, glomerular lesions can develop, presenting histopathological patterns of segmental or focal membranoproliferative glomerulosclerosis, with or without thrombotic microangiopathy. We report two cases of acute post-infectious glomerulonephritis (APIGN), with atypical presentations, in patients with SCD. Read More

Am J Kidney Dis 2020 03;75(3):A13-A15

Department of Internal Medicine, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico. Electronic address:

Dtsch Med Wochenschr 2020 Feb 18;145(4):232-239. Epub 2020 Feb 18.

Membranoproliferative glomerulonephritis (MPGN) and glomerulopathy with dominant C3 deposits are very rare autoimmune disorders of the kidney that had been classified in its current form in 2010 due to a better understanding of the underlying pathophysiology.Today, the immune complex-associated membranoproliferative glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G) represent a disease spectrum which is heterogeneous in terms of pathophysiology and the clinical time course. Interestingly, recent research demonstrated more common pathophysiological aspects with respect to secondary causes, autoantibodies, and genetics of IC-MPGN and C3G than it had been suggested with the creation of the 2010 classification. Read More

Nephron 2020 12;144(4):195-203. Epub 2020 Feb 12.

Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Bergamo, Italy.

A membranoproliferative pattern of glomerular injury is frequently observed in patients with complement-mediated disorders, such as C3 glomerulopathies (C3G) and primary immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN). The outcomes of C3G and -IC-MPGN are poor, independently of immunosuppressive therapy. However, two 48-week treatment periods with the anti-C5 monoclonal antibody eculizumab, divided by a -12-week washout period, achieved remission of proteinuria and stabilization/improvement of the glomerular filtration rate (GFR), measured through iohexol plasma clearance, in 3 of 10 patients with biopsy-proven MPGN, nephrotic syndrome and terminal complement complex sC5b-9 plasma levels >1,000 mg/mL, at inclusion. Read More

Clin Exp Med 2020 May 11;20(2):191-197. Epub 2020 Feb 11.

National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, #305 East Zhongshan Road, Nanjing, 210093, China.

The objective of the study was to evaluate the characteristics and prognosis of 56 patients with rheumatoid arthritis (RA)-associated renal involvement by retrospective review of their renal biopsy specimens. Included in this cross-sectional study were 56 RA patients with renal involvement, in whom renal biopsy was performed to analyze the histological pattern and renal prognosis. IgA nephropathy (IgAN) was detected in 48. Read More

Clin J Am Soc Nephrol 2020 Feb 4. Epub 2020 Feb 4.

Department of Nephrology Day Hospital, Tenon Hospital Assistance Publique des Hôpitaux de Paris, Paris, France;

Kidney Int 2020 Mar 9;97(3):589-601. Epub 2019 Nov 9.

Department of Immunology, Joint Research Unit CNRS 7276, INSERM 1262, University of Limoges, French Reference Center for AL Amyloidosis, University Hospital Dupuytren, Limoges, France; Department of Nephrology, Dialysis and Renal Transplantation, University Hospital of Poitiers, French Reference Center for AL Amyloidosis, Poitiers, France.

IgG (mainly IgG3) is the most commonly involved isotype in proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID). Here we describe the first series of PGNMID with deposition of monoclonal immunoglobulin light chain only (PGNMID-light chain). This multicenter cohort of 17 patients presented with nephritic or nephrotic syndrome with underlying hematologic conditions of monoclonal gammopathy of renal significance (71%) or multiple myeloma (29%). Read More

BMC Nephrol 2020 Jan 29;21(1):27. Epub 2020 Jan 29.

Department of Diagnostic Pathology, Kagawa University, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan.

Background: Cryofibrinogenemia is a rare disorder that mainly affects the skin and occasionally the kidney. However, there are few published reports of cryofibrinogenemia-associated renal pathology. We therefore report a patient with cryofibrinogen-associated glomerulonephritis. Read More

Nat Rev Nephrol 2020 04;16(4):188

Nature Reviews Nephrology, .

Int J Mol Sci 2020 Jan 14;21(2). Epub 2020 Jan 14.

Department of Emergency and Organ Transplantation, Renal Unit, University of Bari, 70124 Bari, Italy.

