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J Clin Med 2021 Apr 18;10(8). Epub 2021 Apr 18.

Statistics and Epidemiology Consultant, ARJR Media LLC, Scottsdale, AZ 85259, USA.

The current data on rates and geographic distribution of vasculitis mortality are limited. We aimed to estimate the mortality rates of primary systemic vasculitis and its geographic distribution using recent population data in the United States. The mortality rates of vasculitis from 1999 to 2019 were obtained from the Center for Disease Control (CDC) Wonder Multiple Cause of Death (MCD). Read More

J Am Soc Nephrol 2021 Apr 23. 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 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

Am J Case Rep 2020 Nov 24;21:e927030. Epub 2020 Nov 24.

Department of Internal Medicine, University of Pittsburgh Medical Center (UPMC) Pinnacle, Harrisburg, PA, USA.

BACKGROUND The diagnosis of systemic sclerosis sine scleroderma (ssSSc) with renal crisis is difficult because of its unusual presentation and rarity. CASE REPORT A 45-year-old man presented to the Emergency Department with worsening nausea, vomiting, and exertional dyspnea for 3 weeks. Initial examination showed blood pressure 182/108 mmHg without skin thickening or other skin manifestations. Read More

Can J Kidney Health Dis 2020 15;7:2054358120962680. Epub 2020 Oct 15.

Department of Medicine, Queen Elizabeth II Health Sciences Centre, Halifax, NS, Canada.

Rationale: Alemtuzumab is a monoclonal antibody approved for the treatment of relapsing-remitting multiple sclerosis (RRMS). Many autoimmune-mediated adverse events have been associated with alemtuzumab, including renal-limited anti-glomerular basement membrane (GBM) disease.

Presenting Concern: A 52-year-old female with RRMS presented with acute kidney injury 39 months after receiving 1 cycle of alemtuzumab. Read More

Wiad Lek 2020 ;73(9 cz. 1):1861-1866

DEPARTAMENT OF REHABILITATION, CITY HOSPITAL OF TORUN, TORUN, POLAND.

One of the diseases leading to chronic end-stage renal disease is membranous nephropathy (MN). The main cause of this disease is the formation of antibodies to foreign and native antigens. Membranous nephropathy can be conventionally divided into 2 types: primary form (when the primary disease is unknown) and secondary form. Read More

Front Immunol 2020 3;11:2035. Epub 2020 Sep 3.

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

Atypical cases of anti-glomerular basement membrane (GBM) disease had absent circulating antibodies but linear IgG deposits along GBM in the kidneys. Herein, we reported the clinical-pathological features and outcome of these rare cases. Linear IgG deposit along GBM were examined by immunofluorescence on renal specimens, with exclusion of diabetic kidney disease. Read More

Medicine (Baltimore) 2020 Sep;99(39):e22341

Division of Nephrology, Department of Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan.

Rationale: The Goodpasture syndrome is an extremely rare disease, with renal and pulmonary manifestations, and is mediated by anti-glomerular basement membrane (anti-GBM) antibodies. Renal pathological changes are mainly characterized by glomerular crescent formation and linear immunofluorescent staining for immunoglobulin G on the GBM. There are few reports on the atypical course of the syndrome involving serum-negative anti-GBM antibodies. Read More

Vnitr Lek 2020 ;66(5):56-61

Pulmonary syndrome is defined by occurrence of lung involvement (alveolar haemorrhage) in association with renal failure (with a typical crescentic necrotizing rapidly progressive glomerulonephritis). It is caused by an autoimmune disease, most frequently ANCA-associated vasculitides and anti-GBM (glomerular basement membrane) disease. Early establishment of the right diagnosis and immediate treatment are crucial for favourable prognosis of the patients. Read More

Ir Med J 2020 03 13;113(3):41. Epub 2020 Mar 13.

St. Vincent's University Hospital Group, Dublin 4.

Presentation A 28 year old female presented to the emergency department with a one week history of headache, vomiting and diaphoresis. Creatinine on admission was 492 and urinalysis revealed blood and protein. This was 5 months after a second infusion of Alemtuzumab, for treatment of highly active relapsing remitting multiple sclerosis. Read More

J Am Soc Nephrol 2020 06;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

Am J Med 2020 08 17;133(8):e436-e437. Epub 2020 Feb 17.

Department of Nephrology, Icahn School of Medicine at Mount Sinai, New York, NY.

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 21;51(2):96-98. Epub 2020 Jan 21.

Geffen School of Medicine at UCLA, Los Angeles, California, USA,

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

Rev Med Interne 2020 Jan 12;41(1):21-26. Epub 2019 Dec 12.

Service de médecine interne et immunologie clinique, UNICAEN, Normandie Université, CHU de Caen Normandie, 14000 Caen, France.

Double-positive vasculitis with anti-polynuclear cytoplasm (ANCA) and anti-glomerular basement membrane (GBM) antibodies is a rare entity of systemic vasculitis defined by the presence of ANCA and anti-GBM antibodies. The gradual accumulation of clinical and therapeutic data shows the usefulness of identifying and differentiating this entity from the two vasculitis respectively associated with the isolated presence of each of these two antibodies. Indeed, the double-positive ANCA and anti-GBM vasculitis appears to associate the characteristics of the demography and the extra-renal and pulmonary involvement of the ANCA-associated vasculitis on the one hand, and of the histological type and severe renal prognosis of the anti-MBG vasculitis on the other hand, with the renal involvement which is the only involvement consistently observed in double-positive vasculitis. Read More

Clin Rheumatol 2020 Mar 4;39(3):831-840. Epub 2019 Dec 4.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.

Objectives: To investigate the detection rate of double positivity for antineutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane (GBM) antibody at diagnosis and its clinical implication during follow-up in Korean patients with ANCA-associated vasculitis (AAV).

Methods: We retrospectively reviewed the medical records of 96 Korean AAV patients. We obtained data at diagnosis and assessed the poor outcomes of AAV such as all-cause mortality, relapse and end-stage renal disease (ESRD). Read More

Front Immunol 2019 14;10:2659. Epub 2019 Nov 14.

Division of Nephrology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, United States.

Goodpasture's disease (GP) is mediated by autoantibodies that bind the glomerular and alveolar basement membrane, causing rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. The autoantibodies bind neoepitopes formed upon disruption of the quaternary structure of α345NC1 hexamer, a critical structural domain of α345 collagen IV scaffolds. Hexamer disruption leads to a conformational changes that transitions α3 and α5NC1 subunits into immunogens, however, the trigger remains unknown. 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

J Am Soc Nephrol 2020 01 30;31(1):40-53. Epub 2019 Oct 30.

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

Background: In Goodpasture disease, the noncollagenous domain 1 of the 3 chain (3NC1) of type IV collagen is the main target antigen of antibodies against glomerular basement membrane (GBM). We previously identified a nephritogenic epitope, P14 (3), that could induce crescentic nephritis in WKY rats, and defined its core motif. Designing a modified peptide, replacing critical pathogenic residues with nonpathogenic ones (on the basis of homologous regions in 1NC1 chain of type IV collagen, known to be nonpathogenic), might provide a therapeutic option for anti-GBM GN. 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