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J Am Soc Nephrol 2020 Jun;31(6):1282-1295

Renal Division, Peking University First Hospital, Beijing, PR China.

Background: Antiglomerular basement membrane (anti-GBM) disease is associated with HLA-DRB1*1501 (the major predisposing genetic factor in the disease), with 3 as a nephritogenic T and B cell epitope. Although the cause of disease remains unclear, the association of infections with anti-GBM disease has been long suspected.

Methods: To investigate whether microbes might activate autoreactive T and B lymphocytes molecular mimicry in anti-GBM disease, we used bioinformatic tools, including BLAST, SYFPEITHI, and ABCpred, for peptide searching and epitope prediction. Read More

Indian J Pathol Microbiol 2020 Apr-Jun;63(2):226-229

Department of Nephrology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana, India.

Introduction: Antiglomerular basement membrane disease manifests as rapidly progressive glomerulonephritis and alveolar hemorrhage. It encompasses 10-15% of crescentic glomerulonephritis and is associated with poor outcome. In this study, we have elaborated on the clinical details, morphological features, and outcome of anti-GBM glomerulonephritis. Read More

Acad Pathol 2020 Jan-Dec;7:2374289520911185. Epub 2020 Mar 30.

Department of Pathology and Laboratory Medicine, Baylor Scott & White Medical Center, Temple, TX, USA.

http://journals.sagepub.com/doi/10. Read More

Internist (Berl) 2020 Apr;61(4):416-423

Abteilung Nephrologie, Universitätsklinikum Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Deutschland.

This article presents a case of recurrent anti-GBM disease (with antibodies against the glomerular basement membrane [GBM]) in a 17-year-old patient successfully treated with rituximab. Kidney biopsy with detection of linear deposition of immunoglobulin G (IgG) along the basement membrane is the diagnostic gold standard, which should be accompanied by serological testing. However, standard assays for the detection of anti-GBM antibodies have a high rate of false-negative results. Read More

Indian J Nephrol 2020 Jan-Feb;30(1):47-49. Epub 2019 Dec 27.

Department of Nephrology, Government Stanley Medical College and Hospital, Old Jail Road, Chennai, Tamil Nadu, India.

We report a 49-year-old man with microscopic hematuria, subnephrotic proteinuria, and rapidly progressive renal failure. His biopsy had features of PhosphoLipase A2 Receptor (PLA2R) positive membranous nephropathy with circumferential cellular crescents. Further work-up revealed IgG antiGlomerular Basement Membrane (anti-GBM) antibody titer of 188 U/mL (normal <7 U/mL). Read More

Am J Nephrol 2020 10;51(2):99-107. Epub 2020 Jan 10.

Division of Renal, Department of Medicine, Peking University First Hospital, Institute of Nephrology, Peking University, Key Laboratory of Renal Disease, Ministry of Health of China, Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, China.

Background And Aim: Primary membranous nephropathy (pMN) is the most common cause of nephrotic syndrome in adults. Recent studies suggested that immunization of DBA/1 mice with the main antigen of antiglomerular basement membrane disease (GBM) disease, α3(IV)NC1, could lead to MN lesions. This study aimed to explore the pathogenic epitopes for mouse MN. Read More

Ren Fail 2020 Nov;42(1):48-53

Renal Division, Department of Medicine, Peking University First Hospital, Beijing, PR. China.

Autoantibodies to the α3 chain noncollagen 1 domain of type IV collagen (α3(IV)NC1) are a serological hallmark in the diagnosis of anti-glomerular basement membrane (GBM) disease. The objective of our study was to compare the performance of anti-glomerular basement membrane (GBM) antibody detection by chemiluminescence immunoassay (CIA) and by enzyme-linked immunosorbent assays (ELISAs). Sera from outpatients who were suspected to have anti-GBM disease and 31 patients with biopsy-proven anti-GBM disease were collected. Read More

Clin Nephrol 2020 Mar;93(3):152-157

Introduction: Patients without detectable serum antiglomerular basement membrane (GBM) antibodies but with GBM staining for immunoglobulins (Ig), absence of a crescentic phenotype, mild renal insufficiency, and absence of pulmonary hemorrhage have atypical anti-GBM diseases. We report the case of a 64-year-old man with slowly progressive glomerulonephritis.

