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Pharmacol Res Perspect 2022 06;10(3):e00973

Research Division, Chugai Pharmaceutical Co., Ltd., Gotemba, Shizuoka, Japan.

Hyperphosphatemia associated with chronic kidney disease (CKD) not only dysregulates mineral metabolism and bone diseases, but also strongly contributes to the progression of kidney disease itself. We have identified a novel drug for hyperphosphatemia, EOS789, that interacts with several sodium-dependent phosphate transporters (NaPi-IIb, PiT-1, and PiT-2) known to contribute to intestinal phosphate absorption. In this study, we investigated whether EOS789 could ameliorate kidney disease progression in glomerulonephritis rats. Read More

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

Sci Rep 2022 May 17;12(1):8234. Epub 2022 May 17.

University of Melbourne Department of Medicine (Melbourne Health), Parkville, VIC, 3076, Australia.

Retinal drusen are characteristic of macular degeneration and complement activation, but also occur in C3, lupus and IgA nephropathy. This cross-sectional observational study compared drusen counts in different forms of glomerulonephritis. Consecutive individuals with glomerulonephritis attending a general renal or transplant clinic underwent retinal imaging with a non-mydriatic camera. Read More

J Am Soc Nephrol 2022 04 8;33(4):829-838. Epub 2022 Mar 8.

Department of Nephrology and Department of Health, Medicine and Caring Sciences, Linköping University, Linköping, Sweden

Background: The prognosis for kidney survival is poor in patients presenting with circulating anti-glomerular basement membrane (GBM) antibodies and severe kidney injury. It is unknown if treatment with an endopeptidase that cleaves circulating and kidney bound IgG can alter the prognosis.

Methods: An investigator-driven phase 2a one-arm study (EudraCT 2016-004082-39) was performed in 17 hospitals in five European countries. Read More

Kidney Int Rep 2022 Mar 17;7(3):658-659. Epub 2022 Jan 17.

Division of Nephropathology, Department of Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.

Clin Exp Rheumatol 2022 May 4;40(4):691-704. Epub 2022 Feb 4.

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

Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is sometimes complicated by anti-glomerular basement membrane (GBM) disease. Proteases, including elastase, released from neutrophils activated by ANCA are implicated in the pathogenesis of AAV. Epitopes of anti-GBM antibody exist in the α3-subunit non-collagenous (NC1) domain of collagen type IV [Col (IV)]. Read More

Beyoglu Eye J 2021 11;6(1):66-69. Epub 2021 Feb 11.

Department of Ophthalmology at General Hospital "Prim. Dr. Abdulah Nakas", Sarajevo, Bosnia and Herzegovina.

Alport syndrome is a rare basement membrane disorder that may include ocular manifestations: dot-and-fleck retinopathy, anterior lenticonus, posterior polymorphous corneal dystrophy, or temporal macular thinning. It is primarily an X-linked inheritance condition (85%). This case report describes a 51-year-old male patient with Alport syndrome who was diagnosed due to ocular manifestations that were subsequently linked with a history of renal failure and bilateral sensorineural hearing loss. Read More

Nephrology (Carlton) 2022 Mar 6;27(3):290-292. Epub 2021 Dec 6.

Department of Medicine, St John of God Subiaco Hospital, Subiaco, Australia.

Dtsch Med Wochenschr 2021 11 26;146(23):1552-1558. Epub 2021 Nov 26.

Medizinische Klinik und Poliklinik IV, Nephrologisches Zentrum, LMU Klinikum, Campus Großhadern.

In 2021 new KDIGO (Kidney Disease: Improving Global Outcomes) guidelines for the management of glomerular diseases were published.For ANCA-associated glomerulonephritis the new recommendations comprise a more rapid steroid taper during induction treatment with cyclophosphamide or rituximab, the advice against routine use of plasma exchange, the choice of drug for and duration of maintenance treatment in accordance with predictors of relapse. A kidney transplant should be performed after at least 6 months of remission irrespective of the ANCA titer in ANCA-associated disease, and 6 months after absence of anti-GBM-antibodies in anti-GBM-disease. Read More

Int J Nephrol Renovasc Dis 2021 1;14:393-398. Epub 2021 Nov 1.

