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Clinical validation of immunoglobulin A nephropathy diagnosis in Swedish biopsy registers
Örebro University, School of Medical Sciences. Department of Pediatrics, Faculty of Health and Medical Sciences, Örebro University, Örebro, Sweden.
Karolinska University Hospital, Stockholm, Sweden; Karolinska Institutet, Stockholm, Sweden.
Boston Consulting Group, Stockholm, Sweden; Karolinska Institutet, Stockholm, Sweden.
Karolinska Institutet, Stockholm, Sweden.
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2017 (English)In: Clinical Epidemiology, E-ISSN 1179-1349, Vol. 9, p. 67-73Article in journal (Refereed) Published
Abstract [en]

Aims: The aims of this study were to validate the diagnosis of IgA nephropathy (IgAN) in Swedish biopsy registers against patient charts and to describe the clinical characteristics of patients with a biopsy indicating IgAN.

Methods: This is a population-based cohort study. Out of 4,069 individuals with a renal biopsy consistent with IgAN (biopsies performed in 1974-2011), this study reviewed patient charts of a random subset of 127 individuals. Clinical and biopsy characteristics at the time of biopsy were evaluated, and positive predictive values (PPV) were calculated with 95% confidence intervals (CI).

Results: Out of 127 individuals with a renal biopsy consistent with IgAN, 121 had a likely or confirmed clinical diagnosis of IgAN, primary or secondary to Henoch-Schonlein purpura, yielding a PPV of 95% (95% CI =92%-99%). The median age at biopsy was 39 years (range: 4-79 years); seven patients (6%) were <16 years. The male to female ratio was 2.8:1. The most common causes for consultation were macroscopic hematuria (n=37; 29%), screening (n=33; 26%), and purpura (n=14, 11%). In patients with available data, the median creatinine level was 104 mu mol/L (range 26-986 mu mol/L, n=110) and glomerular filtration rate 75 mL/min/1.73m(2) (range 5-173 mL/min/1.73m(2), n=114). Hypertension was noted in 59 (46%) individuals. IgA deposits were reported in 97% of the biopsy records (n= 123), mesangial hypercellularity in 76% (n= 96), C3 deposits in 89% (n=113), and C1q deposits in 12% (n=15). Conclusion: A histologic diagnosis of IgAN has a high PPV for a diagnosis of IgAN confirmed by review of patient charts.

Place, publisher, year, edition, pages
Dove Medical Press Ltd , 2017. Vol. 9, p. 67-73
Keywords [en]
general population-based, histopathology, IgA nephropathy, kidney, renal disease, validation studies
National Category
Occupational Health and Environmental Health
Identifiers
URN: urn:nbn:se:oru:diva-63791DOI: 10.2147/CLEP.S118730ISI: 000392987200001PubMedID: 28203107Scopus ID: 2-s2.0-85011961668OAI: oai:DiVA.org:oru-63791DiVA, id: diva2:1170473
Note

Funding Agency: Orebro County Council OLL-406471 

Available from: 2018-01-03 Created: 2018-01-03 Last updated: 2024-07-04Bibliographically approved
In thesis
1. Immunoglobulin A nephropathy and disease complications: register-based studies
Open this publication in new window or tab >>Immunoglobulin A nephropathy and disease complications: register-based studies
2023 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Immunoglobulin A nephropathy (IgAN) is the commonest primary glomerular disease worldwide. A kidney biopsy is required for the diagnosis. IgA immune-complex depositions sets off a cascade leading to renal scarring, proteinuria and hypertension. Peaking in young adults, IgAN contributes significantly to the burden of chronic kidney disease, which in turn may lead to cardiovascular disease and death. As IgAN peaks in childbearing age, its effect on pregnancy outcomes is of interest. 

All studies use the same cohort of 4126 patients with a biopsy diagnosis of IgAN, identified through the combination of computerized andmanual search in biopsy reports from all Swedish kidney pathology labs. In study I, a random subset of 127 patients from the biopsy cohort were selected for diagnosis validation by patient chart review. IgAN was confirmed or likely in 121 cases (positive predictive value > 95 %). Mean age at diagnosis was 39.6 years, 74 % were male. 

Study II compared mortality in IgAN patients and an individuallymatched reference population by survival analysis. IgAN was associated with an increase of 53 % in all-cause and 59 % in cardiovascular mortality, with an absolute excess death rate of in 310 person years. Mortality before end-stage renal disease was not significantly increased.

Study III used a similar design to examine incident fatal and non-fatal ischemic heart disease (IHD) in IgAN patients and the same reference populations. We found an 86 % increase in IHD hazard and an absolute excess IHD risk of one per 340 person-years. 

In study IV, outcomes of 327 pregnancies in 208 women with IgAN were compared to reference pregnancies without IgAN, indicating increased odds of preterm birth < 37 weeks gestation, but not for very preterm birth < 34 weeks. Preeclampsia odds were quadrupled. Stillbirth and neonatal death were both uncommon and not increased in IgAN.

Place, publisher, year, edition, pages
Örebro: Örebro University, 2023. p. 90
Series
Örebro Studies in Medicine, ISSN 1652-4063 ; 275
Keywords
immunoglobulin A nephropathy, nephrology, validation studies, register studies, cohort studies, mortality, ischemic heart disease, pregnancy, preterm birth
National Category
General Practice Urology and Nephrology
Identifiers
urn:nbn:se:oru:diva-102261 (URN)9789175294834 (ISBN)
Public defence
2023-01-20, Örebro universitet, Campus USÖ, hörsal C1, Södra Grev Rosengatan 32, Örebro, 13:00 (English)
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Available from: 2022-11-17 Created: 2022-11-17 Last updated: 2023-01-26Bibliographically approved

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Ludvigsson, Jonas F.

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