Case History: A 64-year-old Peruvian man presented with persistent microscopic hematuria, proteinuria of 2. Read More

N Engl J Med 2019 Nov;381(19):1854-1860

From the Veterans Affairs (VA) Ann Arbor Medical Center (C.S., S.S., P.G.) and the University of Michigan Medical School (C.S., S.S., E.A.F., P.G.) - both in Ann Arbor; and the University of California, San Francisco, School of Medicine and the San Francisco VA Medical Center - both in San Francisco (G.D.).

Medicine (Baltimore) 2019 Nov;98(44):e17801

Department of Nephrology & Hypertension, Dokkyo Medical University, Mibu, Tochigi.

Rationale: Anti-glomerular basement membrane (GBM) disease is a T cell-mediated disease that has a poor prognosis with conventional therapy. We tested rituximab as a primary therapy to reduce anti-GBM antibody produced by B cells.

Patient Concerns: A 53-year old woman with complaints of a fever, headache and abdominal discomfort showed renal failure with elevated anti-GBM antibody, and renal biopsy revealed crescentic necrotizing glomerulonephritis with linear immunoglobulin G (IgG) 1 deposition along GBM. Read More

Indian J Nephrol 2019 Sep-Oct;29(5):375-377

Department of Histopathology, Apollo Hospitals, Hyderabad, Telangana, India.

Saudi J Kidney Dis Transpl 2019 Jul-Aug;30(4):953-959

Department of Nephrology-Hemodialysis and Kidney Ttransplantation, University Hospital Mohammed VI, Marrakech, Morocco.

The combination of Goodpasture's disease and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is not exceptional. However, to the best of our knowledge, their association with multiple myeloma (MM) with kidney involvemen thas not been described. We report probably the first case of double-seropositive vasculitis for antiglomerular basement membrane (GBM) antibodies and ANCA associated with MM with renal involvement. Read More

BMJ Case Rep 2019 Aug 1;12(8). Epub 2019 Aug 1.

Department of Medicine, Northwestern Memorial Hospital, Chicago, Illinois, USA.

A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linear immunofluorescence of the basement membrane, and electron microscopy which exhibited thickening of the glomerular basement membrane. Incidentally, the patient was discovered to have a latent hepatitis B infection, which complicated immunosuppressive therapy. Read More

Rev Med Chil 2019 Mar;147(3):390-394

Departamento de Nefrología, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.

Goodpasture Syndrome is described as a single episode disease entity. It is diagnosed with the demonstration of antiglomerular basement (anti-GBM) antibodies in plasma or renal tissue. Although the recurrence of anti-GBM disease is rare, it has been reported in up to 3% of cases. Read More

J Am Soc Nephrol 2019 08 23;30(8):1365-1374. Epub 2019 Jul 23.

Department of Medicine, Centre for Inflammatory Diseases, Monash University, Clayton, Victoria, Australia; and Departments of.

Background: Myeloperoxidase (MPO)-ANCA-associated GN is a significant cause of renal failure. Manipulating autoimmunity by inducing regulatory T cells is potentially a more specific and safer therapeutic option than conventional immunosuppression.

Methods: To generate MPO-specific regulatory T cells, we used a modified protein-conjugating compound, 1-ethyl-3-(3'dimethylaminopropyl)-carbodiimide (ECDI), to couple the immunodominant MPO peptide (MPO) or a control ovalbumin peptide (OVA) to splenocytes and induced apoptosis in the conjugated cells. Read More

Case Rep Med 2019 24;2019:2975131. Epub 2019 Apr 24.

Department of Graduate Medical Education, WellStar Kennestone Regional Medical Center, Marietta, GA, USA.

Goodpasture syndrome is a rare autoimmune disease comprising antiglomerular basement membrane (anti-GBM) crescentic glomerulonephritis and pulmonary capillaritis with circulating anti-GBM antibodies. Rarely, antibody-negative cases have been described. We report a young, African American adult woman admitted with flank pain and hematuria with laboratory testing and kidney biopsy demonstrating anti-GBM crescentic glomerulonephritis with elevated anti-GBM antibody levels. Read More

Am J Physiol Renal Physiol 2019 07 29;317(1):F152-F162. Epub 2019 May 29.

Department of Medicine, McMaster University , Hamilton, Ontario , Canada.

Glomerulonephritis (GN) is a common cause of end-stage kidney disease and is characterized by glomerular inflammation, hematuria, proteinuria, and progressive renal dysfunction. Transforming growth factor (TGF)-β is involved in glomerulosclerosis and interstitial fibrosis. TGF-β activates multiple signaling pathways, including the canonical SMAD pathway. Read More

Exp Mol Pathol 2019 04 25;107:165-170. Epub 2019 Feb 25.

