Systemic sclerosis and its pulmonary complications in the Netherlands An epidemiological study

M C Vonk ^1*, B Broers MD², Y F Heijdra ¹, E Ton ³, R Snijders ⁴, A PJ van Dijk ¹, J M van Laar ¹, H Bootsma ⁵, P Th W van Hal ⁶, F HJ van den Hoogen ¹ and P LA van Daele ⁶

¹ Radboud University Nijmegen Medical Centre, Netherlands
² Academic Hospital Maastricht, Netherlands
³ University Medical Centre Utrecht, Netherlands
⁴ Antonius Hospital Nieuwegein, Netherlands
⁵ University Medical Centre Groningen, Netherlands
⁶ Erasmus Medical Centre Rotterdam, Netherlands

^* To whom correspondence should be addressed. E-mail: m.vonk{at}reuma.umcn.nl.

Accepted 22 May 2008

Abstract

The prevalence and incidence of systemic sclerosis (SSc) in the Netherlands is unknown. The same holds true for its leading causes of death: pulmonary fibrosis and pulmonary arterial hypertension (PAH), for which effective treatment options have become available recently.

Objective: to establish the prevalence and incidence of SSc and its pulmonary complications.

Methods: detailed information of patients in the POEMAS registry, "Pulmonary Hypertension Screening, a Multidisciplinary Approach in Scleroderma", consisting of 819 patients, was combined with a nationwide questionnaire.

Results: By combining the two sources we found the prevalence of SSc to be 8.9 per 105 adults. The incidence was 0.77 patients per 105 per year. PAH was diagnosed in 9.9% of the SSc patients. The prevalence of interstitial lung disease in SSc varied from 19 to 47% depending on the definition used.

Conclusion: This study clarifies the epidemiology of SSc in the Netherlands and confirms the frequent occurrence of pulmonary complications, based on 654 cases. This can and will be further studied in the ongoing POEMAS study.