Systemic sclerosis and its pulmonary complications in The Netherlands: an epidemiological study.
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 recently become available.


OBJECTIVE
To establish the prevalence and incidence of SSc and its pulmonary complications.


METHODS
Detailed information on 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 the prevalence of SSc was found to be 8.9 per 100 000 adults. The incidence was 0.77 patients per 100 000 per year. PAH was diagnosed in 9.9% of SSc patients. The prevalence of interstitial lung disease in SSc varied from 19% to 47% depending on the definition used.


CONCLUSIONS
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 studied further in the ongoing POEMAS study.
Department of Rheumatology, Cardiology of the Radboud University Nijmegen Medical Centre, The Netherlands. M.Vonk@reuma.umcn.nl
M C
MC
Vonk
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B
Broers
Y F
YF
Heijdra
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E
Ton
R
R
Snijder
A P J
AP
van Dijk
J M
JM
van Laar
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H
Bootsma
P Th W
PT
van Hal
F H J
FH
van den Hoogen
P L A
PL
van Daele
D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000453:epidemiology / Q000503:physiopathology; D015994:Incidence; D008168:Lung / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009426:Netherlands / Q000453:epidemiology; D015995:Prevalence; D011658:Pulmonary Fibrosis / Q000150:complications / Q000453:epidemiology / Q000503:physiopathology; D012042:Registries; D012595:Scleroderma, Systemic / Q000150:complications / Q000453:epidemiology* / Q000503:physiopathology; D017678:Sex Distribution; D011795:Surveys and Questionnaires; D014109:Total Lung Capacity; D055815:Young Adult