In April 2012, a group of nephrologists organized a consensus conference in Cambridge (UK) on type II membranoproliferative glomerulonephritis and decided to use a new terminology, "C3 glomerulopathy" (C3 GP). Further knowledge on the complement system and on kidney biopsy contributed toward distinguishing this disease into three subgroups: dense deposit disease (DDD), C3 glomerulonephritis (C3 GN), and the CFHR5 nephropathy. The persistent presence of microhematuria with or without light or heavy proteinuria after an infection episode suggests the potential onset of C3 GP. Read More

Nephrol Ther 2020 Mar 9;16(2):124-135. Epub 2020 Jan 9.

Centre universitaire des maladies rénales, CHU de Caen, avenue de la côte de Nacre, 14033 Caen cedex 9, France; Unicaen, UFR de médecine, Normandie Université, 2, rue des Rochambelles, 14032 Caen cedex, France. Electronic address:

Hypocomplementemic urticarial vasculitis is a rare systemic vasculitis, affecting small vessels, characterised by chronicle urticaria, hypocomplementemia, and systemic manifestations. Renal involvement, whose prevalence varies between 9% and 60%, is mainly glomerular. We here report the case of a 59 years old woman presenting kidney failure, associated with chronicle urticaria and arthralgias. Read More

J Am Soc Nephrol 2020 Feb 9;31(2):365-373. Epub 2020 Jan 9.

Department of Renal Medicine, University College London, London, United Kingdom;

Background: Primary membranoproliferative GN, including complement 3 (C3) glomerulopathy, is a rare, untreatable kidney disease characterized by glomerular complement deposition. Complement gene mutations can cause familial C3 glomerulopathy, and studies have reported rare variants in complement genes in nonfamilial primary membranoproliferative GN.

Methods: We analyzed whole-genome sequence data from 165 primary membranoproliferative GN cases and 10,250 individuals without the condition (controls) as part of the National Institutes of Health Research BioResource-Rare Diseases Study. Read More

BMJ Case Rep 2020 Jan 6;13(1). Epub 2020 Jan 6.

Nephrology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.

Multicentric Castleman disease is a rare polyclonal lymphoproliferative disorder mainly associated with two renal manifestations: thrombotic microangiopathy and amyloidosis. Nevertheless, we report here a case of human herpes virus-8 negative multicentric Castleman disease with membranous proliferative glomerulonephritis and extracapillary proliferation. A patient was successfully treated with corticosteroids, anti-CD20 and cyclophosphamide therapy. Read More

J Bras Nefrol 2020 Mar 20;42(1):77-93. Epub 2019 Dec 20.

Departamento de Nefrologia, Hospital Espírito Santo de Évora, Évora, Portugal.

Glomerulopathies are one of the leading causes of end-stage renal disease. In the last years, clinical research has made significant contributions to the understanding of such conditions. Recently, rituximab (RTX) has appeared as a reasonably safe treatment. Read More

Asian J Transfus Sci 2019 Jul-Dec;13(2):136-139. Epub 2019 Dec 3.

Department of Transfusion Medicine, Indraprastha Apollo Hospital, New Delhi, India.

The human leukocyte antigen (HLA) matching plays an important role in determining the clinical outcome of renal transplantation. The development of donor specific antibodies (DSA) against HLA is associated with antibody mediated allograft tissue injury, poor outcome and rejection. The DQ-DSA develops in a denovo pattern and its unfavorable impact on renal transplantation has not yet been widely reported. Read More

Kidney Int Rep 2019 Nov 30;4(11):1555-1567. Epub 2019 Jul 30.

Department of Medicine, Division of Nephrology, University of Maryland School of Medicine, Baltimore, Maryland, USA.

Introduction: Routine C4d staining in renal transplantation has stimulated its use in kidney biopsies with glomerulonephritis (GN). Methodical description on staining patterns in the native kidney is not available.

Methods: We retrospectively evaluated C4d staining in formalin-fixed paraffin-embedded sections from 519 native kidney biopsies (bx) with and without glomerular disease. Read More

Pathol Int 2020 Jan 16;70(1):47-52. Epub 2019 Dec 16.

Pathos Tsutsumi, Aichi, Japan.

An autopsy case of chronic Chagas disease, a debilitating disorder caused by persistent infection by protozoa, Trypanosoma cruzi (Tr. cruzi), is reported. The patient was a 73-year-old Brazilian woman of Japanese descent, who had emigrated to Japan at the age of about 40 years. Read More

Indian J Med Res 2019 10;150(4):399-406

Division of Nephrology, Department of Medicine, University of Celal Bayar, Manisa, Turkey.

