Renal and overall outcomes of double-positive (ANCA and anti-GBM antibodies) patients compared to ANCA-associated vasculitis patients with severe renal involvement: A multicenter retrospective study with systematic renal pathology analysis
| Autor: | Nolwenn Rabot, Xavier Puéchal, E Cornec-Le Gall, C. Levi, A. Karras, Albertine Aouba, R. Philip, A Huart, M. Touzot, Vincent Audard, M. Clerte, Eric Thervet |
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| Rok vydání: | 2021 |
| Předmět: |
Male
medicine.medical_specialty medicine.medical_treatment Immunology Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis urologic and male genital diseases Gastroenterology Antibodies Antineutrophil Cytoplasmic 03 medical and health sciences chemistry.chemical_compound Glomerulonephritis 0302 clinical medicine Rheumatology Internal medicine medicine Humans Immunology and Allergy 030212 general & internal medicine Renal replacement therapy Autoantibodies Retrospective Studies 030203 arthritis & rheumatology Creatinine medicine.diagnostic_test business.industry Glomerular basement membrane Autoantibody General Medicine medicine.disease medicine.anatomical_structure chemistry Renal pathology Female Renal biopsy business Vasculitis |
| Zdroj: | Scandinavian Journal of Rheumatology. 51:205-213 |
| ISSN: | 1502-7732 0300-9742 |
| Popis: | 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.Results: All DPPs (including 18% exhibiting negative serum anti-GBM antibodies) presented severe acute kidney failure with histological GBM involvement. Compared to AAV patients, they had higher serum creatinine (719 vs 501 μmol/L; p = 0.006) and a higher proportion of patients requiring initial renal replacement therapy (82% vs 36%; p < 0.001). Berden classification differed significantly (p = 0.003), with more crescentic glomerulonephritis and fewer sclerotic lesions in DPPs. One-year renal survival was significantly lower in DPPs than in AAV patients (27% vs 64%; p < 0.0002). With comparable proportions of ANCA subtypes (two-thirds with anti-myeloperoxidase autoantibodies), numbers of extrarenal manifestations (mostly pulmonary in two-thirds), remission-inducing immunosuppressants, and median follow-ups (3 years) between groups, relapse rates were similar: 9.1% of DPPs and 10% of AAV patients.Conclusion: Although DPPs have features of both kinds of vasculitis, the anti-GBM component is the dominant phenotype, with more severe renal presentation and prognosis compared to AAV patients with severe renal failure. Simultaneous testing of both antibodies and systematically performed renal biopsy should be recommended in all rapidly progressing glomerulonephritis patients to recognize this difficult-to-treat, rare disease. |
| Databáze: | OpenAIRE |
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