A nationwide study of the epidemiology of relapsing polychondritis

Anna Horváth, Nóra Páll, Katalin Molnár, Tamás Kováts, György Surján, T. Vicsek, Péter Pollner

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Objective: Relapsing polychondritis (RP) is a rare autoimmune inflammatory disease that attacks mainly cartilaginous structures or causes serious damage in proteoglycan-rich structures (the eyes, heart, blood vessels, inner ear). This study shows results regarding the epidemiology, progression, and associations of this highly variable disease by collecting all cases from a 124-million-person-year Central European nationwide cohort. Methods: We used the Hungarian Health Care Database to identify all persons with possible RP infection. We followed patients who had International Classification of Diseases 10th edition code M94.1 at least once in their inpatient or outpatient records between January 1, 2002 and December 31, 2013 in Hungary. We classified these patients into disease severity groups by their drug consumption patterns between January 1, 2010 and December 31, 2013. We analyzed the regional distribution of RP incidences as well. Overall maps of comorbidity are presented with network layouts. Results: We identified 256 patients with RP among cumulatively 11.5 million registered inhabitants. We classified these patients into four severity classes as “extremely mild” (n=144), “mild” (n=22), “moderate” (n=41), and “severe” (n=4). Two additional groups were defined for patients without available drug data as “suspected only” (n=23) and “confirmed but unknown treatment” (n=22). The age and sex distributions of patients were similar to worldwide statistics. Indeed, the overall survival was good (95% confidence interval for 5 years was 83.6%–92.9% and for 10 years was 75.0%–88.3% which corresponds to the overall survival of the general population in Hungary), and the associations with other autoimmune disorders were high (56%) in Hungary. Almost any disease can occur with RP; however, the symptoms of chromosomal abnormalities are only incidental. Spondylosis can be a sign of the activation of RP, while Sjögren syndrome is the most frequent autoimmune association. Regional distribution of incidences suggests arsenic drinking water and sunlight exposure as possible triggering factors. Conclusion: The good survival rate of RP in Hungary is probably associated with the early diagnosis of the disease.

Original languageEnglish
Pages (from-to)211-230
Number of pages20
JournalClinical Epidemiology
Volume8
DOIs
Publication statusPublished - Jun 23 2016

Fingerprint

Relapsing Polychondritis
Epidemiology
Hungary
Spondylosis
Sex Distribution
Survival
Sunlight
Incidence
Age Distribution
Arsenic
International Classification of Diseases
Inner Ear
Proteoglycans
Chromosome Aberrations
Drinking Water
Pharmaceutical Preparations
Autoimmune Diseases
Blood Vessels
Comorbidity
Inpatients

Keywords

  • Autoimmune comorbidity
  • Cohort of Hungary
  • Environmental factors
  • Incidence rate
  • Network representation
  • Severity prevalence

ASJC Scopus subject areas

  • Epidemiology

Cite this

Horváth, A., Páll, N., Molnár, K., Kováts, T., Surján, G., Vicsek, T., & Pollner, P. (2016). A nationwide study of the epidemiology of relapsing polychondritis. Clinical Epidemiology, 8, 211-230. https://doi.org/10.2147/CLEP.S91439

A nationwide study of the epidemiology of relapsing polychondritis. / Horváth, Anna; Páll, Nóra; Molnár, Katalin; Kováts, Tamás; Surján, György; Vicsek, T.; Pollner, Péter.

In: Clinical Epidemiology, Vol. 8, 23.06.2016, p. 211-230.

Research output: Contribution to journalArticle

Horváth, A, Páll, N, Molnár, K, Kováts, T, Surján, G, Vicsek, T & Pollner, P 2016, 'A nationwide study of the epidemiology of relapsing polychondritis', Clinical Epidemiology, vol. 8, pp. 211-230. https://doi.org/10.2147/CLEP.S91439
Horváth, Anna ; Páll, Nóra ; Molnár, Katalin ; Kováts, Tamás ; Surján, György ; Vicsek, T. ; Pollner, Péter. / A nationwide study of the epidemiology of relapsing polychondritis. In: Clinical Epidemiology. 2016 ; Vol. 8. pp. 211-230.
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