Background & Objectives: Rapidly progressive glomerulonephritis (RPGN) is a clinical syndrome manifested by features of nephritic syndrome and progressive loss of renal function over a short time. The objective of this study was to investigate the relationship between neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR) and prognostic factors and pathological findings of renal biopsy in RPGN.

Methods: Consecutive newly diagnosed RPGN patients who had follow up for at least six months were retrospectively analyzed. Read More

BMC Nephrol 2019 12 10;20(1):459. Epub 2019 Dec 10.

Renal Division, Department of Medicine, Peking University First Hospital, Institute of Nephrology, Peking University, Beijing, 100034, People's Republic of China.

Background: C3 glomerulonephritis (C3GN) is a rare disease caused by inherited or acquired complement alternative pathway (CAP) dysregulation, which could also be secondary to monoclonal gammopathy of undetermined significance (MGUS). Herein, we described a patient presenting with C3GN and monoclonal gammopathy, and the pathogenic association between the two diseases was further explored in vitro.

Case Presentation: A 76-year-old Chinese man presented with low serum C3 level, haematuria and nephrotic syndrome, and experienced rapid worsening of renal function over a period of 10 months. Read More

Lakartidningen 2019 Nov 29;116. Epub 2019 Nov 29.

Institutionen för Kliniska vetenskaper, Reumatologi, Lunds universitet och Reumatologkliniken, Skånes universitetssjukhus - Lund, Sweden Institutionen för Kliniska vetenskaper, Reumatologi, Lunds universitet och Reumatologkliniken, Skånes universitetssjukhus - Lund, Sweden.

Although more than 45 years have passed since hypocomplementemic urticarial vasculitis (HUVS) was first described by McDuffie and colleagues at the Mayo clinic, data on epidemiology, disease outcomes, prognosis and clinical features are scarce. Recently, we published the first epidemiological study of HUVS including data on incidence, prevalence, disease outcomes, prognosis and clinical features using data from two separate Swedish regions during a period of 16 years. The estimation of incidence and prevalence rates indicates that HUVS is rare but not always benign. Read More

Ann Hematol 2020 Feb 26;99(2):367-369. Epub 2019 Nov 26.

Centro Hospitalar de Lisboa Central, Lisbon, Portugal.

BMC Nephrol 2019 11 21;20(1):417. Epub 2019 Nov 21.

Department of Nephrology, Princess Alexandra Hospital, 199 Ipswich Rd, Wooloongabba, Brisbane, Australia.

Background: Membranoproliferative glomerulonephritis (MPGN) is an uncommon cause of end stage kidney disease (ESKD) and the clinical outcomes of patients with MPGN who commence kidney replacement therapy have not been comprehensively studied.

Methods: All adult patients with ESKD due to glomerulonephritis commencing kidney replacement therapy in Australia and New Zealand from January 1, 1996 to December 31, 2016 were reviewed. Patients with ESKD due to MPGN were compared to patients with other forms of glomerulonephritis. Read More

Semin Immunol 2019 10 9;45:101331. Epub 2019 Nov 9.

Department of Biomedical Sciences, Humanitas University, Milan, Italy; Department of Bacteriology and Immunology, Translational Immunology Research Program, University of Helsinki, Helsinki, Finland; HUSLAB, Helsinki University Central Hospital, Helsinki, Finland. Electronic address:

Glomerulonephritis (GN) refers to a group of renal diseases affecting the glomeruli due to the damage mediated by immunological mechanisms. A large proportion of the disease manifestations are caused by disturbances in the complement system. They can be due to genetic errors, autoimmunity, microbes or abnormal immunoglobulins, like modified IgA or paraproteins. Read More

Fetal Pediatr Pathol 2019 Nov 9:1-8. Epub 2019 Nov 9.

Department of Paediatrics, University of Malaya, Kuala Lumpur, Malaysia.

: Denys-Drash Syndrome (DDS) consists of a triad of pseudohermaphroditism, Wilms'tumor and nephropathy. This condition may manifest as a complete triad or in an incomplete form; with either one or a combination of the above features. The characteristic glomerular abnormality in DDS is diffuse mesangial sclerosis (DMS). Read More

Orphanet J Rare Dis 2019 11 8;14(1):247. Epub 2019 Nov 8.

Research Laboratory, 3rd Department of Internal Medicine, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Kútvölgyi St 4, Budapest, H-1125, Hungary.