Department of Nephrology, Government Rajaji Hospital, Madurai Medical College, Madurai, Tamil Nadu, India.

Anti-glomerular basement membrane disease (anti-GBM) affects mainly kidneys and lungs. It requires aggressive immunosuppressive treatment. Since the emergence of the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), there has been an increased number of new cases of anti-GBM disease presenting as rapidly progressive glomerulonephritis (RPGN). Read More

Kidney Int Rep 2021 Dec 13;6(12):3090-3091. Epub 2021 Oct 13.

Nephropathology, Renopath, Center for Renal and Urological Pathology, Chennai, India.

Pan Afr Med J 2021 16;39:243. Epub 2021 Aug 16.

Department of Nephrology, Dialysis and Kidney Transplantation, La Rabta Hospital, Tunis, Tunisia.

Anti-glomerular basement membrane (anti-GBM) disease was usually described as a small vessel vasculitis presenting with acute kidney injury, haematuria and non-nephrotic proteinuria. We report a case of anti-GBM disease revealed by an intense nephrotic syndrome. The urinary protein level was 12g/day. Read More

CEN Case Rep 2022 May 15;11(2):166-170. Epub 2021 Sep 15.

Department of Nephrology, Hitachi General Hospital, 2-1-1 Jonan-cho, Hitachi, Ibaraki, 317-0077, Japan.

A case of newly developed anti-glomerular basement membrane (GBM) glomerulonephritis (GN) following centipede bites and COVID-19 vaccination is presented. A 70-year-old woman presented for investigation of mild fever, generalized fatigue, and macroscopic hematuria with no past history of renal disease. One year earlier, she had been bitten by a centipede. Read More

Nephrology (Carlton) 2022 03 8;27(3):288-289. Epub 2021 Sep 8.

Division of Nephrology, Department of Internal Medicine, Seoul St. Mary's Hospital, Seoul, South Korea.

Case Rep Nephrol 2021 17;2021:2586693. Epub 2021 Jul 17.

Department of Pediatric Nephrology, The Permanente Medical Group, Roseville, CA, USA.

Classic antiglomerular basement membrane (anti-GBM) disease is an exceedingly rare but extremely aggressive form of glomerulonephritis, typically caused by autoantibodies directed against cryptic, conformational epitopes within the noncollagenous domain of the type IV collagen alpha-3 subunit. Pathologic diagnosis is established by the presence of strong, diffuse, linear staining for immunoglobulin on immunofluorescence microscopy. Recently, patients with atypical clinical and pathologic findings of anti-GBM disease have been described. Read More

BMJ Case Rep 2021 Jul 13;14(7). Epub 2021 Jul 13.

Department of Renal Medicine, Cork University Hospital, Cork, Ireland

We describe antiglomerular basement membrane (anti-GBM) disease with rapidly progressive glomerulonephritis and concurrent parainfluenza pneumonia. Circulating anti-GBM antibodies were barely detectable and disappeared rapidly following corticosteroids, cyclophosphamide and plasma exchange. Kidney biopsy demonstrated strong linear GBM staining for IgG and IgG4 and unusually prominent endocapillary hypercellularity, suggesting 'atypical anti-GBM disease', although glomerular necrosis and crescents were also seen. Read More

Vnitr Lek 2021 ;67(E-3):24-27

We report on a case of a 40-year-old female who presented with two respiratory arrests occurring a few months apart. The investigations performed in our hospital during admission showed diffuse alveolar haemorrhage of unknown aetiology. Once the correct diagnosis of systemic lupus erythematosus was made, adequate treatment was initiated. Read More

Scand J Rheumatol 2022 05 25;51(3):205-213. Epub 2021 Jun 25.