Department of Medical Laboratory Science, Faculty of Health Sciences, Hokkaido University, Sapporo, Japan. Electronic address:

The major epitopes recognized by autoantibodies in anti-glomerular basement membrane (GBM) disease are found in the α3-subunit non-collagenous domain of type IV collagen [α3(IV)NC1], which is present in the glomerular and alveolar basement membranes. These epitopes are structurally cryptic, owing to the hexamer formation of the non-collagenous domain of α3, α4, and α5 subunits and are expressed by the dissociation of the hexamer. Anti-GBM disease usually manifests as a single attack (SA), and we rarely see patients who repeatedly relapse. Read More

Medicine (Baltimore) 2019 Feb;98(7):e14557

Department of Nephrology.

Rationale: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and anti-glomerular basement membrane (GBM) antibody disease are both rare autoimmune diseases. Monoclonal gammopathy of undetermined significance (MGUS) is one of the most common causes of plasma cell dyscrasias (PCD). The three entities can cause renal lesions via different mechanisms and, however, they have not been reported in a single patient with renal lesion. Read More

BMC Nephrol 2019 01 31;20(1):29. Epub 2019 Jan 31.

Department of Pathology and Laboratory Medicine, Cumming School of Medicine, University of Calgary, Foothills Medical Center, 1403 29 St NW, Calgary, AB, T2N 2T9, Canada.

Background: Anti-glomerular basement membrane (anti-GBM) disease is characterized by circulating IgG glomerular basement membrane antibodies and is clinically expressed as a rapidly progressive crescentic glomerulonephritis (GN), with 30-60% of patients also developing pulmonary hemorrhage. Classically, the renal biopsy shows glomerular crescent formation, bright linear staining of glomerular basement membranes (GBM) for IgG on direct immunofluorescence (IF), and the serologic presence of circulating anti-GBM antibodies. Recently, patients with linear IgG IF staining, undetectable circulating anti-GBM antibodies and glomerular changes atypical for anti-GBM disease have been described as "atypical anti-GBM disease", with a distinctly more benign clinical course than typical anti-GBM disease. Read More

BMC Nephrol 2019 01 25;20(1):25. Epub 2019 Jan 25.

Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan.

Background: Anti-glomerular basement membrane (GBM) glomerulonephritis does not usually coexist with another glomerulonephritis such as IgA nephropathy. We present a rare case having a combination of these two diseases, and furthermore, histological evaluation could be performed before and after the development of anti-GBM glomerulonephritis over a period of only10 months.

Case Presentation: A 66-year-old woman was admitted with complaints of microscopic hematuria and mild proteinuria for the past 3 years. Read More

Clin Nephrol 2019 Feb;91(2):101-106

Background: Antiglomerular basement membrane (anti-GBM) antibody disease is a rare condition causing pulmonary hemorrhage and necrotizing glomerulonephritis (pulmonary renal syndrome).

Case: We report a 30-year-old woman who presented with life-threatening pulmonary hemorrhage and an active urinary sediment, with normal glomerular filtration rate in the 13 week of pregnancy. Anti-GBM antibodies in serum were negative, but perinuclear antineutrophil cytoplasmatic antibodies (p-ANCA) were detected. Read More

Int J Nephrol Renovasc Dis 2018 16;11:267-270. Epub 2018 Oct 16.

Division of Nephrology, Department of Pediatrics, University of Minnesota Medical School, Minneapolis, MN 55454, USA,

Alport syndrome is an inherited disorder of basement membrane collagen IV that frequently results in end-stage renal disease. Patients with Alport syndrome who undergo renal transplantation have generally excellent outcomes. Posttransplant antiglomerular basement membrane nephritis is a rare complication of renal transplantation for Alport syndrome. Read More

Semin Respir Crit Care Med 2018 08 7;39(4):504-510. Epub 2018 Nov 7.

Department of Internal Medicine, Hôpital Cochin, Université Paris, Paris, France.

Pulmonary vasculitis can be heterogeneous and have some pathophysiological, clinical, and biological specificities that might influence treatment choices. Main vasculitides associated with pulmonary vasculitis include antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, immune complex small vessel vasculitis, and antiglomerular basement membrane disease. Patient characteristics and association of lung involvement with other manifestations affecting disease severity may also influence treatment strategy. Read More

Semin Respir Crit Care Med 2018 08 7;39(4):494-503. Epub 2018 Nov 7.

Department of Medicine, Centre for Inflammatory Disease, Imperial College London, London, United Kingdom.

Antiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of cases may present with pulmonary disease in isolation. Read More

BMC Nephrol 2018 10 22;19(1):286. Epub 2018 Oct 22.

Ziekenhuisgroep Twente, Department of Nephrology, Almelo, The Netherlands.

Background: Hantavirus infection is an uncommon cause of acute renal failure with massive proteinuria. Serology tests to support a presumptive diagnosis usually take a few days. During the initial work-up, autoimmune causes including anti-glomerular basement membrane (GBM) glomerulonephritis need to be excluded, because these require urgent therapy. Read More

Medicina (Kaunas) 2018 Apr 17;54(2). Epub 2018 Apr 17.

Centre of Nephrology, Vilnius University Hospital Santaros Klinikos, LT⁻08661 Vilnius, Lithuania.

Recently proposed histopathological classification may predict patient outcome in pauci-immune glomerulonephritis. This study sought to prove that the prognostic effect could be extended to all types of rapidly progressive glomerulonephritis. Retrospective analysis of patients diagnosed with rapidly progressive glomerulonephritis between April 1999 and August 2015 was performed. Read More

J Clin Pathol 2019 Jan 12;72(1):31-37. Epub 2018 Oct 12.

National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China

Aim: To explore the clinicopathological characteristics of patients with anti-GBM antibody-negative anti-GBM disease.

Methods: The clinical and renal pathological findings were retrospectively studied in 19 patients. All patients met the following inclusion criteria: linear GBM IgG deposition on immunofluorescence(IF); and lack of serum anti-GBM antibodies by ELISA and indirect immunofluorescence assay. Read More

Ann Hematol 2019 Apr 9;98(4):1045-1046. Epub 2018 Oct 9.

Pathology Department, Facultad de Medicina, Pontificia Universidad Católica, Santiago de Chile, Chile.

BMC Nephrol 2018 09 12;19(1):228. Epub 2018 Sep 12.

UCL Centre for Nephrology, Royal Free Hospital, London, NW3 2PF, UK.

Background: Anti-glomerular basement membrane (GBM) antibodies are highly specific for Goodpasture's or anti-GBM disease, in which they are generally directed against the non-collagenous (NC1) domain of the alpha 3 chain of type IV collagen(α3(IV)), and less commonly, toward the α 4(IV) or α 5(IV) chains, which form a triple helical structure in GBM and alveolar basement membrane (ABM). Alterations in the hexameric structure of the NC1 (α3 (IV)), allows novel epitopes to be exposed and an immune response to develop, with subsequent linear antibody deposition along the GBM, leading to a crescentic glomerulonephritis. Positive anti-GBM antibodies are assumed to be pathogenic and capable of binding GBM in vivo, especially in the context of rapidly progressive glomerulonephritis. Read More

Clin Exp Rheumatol 2019 Mar-Apr;37(2):279-285. Epub 2018 Aug 29.

Division of Nephrology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.

Objectives: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder and is characterised by elevated serum IgG4 concentrations and dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells. IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common manifestation of IgG4-related kidney disease (IgG4-RKD). We report four cases of kidney injury with concurrent IgG4-TIN and crescentic glomerulonephritis confirmed by renal pathology. Read More

Clin Case Rep 2018 Aug 1;6(8):1647-1648. Epub 2018 Jul 1.

Division of Pathology, Immunology and Laboratory Medicine University of Florida Gainesville FL USA.

Antiglomerular basement membrane (GBM) antibody disease is a vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of autoantibodies. It can be both life- and organ-threatening and delayed diagnosis may be detrimental. Alveolar hemorrhage and microscopic hematuria should be excluded in anybody who is suspected of this disease. Read More

BMJ Case Rep 2018 Jul 24;2018. Epub 2018 Jul 24.

Nephrology, Johns Hopkins University, Baltimore, Maryland, USA.

Approximately one in four patients (23.3%) with inflammatory myositis including dermatomyositis can require evaluation for acute kidney injury. The main cause of kidney injury is acute tubular necrosis from medications or myoglobinuria, though clinicians should be aware of a wide variety of possible aetiologies. Read More

Lung Cancer 2018 08 19;122:22-24. Epub 2018 May 19.

Department of Internal Medicine, Kochi Red Cross Hospital, Kochi, 2-13-51 Shin-honmachi, Kochi, 780-8562, Japan.

