Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome
Autor: | Renato Alberto Sinico, Alessandra Palmisano, Federica Maritati, Paolo Gilles Vercelloni, Gina Gregorini, Maria Letizia Urban, Marta Calatroni, Giorgio Trivioli, Augusto Vaglio, Matthias A. Cassia, Alessandra Bettiol, Pasquale Esposito, Corrado Murtas, Paola Romagnani, David Jayne, Davide Gianfreda, Federico Alberici, Gabriella Moroni, Lucio Manenti, Giacomo Emmi, Seerapani Gopaluni |
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Přispěvatelé: | Trivioli, G, Gopaluni, S, Urban, M, Gianfreda, D, Cassia, M, Vercelloni, P, Calatroni, M, Bettiol, A, Esposito, P, Murtas, C, Alberici, F, Maritati, F, Manenti, L, Palmisano, A, Emmi, G, Romagnani, P, Moroni, G, Gregorini, G, Sinico, R, Jayne, D, Vaglio, A, Gopaluni, Seerapani [0000-0002-1584-6186], Jayne, David [0000-0002-1712-0637], Apollo - University of Cambridge Repository |
Jazyk: | angličtina |
Rok vydání: | 2020 |
Předmět: |
Vasculiti
medicine.medical_specialty medicine.medical_treatment ANCA end-stage renal disease glomerulonephritis microscopic polyangiitis rituximab vasculitis 030232 urology & nephrology urologic and male genital diseases Gastroenterology End stage renal disease 03 medical and health sciences 0302 clinical medicine Internal medicine Medicine Rapidly progressive glomerulonephritis Microscopic polyangiiti Renal replacement therapy AcademicSubjects/MED00340 Glomerulonephriti Anti-neutrophil cytoplasmic antibody 030203 arthritis & rheumatology Transplantation Kidney medicine.diagnostic_test business.industry Original Articles medicine.disease medicine.anatomical_structure Nephrology Renal biopsy business Microscopic polyangiitis Granulomatosis with polyangiitis |
Zdroj: | Clinical Kidney Journal |
Popis: | 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 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 m2 (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. |
Databáze: | OpenAIRE |
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