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Positive predictive value of hospital discharge diagnosis code to identify immunoglobulin A vasculitis in France: A validation study

      Immunoglobulin A vasculitis (IgAV), formerly called Schonlein-Henoch purpura, belongs to immune complex small vessel vasculitis. IgAV is the most common vasculitis in childhood but is more rare in adults [
      • Audemard-Verger A.
      • Pillebout E.
      • Guillevin L.
      • Thervet E.
      • Terrier B.
      IgA vasculitis (Henoch-Shönlein purpura) in adults: diagnostic and therapeutic aspects.
      ,
      • Piram M.
      • Mahr A.
      Epidemiology of immunoglobulin A vasculitis (Henoch-Schönlein): current state of knowledge.
      ]. To date, very few epidemiological studies have addressed the incidence of IgAV (n = 6) (Table 1). Most studies have used monocentric patient hospital register or discharge diagnosis codes as the source of case identification, including patients who fulfilled pre-specified criteria, resulting in an annual incidence rate of 0.34–5.1/100,000 adults [
      • Nielsen H.E.
      Epidemiology of Schönlein-Henoch purpura.
      ,
      • García-Porrúa C.
      • González-Gay M.A.
      Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch-Schönlein purpura in adults.
      ,
      • Watts R.A.
      • Jolliffe V.A.
      • Grattan C.E.
      • Elliott J.
      • Lockwood M.
      • Scott D.G.
      Cutaneous vasculitis in a defined population-clinical and epidemiological associations.
      ,
      • Ameal Guirado A.I.
      • Montes Santiago J.
      Henoch-Schönlein purpura in adults: a study of 9 cases.
      ,
      • Penny K.
      • Fleming M.
      • Kazmierczak D.
      • Thomas A.
      An epidemiological study of Henoch-Schönlein purpura.
      ,
      • Hočevar A.
      • Rotar Z.
      • Ostrovršnik J.
      • Jurčić V.
      • Vizjak A.
      • Dolenc Voljč M.
      • et al.
      Incidence of IgA vasculitis in the adult Slovenian population.
      ]. The only multicentric study was conducted by Penny et al. and used patient hospital discharge records, but a procedure for diagnosis validation of the D69.0 code was not mentioned, with a probable IgAV incidence overestimation [
      • Penny K.
      • Fleming M.
      • Kazmierczak D.
      • Thomas A.
      An epidemiological study of Henoch-Schönlein purpura.
      ]. Currently, there is growing interest in epidemiology research using health insurance databases and the hospital discharge PMSI diagnosis codes to assess the nation-wide incidence of a given disease [
      • Moulis G.
      • Lapeyre-Mestre M.
      • Palmaro A.
      • Pugnet G.
      • Montastruc J.-L.
      • Sailler L.
      French health insurance databases: what interest for medical research?.
      ]. Moreover, most of these studies are limited by their small samples and use very different approaches to identify the cases. Thus, a national population-based study should be of interest to better understand the epidemiology of this condition. The objective of this work was to assess the positive predictive value (PPV) of a selected hospital discharge diagnosis code to identify adult IgAV in the French hospital database.
      Table 1Epidemiological studies on immunoglobulin A vasculitis in adults.
      First author (reference) Study area Study period Population Source of case identification Definition of IgA diagnosis Number of cases Annual incidence rate
      Nielsen et al.
      • Nielsen H.E.
      Epidemiology of Schönlein-Henoch purpura.
      Københavns Amt county (Denmark)

      Unicentric
      1977–1983 15 yr Review of diagnostic files from the central registration NR (patients identified as such from diagnostic files) NR 0.8/100,000
      García-Porrúa et al.
      • García-Porrúa C.
      • González-Gay M.A.
      Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch-Schönlein purpura in adults.
      Lugo (Spain)

      Unicentric
      1988–1997 21 yr Review of hospital cases Proven cutaneous vasculitis and criteria by Michel et al. 27 1.4/100,000
      Watts et al.
      • Watts R.A.
      • Jolliffe V.A.
      • Grattan C.E.
      • Elliott J.
      • Lockwood M.
      • Scott D.G.
      Cutaneous vasculitis in a defined population-clinical and epidemiological associations.
      Norfolk (United Kingdom)

      Unicentric
      1990–1994 >16 yr Review of hospital cases from a prospective register and department of pathology Biopsy-proven cutaneous vasculitis and ACR or CHCC criteria 7 (CHCC) -27 (ACR) 0.34–1.3/100,000
      Ameal Guirado et al.
      • Ameal Guirado A.I.
      • Montes Santiago J.
      Henoch-Schönlein purpura in adults: a study of 9 cases.
      Vigo (Spain)

      Unicentric
      1995–2002 >15 yr Review of hospital cases with 287.0 ICD-9 code Criteria by Michel et al. 9 0.65/100,000
      Penny et al.
      • Penny K.
      • Fleming M.
      • Kazmierczak D.
      • Thomas A.
      An epidemiological study of Henoch-Schönlein purpura.
      Scotland

      Multicentric
      1995–2007 16 yr Review of hospital cases from the General registrar office with 287.0 ICD-9 code and D69.0 ICD-10 code NR (patients identified as such from the General registrar office) 751 1.1–1.8/100,000
      Hočevar et al.
      • Hočevar A.
      • Rotar Z.
      • Ostrovršnik J.
      • Jurčić V.
      • Vizjak A.
      • Dolenc Voljč M.
      • et al.
      Incidence of IgA vasculitis in the adult Slovenian population.
      Ljubljana (Slovenia)

      Unicentric
      2010–2013 18 yr Review of hospital cases from electronic medical records with D69.0 ICD-10 code and from list of adult patients with an IgA vasculitis-compatible histological pattern on biopsy from the department of pathology Proven leukocytoclastic with IgA-dominant immune deposits and EULAR/PRINTO/PRESS criteria 81 5.1/100,000
      NR: not reported; yr: years; IgA: immunoglobulin A: ICD: International Classification of Diseases; ACR: American College of Rheumatology; CHCC: Chapel Hill Consensus Conference.

      Keywords

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