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Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome
BACKGROUND: Although rapidly progressive glomerulonephritis is the main renal phenotype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), slow renal disease progression is sometimes observed. These forms have been rarely discussed; we analysed their prevalence, clinico-path...
Autores principales: | , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7857823/ https://www.ncbi.nlm.nih.gov/pubmed/33564436 http://dx.doi.org/10.1093/ckj/sfaa139 |
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author | Trivioli, Giorgio Gopaluni, Seerapani Urban, Maria L. Gianfreda, Davide Cassia, Matthias A. Vercelloni, Paolo G. Calatroni, Marta Bettiol, Alessandra Esposito, Pasquale Murtas, Corrado Alberici, Federico Maritati, Federica Manenti, Lucio Palmisano, Alessandra Emmi, Giacomo Romagnani, Paola Moroni, Gabriella Gregorini, Gina Sinico, Renato A. Jayne, David R.W. Vaglio, Augusto |
author_facet | Trivioli, Giorgio Gopaluni, Seerapani Urban, Maria L. Gianfreda, Davide Cassia, Matthias A. Vercelloni, Paolo G. Calatroni, Marta Bettiol, Alessandra Esposito, Pasquale Murtas, Corrado Alberici, Federico Maritati, Federica Manenti, Lucio Palmisano, Alessandra Emmi, Giacomo Romagnani, Paola Moroni, Gabriella Gregorini, Gina Sinico, Renato A. Jayne, David R.W. Vaglio, Augusto |
author_sort | Trivioli, Giorgio |
collection | PubMed |
description | BACKGROUND: Although rapidly progressive glomerulonephritis is the main renal phenotype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), slow renal disease progression is sometimes observed. These forms have been rarely discussed; we analysed their prevalence, clinico-pathological characteristics and outcome. METHODS: We screened patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis followed at seven referral centres and selected those with estimated glomerular filtration rate (eGFR) reduction <50% over a 6-month period preceding diagnosis. Data regarding patient features and response to treatment were retrieved. RESULTS: Of 856 patients, 41 (5%) had slowly progressive renal AAV. All had MPA and all but one was P-ANCA/myeloperoxidase (MPO) ANCA-positive. At diagnosis, the median age was 70 years [interquartile range (IQR) 64–78] and extra-renal manifestations were absent or subclinical (interstitial lung lesions in 10, 24%). The median (IQR) eGFR was 23 mL/min/1.73 m(2) (15–35); six patients (15%) had started renal replacement therapy (RRT). All had proteinuria (median 1180 mg/24 h, IQR 670–2600) and micro-haematuria. Main histologic findings were extracapillary proliferation at chronic stages and glomerulosclerosis; following Berden’s classification, 6/28 biopsies (21%) were ‘focal’, 1/28 (4%) ‘crescentic’, 9/28 (32%) ‘mixed’ and 12/28 (43%) ‘sclerotic’. At last follow-up (median 32 months, IQR 12–52), 20/34 patients (59%) treated with immunosuppression had eGFR improvement >25% as compared with diagnosis, while 4/34 (12%) had started RRT. CONCLUSIONS: AAV may present with slow renal disease progression; this subset is hallmarked by advanced age at diagnosis, positive MPO-ANCA, subclinical interstitial lung lesions and chronic damage at kidney biopsy. Partial renal recovery may occur following immunosuppression. |
format | Online Article Text |
id | pubmed-7857823 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-78578232021-02-08 Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome Trivioli, Giorgio Gopaluni, Seerapani Urban, Maria L. Gianfreda, Davide Cassia, Matthias A. Vercelloni, Paolo G. Calatroni, Marta Bettiol, Alessandra Esposito, Pasquale Murtas, Corrado Alberici, Federico Maritati, Federica Manenti, Lucio Palmisano, Alessandra Emmi, Giacomo Romagnani, Paola Moroni, Gabriella Gregorini, Gina Sinico, Renato A. Jayne, David R.W. Vaglio, Augusto Clin Kidney J Original Articles BACKGROUND: Although rapidly progressive glomerulonephritis is the main renal phenotype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), slow renal disease progression is sometimes observed. These forms have been rarely discussed; we analysed their prevalence, clinico-pathological characteristics and outcome. METHODS: We screened patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis followed at seven referral centres and selected those with estimated glomerular filtration rate (eGFR) reduction <50% over a 6-month period preceding diagnosis. Data regarding patient features and response to treatment were retrieved. RESULTS: Of 856 patients, 41 (5%) had slowly progressive renal AAV. All had MPA and all but one was P-ANCA/myeloperoxidase (MPO) ANCA-positive. At diagnosis, the median age was 70 years [interquartile range (IQR) 64–78] and extra-renal manifestations were absent or subclinical (interstitial lung lesions in 10, 24%). The median (IQR) eGFR was 23 mL/min/1.73 m(2) (15–35); six patients (15%) had started renal replacement therapy (RRT). All had proteinuria (median 1180 mg/24 h, IQR 670–2600) and micro-haematuria. Main histologic findings were extracapillary proliferation at chronic stages and glomerulosclerosis; following Berden’s classification, 6/28 biopsies (21%) were ‘focal’, 1/28 (4%) ‘crescentic’, 9/28 (32%) ‘mixed’ and 12/28 (43%) ‘sclerotic’. At last follow-up (median 32 months, IQR 12–52), 20/34 patients (59%) treated with immunosuppression had eGFR improvement >25% as compared with diagnosis, while 4/34 (12%) had started RRT. CONCLUSIONS: AAV may present with slow renal disease progression; this subset is hallmarked by advanced age at diagnosis, positive MPO-ANCA, subclinical interstitial lung lesions and chronic damage at kidney biopsy. Partial renal recovery may occur following immunosuppression. Oxford University Press 2020-09-06 /pmc/articles/PMC7857823/ /pubmed/33564436 http://dx.doi.org/10.1093/ckj/sfaa139 Text en © The Author(s) 2020. Published by Oxford University Press on behalf of ERA-EDTA. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
spellingShingle | Original Articles Trivioli, Giorgio Gopaluni, Seerapani Urban, Maria L. Gianfreda, Davide Cassia, Matthias A. Vercelloni, Paolo G. Calatroni, Marta Bettiol, Alessandra Esposito, Pasquale Murtas, Corrado Alberici, Federico Maritati, Federica Manenti, Lucio Palmisano, Alessandra Emmi, Giacomo Romagnani, Paola Moroni, Gabriella Gregorini, Gina Sinico, Renato A. Jayne, David R.W. Vaglio, Augusto Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome |
title | Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome |
title_full | Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome |
title_fullStr | Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome |
title_full_unstemmed | Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome |
title_short | Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome |
title_sort | slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome |
topic | Original Articles |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7857823/ https://www.ncbi.nlm.nih.gov/pubmed/33564436 http://dx.doi.org/10.1093/ckj/sfaa139 |
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