Systemic sclerosis and its pulmonary complications in The Netherlands: an epidemiological study

M C Vonk; B Broers; Y F Heijdra; E Ton; R Snijder; A P J van Dijk; J M van Laar; H Bootsma; P Th W van Hal; F H J van den Hoogen; P L A van Daele

doi:10.1136/ard.2008.091710

Systemic sclerosis and its pulmonary complications in The Netherlands: an epidemiological study

M C Vonk, B Broers, Y F Heijdra, E Ton, R Snijder, A P J van Dijk, J M van Laar, H Bootsma, P Th W van Hal, F H J van den Hoogen, P L A van Daele

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

UNLABELLED: 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.

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 studied further in the ongoing POEMAS study.

Original language	English
Pages (from-to)	961-965
Number of pages	5
Journal	Annals of the Rheumatic Diseases
Volume	68
Issue number	6
DOIs	https://doi.org/10.1136/ard.2008.091710
Publication status	Published - Jun 2009

Keywords

Adolescent
Adult
Age Distribution
Aged
Aged, 80 and over
Epidemiologic Methods
Female
Humans
Hypertension, Pulmonary
Incidence
Lung
Male
Middle Aged
Netherlands
Prevalence
Pulmonary Fibrosis
Registries
Scleroderma, Systemic
Sex Distribution
Surveys and Questionnaires
Total Lung Capacity
Young Adult
Journal Article
Research Support, Non-U.S. Gov't

Access to Document

10.1136/ard.2008.091710

Cite this

Vonk, M. C., Broers, B., Heijdra, Y. F., Ton, E., Snijder, R., van Dijk, A. P. J., van Laar, J. M., Bootsma, H., van Hal, P. T. W., van den Hoogen, F. H. J., & van Daele, P. L. A. (2009). Systemic sclerosis and its pulmonary complications in The Netherlands: an epidemiological study. Annals of the Rheumatic Diseases, 68(6), 961-965. https://doi.org/10.1136/ard.2008.091710

@article{473863330c3840dcb8acdd34314185ae,

title = "Systemic sclerosis and its pulmonary complications in The Netherlands: an epidemiological study",

abstract = "UNLABELLED: 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.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 studied further in the ongoing POEMAS study.",

keywords = "Adolescent, Adult, Age Distribution, Aged, Aged, 80 and over, Epidemiologic Methods, Female, Humans, Hypertension, Pulmonary, Incidence, Lung, Male, Middle Aged, Netherlands, Prevalence, Pulmonary Fibrosis, Registries, Scleroderma, Systemic, Sex Distribution, Surveys and Questionnaires, Total Lung Capacity, Young Adult, Journal Article, Research Support, Non-U.S. Gov't",

author = "Vonk, {M C} and B Broers and Heijdra, {Y F} and E Ton and R Snijder and {van Dijk}, {A P J} and {van Laar}, {J M} and H Bootsma and {van Hal}, {P Th W} and {van den Hoogen}, {F H J} and {van Daele}, {P L A}",

year = "2009",

month = jun,

doi = "10.1136/ard.2008.091710",

language = "English",

volume = "68",

pages = "961--965",

journal = "Annals of the Rheumatic Diseases",

issn = "0003-4967",

publisher = "BMJ Publishing Group",

number = "6",

}

TY - JOUR

T1 - Systemic sclerosis and its pulmonary complications in The Netherlands

T2 - an epidemiological study

AU - Vonk, M C

AU - Broers, B

AU - Heijdra, Y F

AU - Ton, E

AU - Snijder, R

AU - van Dijk, A P J

AU - van Laar, J M

AU - Bootsma, H

AU - van Hal, P Th W

AU - van den Hoogen, F H J

AU - van Daele, P L A

PY - 2009/6

Y1 - 2009/6

N2 - UNLABELLED: 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.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 studied further in the ongoing POEMAS study.

AB - UNLABELLED: 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.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 studied further in the ongoing POEMAS study.

KW - Adolescent

KW - Adult

KW - Age Distribution

KW - Aged

KW - Aged, 80 and over

KW - Epidemiologic Methods

KW - Female

KW - Humans

KW - Hypertension, Pulmonary

KW - Incidence

KW - Lung

KW - Male

KW - Middle Aged

KW - Netherlands

KW - Prevalence

KW - Pulmonary Fibrosis

KW - Registries

KW - Scleroderma, Systemic

KW - Sex Distribution

KW - Surveys and Questionnaires

KW - Total Lung Capacity

KW - Young Adult

KW - Journal Article

KW - Research Support, Non-U.S. Gov't

U2 - 10.1136/ard.2008.091710

DO - 10.1136/ard.2008.091710

M3 - Article

C2 - 18511546

SN - 0003-4967

VL - 68

SP - 961

EP - 965

JO - Annals of the Rheumatic Diseases

JF - Annals of the Rheumatic Diseases

IS - 6

ER -

Systemic sclerosis and its pulmonary complications in The Netherlands: an epidemiological study

Abstract

Keywords

Access to Document

Fingerprint

Cite this