Department of Nephrology, European Hospital Georges Pompidou, Assistance Publique-Hôpitaux de Paris (APHP), University of Paris, Paris, France.

Objective: Double-positive patients (DPPs), combining serum and/or histological findings for glomerular basement membrane (GBM) disease and anti-neutrophil cytoplasmic antibodies (ANCAs), are rare and poorly described. This study aimed to compare characteristics between DPPs and ANCA-associated vasculitis (AAV) patients with severe renal involvement.

Method: This retrospective multicentre study compared 33 DPPs and 45 AAV patients with severe renal involvement (serum creatinine > 300 μmol/L), all with biopsy-proven nephropathy. Read More

Saudi J Kidney Dis Transpl 2021 Jan-Feb;32(1):240-244

Department of Medicine, King Abdulaziz Medical City, Ministry of the National Guard- Health Affairs, Riyadh, Saudi Arabia.

Double-positive disease, defined by double-seropositivity for serum anti-glomerular basement membrane (GBM) antibodies and anti-neutrophil cytoplasmic antibodies (ANCA) is a rare cause of pulmonary-renal syndrome. Here, we present an exceptional course of a 20-year-old male with seropositivity for anti-myeloperoxidase anti-neutrophil cytoplasmic antibodies and anti-GBM antibody, who presented first with renal impairment due to focal necrotizing crescentic glomerulonephritis. After receiving treatment, he presented two years later with a relapse manifesting with diffuse alveolar hemorrhage and multiple splenic infarcts. Read More

Ren Fail 2021 Dec;43(1):774-778

Department of Nephrology, China-Japan Friendship Hospital, Beijing, People's Republic of China.

Background: Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune condition responsible for rapidly progressive glomerulonephritis. This disease is usually mediated by IgG autoantibodies against the noncollagenous domain of the α3(IV) collagen chain. In rare cases, IgA or IgM anti-GBM antibodies are involved. Read More

J Am Soc Nephrol 2021 08 23;32(8):1887-1897. Epub 2021 Apr 23.

Department of Microbiology, Immunology and Physiology, Meharry Medical College, Nashville, Tennessee

Background: Antiglomerular basement membrane (anti-GBM) disease is characterized by GN and often pulmonary hemorrhage, mediated by autoantibodies that typically recognize cryptic epitopes within α345(IV) collagen-a major component of the glomerular and alveolar basement membranes. Laminin-521 is another major GBM component and a proven target of pathogenic antibodies mediating GN in animal models. Whether laminin-521 is a target of autoimmunity in human anti-GBM disease is not yet known. Read More

Case Rep Nephrol 2021 17;2021:5539205. Epub 2021 Mar 17.

Department of Nephrology, Marshfield Clinic Health System, Marshfield, WI, USA.

. Acute kidney injury (AKI) requiring dialysis during pregnancy is uncommon. We present a case of a young female diagnosed with antiglomerular basement membrane (anti-GBM) disease during pregnancy. Read More

BMJ Case Rep 2021 Apr 1;14(4). Epub 2021 Apr 1.

Department of Nephrology, NHS Dumfries and Galloway, Dumfries, UK

An 18-year-old woman was treated for acute kidney injury (AKI) secondary to antiglomerular basement membrane (GBM) disease with prednisolone, cyclophosphamide and plasma exchange. She also had epistaxis at initial presentation with no other organ involvement and achieved good recovery of her kidney function. Two weeks after completing induction treatment, she re-presented with further AKI and pulmonary haemorrhage. Read More

J Clin Apher 2021 Jun 25;36(3):505-510. Epub 2021 Feb 25.

Children's Hospital Colorado, Aurora, Colorado, USA.

Anti-glomerular basement membrane (GBM) disease is a rarely described entity in the pediatric population, especially in those less than 3 years old. Even rarer, is double seropositive disease, consisting of anti-GBM antibody plus anti-neutrophil cytoplasmic antibodies. Both single and double antibody positive diseases are characterized by rapidly progressive glomerulonephritis, often without pulmonary involvement in the pediatric population. Read More

BMC Nephrol 2021 02 5;22(1):53. Epub 2021 Feb 5.