Background: Acquired or genetic abnormalities of the complement alternative pathway are the primary cause of C3glomerulopathy(C3G) but may occur in immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) as well. Less is known about the presence and role of C4nephritic factor(C4NeF) which may stabilize the classical pathway C3-convertase. Our aim was to examine the presence of C4NeF and its connection with clinical features and with other pathogenic factors. Read More

Kidney Int Rep 2019 Oct 19;4(10):1387-1400. Epub 2019 Jun 19.

Centre for Inflammatory Disease, Division of Immunology and Inflammation, Department of Medicine, Imperial College London, UK.

Introduction: Therapeutic agents that target complement are increasingly available for glomerular diseases. However, the mechanisms linking glomerular complement deposition with inflammation and damage are incompletely understood. Complement factor H-related protein 5 (FHR5) interacts with complement C3 and is considered to promote activation. Read More

Clin J Am Soc Nephrol 2019 11 25;14(11):1605-1615. Epub 2019 Oct 25.

Department of Pathology and Cell Biology and

Background And Objectives: In 2012, the Systemic Lupus International Collaborating Clinics proposed that lupus nephritis, in the presence of positive ANA or anti-dsDNA antibody, is sufficient to diagnose SLE. However, this "stand-alone" kidney biopsy criterion is problematic because the ISN/RPS classification does not specifically define lupus nephritis. We investigated the combination of pathologic features with optimal sensitivity and specificity for the diagnosis of lupus nephritis. Read More

BMC Nephrol 2019 10 17;20(1):375. Epub 2019 Oct 17.

Department of Internal Medicine IV, Teikyo University School of medicine, University Hospital, Mizonokuchi, Kawasaki, Japan.

Background: TAFRO syndrome is a systemic inflammatory disorder that manifests as thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Renal dysfunction is frequently complicated with TAFRO syndrome, however, it is challenging to perform kidney biopsy in patients with TAFRO syndrome in the presence of thrombocytopenia. Renal histology in TAFRO syndrome mainly shows membranoproliferative glomerulonephritis (MPGN)-like lesions or thrombotic microangiopathy (TMA)-like glomerulopathy. Read More

Front Immunol 2019 27;10:2166. Epub 2019 Sep 27.

Department of Infection Biology, Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany.

Defective complement action is a cause of several human glomerular diseases including atypical hemolytic uremic syndrome (aHUS), anti-neutrophil cytoplasmic antibody mediated vasculitis (ANCA), C3 glomerulopathy, IgA nephropathy, immune complex membranoproliferative glomerulonephritis, ischemic reperfusion injury, lupus nephritis, membranous nephropathy, and chronic transplant mediated glomerulopathy. Here we summarize ongoing clinical trials of complement inhibitors in nine glomerular diseases and show which inhibitors are used in trials for these renal disorders (http://clinicaltrials.gov). Read More

Fundam Clin Pharmacol 2020 Apr 12;34(2):296-298. Epub 2019 Nov 12.

Department of Nephrology and Clinical Immunology, University Hospital of Tours, Tours, France.

Human parvovirus B19 has been associated with various cases of kidney injuries with different glomerular phenotypes. In immunocompromised individuals, insufficient production of neutralizing antibodies can lead to chronic PVB19 carriage and manifestations. However, PVB19 DNA has been detected in bone marrow and peripheral blood for months or years in seemingly immunocompetent individuals, despite the presence of neutralizing antibodies. Read More

Clin Mol Hepatol 2020 Jan 11;26(1):16-23. Epub 2019 Oct 11.

Department of Gastroenterology and Hepatology, University Hospital of Ioannina, School of Health Sciences, University of Ioannina, Ioannina, Greece.

Hepatitis E virus (HEV) is a significant health problem with approximately 20 million individuals infected annually. HEV infection has been associated with a wide spectrum of extrahepatic manifestations, including neurological, hematological and renal disorders. Guillain-Barré syndrome and neuralgic amyotrophy are the most frequent neurological manifestations. Read More

Liver Transpl 2019 12 31;25(12):1756-1767. Epub 2019 Oct 31.

Department of Transplant, Mayo Clinic, Jacksonville, FL.

Renal dysfunction is common in liver transplantation (LT) candidates, but differentiating between reversible and irreversible renal injury can be difficult. Kidney biopsy might be helpful in differentiating reversible from irreversible renal injury, but it is associated with significant complications. We aimed to identify pre-LT predictors of potentially reversible renal injury using histological information obtained on pre-LT renal biopsy. Read More