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 [
1
,
2
]. 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 [
3
,
4
,
5
,
6
,
7
,
8
]. 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 [
[7]
]. 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 [
[9]
]. 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.
[3]
|
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.
[4]
|
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.
[5]
|
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.
[6]
|
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.
[7]
|
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.
[8]
|
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
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to European Journal of Internal MedicineAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- IgA vasculitis (Henoch-Shönlein purpura) in adults: diagnostic and therapeutic aspects.Autoimmun Rev. 2015; 14: 579-585https://doi.org/10.1016/j.autrev.2015.02.003
- Epidemiology of immunoglobulin A vasculitis (Henoch-Schönlein): current state of knowledge.Curr Opin Rheumatol. 2013; 25: 171-178https://doi.org/10.1097/BOR.0b013e32835d8e2a
- Epidemiology of Schönlein-Henoch purpura.Acta Paediatr Scand. 1988; 77: 125-131
- Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch-Schönlein purpura in adults.Semin Arthritis Rheum. 1999; 28: 404-412
- Cutaneous vasculitis in a defined population-clinical and epidemiological associations.J Rheumatol. 1998; 25: 920-924
- Henoch-Schönlein purpura in adults: a study of 9 cases.An Med Interna. 2004; 21: 79-80
- An epidemiological study of Henoch-Schönlein purpura.Paediatr Nurs. 2010; 22: 30-35https://doi.org/10.7748/paed2010.12.22.10.30.c8135
- Incidence of IgA vasculitis in the adult Slovenian population.Br J Dermatol. 2014; 171: 524-527https://doi.org/10.1111/bjd.12946
- French health insurance databases: what interest for medical research?.Rev Med Interne. 2015; 36: 411-417https://doi.org/10.1016/j.revmed.2014.11.009
- EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria.Ann Rheum Dis. 2010; 69: 798-806https://doi.org/10.1136/ard.2009.116657
Article info
Publication history
Published online: June 24, 2017
Accepted:
May 24,
2017
Received:
May 20,
2017
Identification
Copyright
© 2017 Published by Elsevier B.V. on behalf of European Federation of Internal Medicine.