Kidney Research Institute, Division of Nephrology, West China Hospital, Sichuan University, Chengdu, 610041, China.

Background: Anti-glomerular basement membrane (anti-GBM) disease is an organ-specific autoimmune disease that involves the lung and kidneys and leads to rapid glomerulonephritis progression, with or without diffuse alveolar hemorrhage, and even respiratory failure. Classic cases of anti-GBM disease are diagnosed based on the presence of the anti-GBM antibody in serum samples and kidney or lung biopsy tissue samples. However, atypical cases of anti-GBM disease are also seen in clinical practice. Read More

CEN Case Rep 2021 08 18;10(3):348-353. Epub 2021 Jan 18.

Department of Nephrology, Asahikawa Red Cross Hospital, 1jo 1 chome, Akebono, Asahikawa, Hokkaido, Japan.

Atypical anti-glomerular basement membrane (GBM) disease, which is characterized by low levels of or negativity for anti-GBM antibodies in circulation but positivity in the kidney, has been recognized in this decade. However, a therapeutic strategy has not been established to date because its outcome is better than that of classic anti-GBM disease. This case report and literature review highlight atypical anti-GBM disease in infection-related rapidly progressive glomerulonephritis. Read More

Front Immunol 2020 14;11:597863. Epub 2020 Dec 14.

Département de médecine interne, Centre Hospitalier Universitaire de Reims, Reims, France.

Introduction: Anti-glomerular basement membrane (GBM) antibodies are pathogenic antibodies first detected in renal-limited anti-GBM disease and in Goodpasture disease, the latter characterized by rapidly progressive crescentic glomerulonephritis combined with intra-alveolar hemorrhage. Studies have suggested that anti-GBM antibody positivity may be of interest in lupus nephritis (LN). Moreover, severe anti-GBM vasculitis cases in patients with systemic lupus erythematosus (SLE) have been described in the literature, but few studies have assessed the incidence of anti-GBM antibodies in SLE patients. Read More

J Rheumatol 2021 02 1;48(2):228-231. Epub 2020 Sep 1.

K. Tselios, MD, PhD, D.D. Gladman, MD, FRCPC, C. Taheri, BHSc, J. Su, MB, MSc, M.B. Urowitz, MD, FRCPC, University of Toronto Lupus Clinic, Centre of Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, Ontario, Canada.

Objective: Lupus nephritis (LN) may lead to endstage kidney disease (ESKD) in 22% of patients over a period of 15 years, with the risk being particularly higher in diffuse proliferative forms. The rate of kidney function decline varies. However, a catastrophic course leading to ESKD within a few years from onset is uncommon. Read More

Nephron 2020 25;144 Suppl 1:49-53. Epub 2020 Nov 25.

Department of Kidney Disease Center, Nagoya Daini Red Cross Hospital, Nagoya, Japan.

Atypical anti-glomerular basement membrane (GBM) nephritis is a rare variant of the classical anti-GBM antibody disease. Patients present with an undetectable anti-GBM antibody but show linear glomerular basement membrane staining for immunoglobulin. We present a 69-year-old man who underwent a living-donor kidney transplant. Read More

Autoimmunity 2021 02 20;54(1):45-50. Epub 2020 Nov 20.

Central Diagnostic Laboratory, Maastricht University Medical Center, Maastricht, The Netherlands.

The use of high-quality antigen-specific immunoassays for detecting anti-neutrophil cytoplasmic antibodies (ANCA) and anti-glomerular basement membrane (GBM) autoantibodies is recommended in patients with suspected ANCA vasculitis and/or anti-GBM disease. We analysed the diagnostic performance of a semi-quantitative and rapid immunoblot (EUROIMMUN AG, Lübeck, Germany) in two settings. Patient sera from different cohorts (ANCA vasculitis  = 187, anti-GBM disease  = 19, and disease controls  = 51) were used. Read More