Nivolumab, an anti-programmed death-1 immune checkpoint inhibitor (ICI), is now widely used to treat numerous cancers. Although most adverse effects related to ICIs are controllable, fulminant immune-related adverse events can occur. A 74-year-old patient with non-small-cell lung cancer was treated with nivolumab as a second-line treatment. Read More

Kidney Int Rep 2018 Jul 23;3(4):991-996. Epub 2018 Mar 23.

Department of Pathology, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada.

J Coll Physicians Surg Pak 2018 Jul;28(7):518-522

Department of Immunology, University of Health Sciences (UHS), Lahore.

Objective: To assess activation of immune system in rheumatic heart disease (RHD) patients in the form of AECA, ACL and anti GBM antibodies.

Study Design: Descriptive, observational study.

Place And Duration Of Study: Department of Immunology, University of Health Sciences (UHS), Lahore, and Outpatient Department, Punjab Institute of Cardiology, from February 2015 to January 2016. Read More

BMJ Case Rep 2018 May 2;2018. Epub 2018 May 2.

Pathology Department, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal.

The antiglomerular basement membrane (anti-GBM) antibody disease is marked by the presence of specific antibodies against the non-collagenous domain of the type IV collagen's α3 chain. We describe a case of a 24-year-old Caucasian man, who may have had an atypical presentation of anti-GBM (slow progressive renal insufficiency, massive proteinuria and no detectable circulating anti-GBM antibody). The patient was treated with steroids and cyclophosphamide. Read More

Am J Physiol Renal Physiol 2018 10 2;315(4):F759-F768. Epub 2018 May 2.

Department of Medicine, Division of Renal Diseases and Hypertension.

Osteopontin (OPN) is a pro-and anti-inflammatory molecule that simultaneously attenuates oxidative stress. Both inflammation and oxidative stress play a role in the pathogenesis of glomerulonephritis and in the progression of kidney injury. Importantly, OPN is highly induced in nephritic kidneys. Read More

Eur J Immunol 2018 06 2;48(6):1082-1084. Epub 2018 May 2.

Laboratoire d'Immunologie, Pôle de Biologie, Centre Hospitalier Universitaire Grenoble Alpes, Grenoble, Cedex 9, France.

Detection of circulating anti-GBM antibodies has a key role for the diagnosis of Goodpasture syndrome but immunoassays using purified or recombinant alpha3(IV)NC1 as antigen do not recognize all anti-GBM antibodies. We show that anti-GBM antibodies directed against epitopes in their native conformation or cryptic epitopes are detected by indirect immunofluorescence. Read More

Zhonghua Nei Ke Za Zhi 2018 Mar;57(3):229-232

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

A 56-year-old female was admitted to the Department of Rheumatology, Peking Union Medical College Hospital with complaint of recurrent fever and acute lumbar pain. Fever was complicated with malaise, cough and occasional blood-streaked sputum. Lab tests showed elevated white blood cell count, increased serum creatinine, erythrocyte sedimentation rate and C-reactive protein. Read More

Clin Nephrol 2018 Jun;89(6):469-473

We report a case of glomerular basement membrane crescentic glomerulonephritis and multicentric Castleman disease-associated interstitial nephritis in a patient with human immunodeficiency virus (HIV) infection. The patient received corticosteroids, cyclophosphamide, and plasmapheresis, and within 3 weeks, there was worsening thrombocytopenia, anemia, and renal function requiring initiation of hemodialysis. He then received 8 weekly doses of rituximab, and there was steady improvement in renal function, such that he stopped dialysis within 6 weeks and has remained in disease remission at 1-year follow-up. Read More

Nephrol Ther 2018 Apr 28;14(2):105-108. Epub 2017 Dec 28.

Service de médecine interne, centre hospitalier Saint-Joseph-Saint-Luc, 20, quai Claude-Bernard, 69007 Lyon, France.

Goodpasture's syndrome is a triad of anti-glomerular basement membrane (anti-GBM) circulating antibodies, glomerulonephritis and pulmonary hemorrhage. We reported a 65-year-old woman with headaches, asthenia and weight loss. Giant cell arteritis was confirmed by temporal artery biopsy. Read More

Kidney Int 2018 03 21;93(3):626-642. Epub 2017 Dec 21.

Department of Nephrology, RIMLS, RIHS, Radboud University Medical Center, Nijmegen, The Netherlands. Electronic address:

A key feature of glomerular diseases such as crescentic glomerulonephritis and focal segmental glomerulosclerosis is the activation, migration and proliferation of parietal epithelial cells. CD44-positive activated parietal epithelial cells have been identified in proliferative cellular lesions in glomerular disease. However, it remains unknown whether CD44-positive parietal epithelial cells contribute to the pathogenesis of scarring glomerular diseases. Read More

Saudi J Kidney Dis Transpl 2017 Nov-Dec;28(6):1404-1407

Department of Nephrology, Government Stanley Hospital, Chennai, Tamil Nadu, India.

