Alvise Berti1,2,3, Emilie Cornec-Le Gall4,5, Divi Cornec3,6, Marta Casal Moura3, Eric L Matteson7,8, Cynthia S Crowson9,10, Aishwarya Ravindran11, Sanjeev Sethi11, Fernando C Fervenza4, Ulrich Specks3. 1. Immunology, Rheumatology, Allergy and Rare Diseases Department, San Raffaele Scientific Institute, Milan, Italy. 2. Rheumatology Unit, Santa Chiara Hospital, Trento, Italy. 3. Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. 4. Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA. 5. INSERM UMR1078, Génétique, Génomique Fonctionnelle et Biotechnologies, Université de Brest, Service de Néphrologie, CHU de Brest, Brest, France. 6. INSERM UMR1227, Lymphocytes B et Autoimmunité, Université de Bretagne Occidentale, Service de Rhumatologie et Centre National de Référence des maladies auto-immunes systémiques rares CERAINO, CHU de Brest, Brest, France. 7. Division of Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. 8. Division of Epidemiology, Department of Health Sciences Research, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. 9. Division of Biomedical Statistics and Informatics, Department of Health Sciences Research, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. 10. Division of Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA and. 11. Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
Abstract
BACKGROUND: True population-based clinical and outcomes data are lacking for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis (AAGN). Therefore we aimed to estimate the incidence, prevalence and mortality of AAGN, as well as the relationship between the grade of chronic renal damage at presentation and renal and non-renal outcomes. METHODS: Patients with AAGN were identified among a population-based incident cohort of 57 Olmsted County residents diagnosed with ANCA-associated vasculitis (AAV) in 1996-2015. Incidence rates were age and sex adjusted to the 2010 US white population. Age- and sex-adjusted prevalence was calculated for 1 January 2015. Survival rates were compared with expected rates in the Minnesota population. Chronic renal damage was assessed by chronicity score (CS) on biopsies performed at diagnosis. RESULTS: Thirty-four (60%) patients had AAGN. Of these, 65% had microscopic polyangiitis (MPA) and 74% were myeloperoxidase (MPO)-ANCA positive. The annual incidence of AAGN was 2.0/100 000 population [95% confidence interval (CI) 1.3-2.7] and the overall prevalence was 35/100 000 (95% CI 24-47). Mortality for AAGN was increased (P < 0.001), whereas mortality for AAV without glomerulonephritis did not differ from the general population. Minimal to mild CS predicted recovery of renal function at 1 year; clinical diagnosis (granulomatosis with polyangiitis versus MPA) and ANCA specificity (proteinase 3 versus MPO) did not. This observation was replicated in an independent cohort of 38 newly diagnosed AAGN patients seen at our centre over the 1999-2014 period. CONCLUSIONS: The annual incidence and prevalence of AAGN in Minnesota are 2.0/100 000 and 35/100 000, respectively. Mortality is worse compared with AAV patients without glomerulonephritis. More advanced renal damage at diagnosis predicts less renal recovery.
BACKGROUND: True population-based clinical and outcomes data are lacking for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis (AAGN). Therefore we aimed to estimate the incidence, prevalence and mortality of AAGN, as well as the relationship between the grade of chronic renal damage at presentation and renal and non-renal outcomes. METHODS:Patients with AAGN were identified among a population-based incident cohort of 57 Olmsted County residents diagnosed with ANCA-associated vasculitis (AAV) in 1996-2015. Incidence rates were age and sex adjusted to the 2010 US white population. Age- and sex-adjusted prevalence was calculated for 1 January 2015. Survival rates were compared with expected rates in the Minnesota population. Chronic renal damage was assessed by chronicity score (CS) on biopsies performed at diagnosis. RESULTS: Thirty-four (60%) patients had AAGN. Of these, 65% had microscopic polyangiitis (MPA) and 74% were myeloperoxidase (MPO)-ANCA positive. The annual incidence of AAGN was 2.0/100 000 population [95% confidence interval (CI) 1.3-2.7] and the overall prevalence was 35/100 000 (95% CI 24-47). Mortality for AAGN was increased (P < 0.001), whereas mortality for AAV without glomerulonephritis did not differ from the general population. Minimal to mild CS predicted recovery of renal function at 1 year; clinical diagnosis (granulomatosis with polyangiitis versus MPA) and ANCA specificity (proteinase 3 versus MPO) did not. This observation was replicated in an independent cohort of 38 newly diagnosed AAGN patients seen at our centre over the 1999-2014 period. CONCLUSIONS: The annual incidence and prevalence of AAGN in Minnesota are 2.0/100 000 and 35/100 000, respectively. Mortality is worse compared with AAV patients without glomerulonephritis. More advanced renal damage at diagnosis predicts less renal recovery.
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