Rapidly progressive glomerulonephritis (RPGN) is a life-threatening disease characterized by rapid progressive deterioration of renal function and extensive formation of crescents. Some antibodies tend to be positive, such as a perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) and anti-glomerular basement membrane (anti-GBM) antibodies, in most patients with the disease. However, cases of double positivity for the above antibodies are considered to be rare. In addition, both rheumatoid arthritis (RA) and Sjogren's syndrome (SS) are deemed to be independent immune disorders that can cause renal impairment. Nevertheless, the association between RPGN and these two diseases has not been elucidated in previous studies. Here, we provide a case of RPGN with the concurrence of RA and SS characterized by double positivity in anti-GBM antibodies and p-ANCA. After aggressive treatment with cyclophosphamide, glucocorticoids, and plasma exchange, the patient improved significantly. Despite the malignant event of arteriovenous fistula rupture and bleeding during treatment, the patient survived with renal function recovery for the rest of the follow-up period.
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http://dx.doi.org/10.3390/jcm11226793 | DOI Listing |
Cureus
December 2024
Medicine, Faculdade de Medicina da Universidade do Porto (FMUP), Porto, PRT.
Anti-glomerular basement membrane disease is a rare small vessel vasculitis caused by the deposition of immunoglobulin G (IgG) autoantibodies in the basement membrane of glomerular capillaries and lung alveoli, leading to rapidly progressive renal failure and/or alveolar hemorrhage. We report the case of an 83-year-old female patient presenting with uremic symptoms, rapidly progressive kidney failure, and a high titer of anti-glomerular basement membrane antibodies. Given the urgent need for kidney replacement therapy, the substantial fibrosis and glomerular scarring observed in the kidney biopsy suggesting a chronic process, and the absence of pulmonary involvement, neither immunosuppressive treatment nor plasmapheresis was initiated, since a low likelihood of a favorable response to these interventions was expected.
View Article and Find Full Text PDFKidney Int
January 2025
Centre for Inflammatory Diseases, Department of Medicine, School of Clinical Sciences, Monash University, Clayton, Australia. Electronic address:
Anti-glomerular basement membrane (anti-GBM) disease is accompanied by insufficient antigen-specific T regulatory cells (Tregs) and clonally expanded antigen-specific T conventional cells (Tconvs). In particular, this applied to the immunodominant T cell auto- epitope of type IV collagen, α3(IV)NC1135-145 , presented by HLA-DR15. Here, we investigated whether Tregs engineered to express GBM-T cell receptors (TCR) specific for α3(IV)NC1135- 145 better suppress autoimmunity.
View Article and Find Full Text PDFClin Chem Lab Med
January 2025
Laboratory of Clinical Pathology, Azienda Sanitaria Universitaria Integrata, Udine, Italy.
Objectives: External quality assessment (EQA) programs play a pivotal role in harmonizing laboratory practices, offering users a benchmark system to evaluate their own performance and identify areas requiring improvement. The objective of this study was to go through and analyze the UK NEQAS "Immunology, Immunochemistry and Allergy" EQA reports between 2012 and 2021 to assess the overall level of harmonization in autoimmune diagnostics and identify areas requiring improvement for future actions.
Methods: The EQA programs reviewed included anti-nuclear (ANA), anti-dsDNA, anti-centromere, anti-extractable nuclear antigen (ENA), anti-phospholipids, anti-neutrophil cytoplasm (ANCA), anti-proteinase 3 (PR3), anti-myeloperoxidase (MPO), anti-glomerular basement membrane (GBM), rheumatoid factor (RF), anti-citrullinated protein antibodies (ACPA), mitochondrial (AMA), liver-kidney-microsomal (LKM), smooth muscle (ASMA), APCA, and celiac disease antibodies.
Front Immunol
January 2025
Department of Rheumatology, University of Texas Southwestern Medical Center, Dallas, TX, United States.
Background: Both intrinsic renal cells and immune cells contribute to driving renal inflammation and damage. However, the respective roles of intrinsic renal cells and immune cells in crescentic glomerulonephritis, and the key molecular factors driving pathogenesis are still unclear.
Methods: The roles of intrinsic renal cells and renal infiltrating immune cells in crescent formation were explored using renal transplantation after experimental anti-GBM disease induction in 129x1/svJ and C57BL/6J mice.
World J Emerg Med
January 2024
Department of Emergency, General Hospital of Tianjin Medical University, Tianjin 300052, China.
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