Anti-glomerular basement membrane (anti-GBM) disease is a systemic autoimmune disorder characterized by circulating IgG antibodies (rarely IgA and IgM) to the carboxyterminal, noncollagenous 1 (NC1) domain of type IV collagen of GBM also known as Goodpasture antigen. Patients typically present with rapidly progressive glomerulonephritis (RPGN) and pulmonary hemorrhage in the presence of which it is referred to as Goodpasture's disease. Anti-GBM disease has been reported to coexist with pauci-immune antineutrophil cytoplasmic autoantibody-positive glomerulonephritis and membranous glomerulopathy. Read More

Saudi J Kidney Dis Transpl 2017 Nov-Dec;28(6):1397-1403

Department of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.

Antiglomerular basement membrane (anti-GBM) disease is an uncommon autoimmune disease characterized by the presence of IgG autoantibodies targeting the alpha-3 chain of type IV collagen. Some of the atypical forms of the disease have been described. Herein, we describe a case of atypical anti-GBM in a 27-year-old Saudi male who presented with lower limb edema, gross hematuria, elevated serum creatinine concentration, and nephrotic-range proteinuria. Read More

Kidney Int 2018 04 11;93(4):1008-1013. Epub 2017 Dec 11.

Science for Life Laboratory, Department of Applied Physics, Royal Institute of Technology, Solna, Sweden; Science for Life Laboratory, Department of Women's and Children's Health, Karolinska Institutet, Solna, Sweden. Electronic address:

The glomerular filtration barrier, has historically only been spatially resolved using electron microscopy due to the nanometer-scale dimensions of these structures. Recently, it was shown that the nanoscale distribution of proteins in the slit diaphragm can be resolved by fluorescence based stimulated emission depletion microscopy, in combination with optical clearing. Fluorescence microscopy has advantages over electron microscopy in terms of multiplex imaging of different epitopes, and also the amount of volumetric data that can be extracted from thicker samples. Read More

Clin J Am Soc Nephrol 2018 01 21;13(1):63-72. Epub 2017 Nov 21.

Departments of Pathology and.

Background And Objectives: Large studies on long-term kidney outcome in patients with anti-glomerular basement membrane (anti-GBM) GN are lacking. This study aimed to identify clinical and histopathologic parameters that predict kidney outcome in these patients.

Design, Setting, Participants, & Measurements: This retrospective analysis included a total of 123 patients with anti-GBM GN between 1986 and 2015 from six centers worldwide. Read More

Case Rep Nephrol 2017 11;2017:7143649. Epub 2017 Oct 11.

Department of Internal Medicine, Teikyo University School of Medicine, Itabashi-ku, Tokyo, Japan.

A 26-year-old man highly suspected of having antiglomerular basement membrane (GBM) disease was treated with corticosteroid pulse therapy 9 days after initial infection-like symptoms with high procalcitonin value. The patient required hemodialysis the next day of the treatment due to oliguria. In addition to corticosteroid therapy, plasmapheresis was introduced and the patient could discontinue hemodialysis 43 days after the treatment. Read More

Pediatrics 2017 Nov;140(5)

Department of Pediatric Nephrology, MARHEA - Necker Hospital - APHP, Imagine Institute, Paris Descartes University, Paris, France; and

Antiglomerular basement membrane glomerulonephritis (anti-GBM GN) is a rare autoimmune disease that is characterized by rapidly progressive glomerulonephritis that may be associated with pulmonary hemorrhage. Anti-GBM GN is caused by autoantibodies (classically type G immunoglobulin) directed against the α3 subunit of type IV collagen. Without any appropriate treatment, the disease is generally fulminant, and patient and kidney survival is poor. Read More

Mol Med Rep 2017 Dec 4;16(6):9086-9094. Epub 2017 Oct 4.

Division of Nephrology, Department of Internal Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430030, P.R. China.

Anti‑glomerular basement membrane glomerulonephritis (anti‑GBM GN) is an autoimmune disease that leads to severe and rapidly progressive renal injury. Inhibition of DNA‑binding factor 3 (ID3) serves a key role in autoimmune diseases, such as asthma and Sjögren's syndrome, and in experimental allergic encephalitis models. However, the role of ID3 in the progression of anti‑GBM GN remains unknown. Read More