Clinical characteristics and outcomes of 566 Thais with systemic sclerosis: A cohort study.
Adolescent
Adult
Age of Onset
Aged
Aged, 80 and over
Disease Progression
Female
Humans
Longitudinal Studies
Male
Middle Aged
Prognosis
Pulmonary Fibrosis
/ mortality
Risk Assessment
Risk Factors
Scleroderma, Diffuse
/ diagnosis
Scleroderma, Limited
/ diagnosis
Thailand
/ epidemiology
Time Factors
Young Adult
clinical trial
cohort study
mortality
scleroderma
systemic sclerosis
Journal
International journal of rheumatic diseases
ISSN: 1756-185X
Titre abrégé: Int J Rheum Dis
Pays: England
ID NLM: 101474930
Informations de publication
Date de publication:
Jul 2020
Jul 2020
Historique:
received:
14
03
2020
revised:
14
04
2020
accepted:
27
04
2020
pubmed:
19
5
2020
medline:
9
6
2021
entrez:
19
5
2020
Statut:
ppublish
Résumé
Most Thai patients with systemic sclerosis (SSc) have diffuse cutaneous SSc (dcSSc) unlike most Caucasians and some Asians. A longitudinal cohort study among Thai dcSSc is needed. We aimed to determine the overall clinical characteristics, define the clinical difference between limited cutaneous SSc (lcSSc) and dcSSc, and ascertain the mortality rate and the factors associated with mortality. We conducted a cohort study including 566 Thai adult SSc patients between January 2013 and June 2019. Clinical difference between lcSSc and dcSSc was investigated using generalized estimating equations (GEE). Females presented more than males (356 vs 210 cases). The majority of cases were dcSSc (411; 72.6%). The median duration of disease at the time of pulmonary fibrosis (PF) detection was 2.5 years, pulmonary arterial hypertension 8.1 years, and renal crisis 4.1 years. By GEE analysis, dcSSc was significantly associated with salt-and-pepper skin, hand deformity, and every 1-point increase in modified Rodnan skin score (mRSS). A greater mortality risk was associated with age at onset >60 years (hazards ratio [HR] 5.5), a World Health Organization functional class (FC) III (HR 5.1), FC IV (HR 34.8), edematous skin (HR 11.4), early onset of PF (HR 1.7), each 5-point increase in the mRSS (HR 4.5), and ≥2 internal organ involvements (HR 10.1). dcSSc is a common SSc subset among Thais. PF was an early complication in SSc and earlier PF detection was associated with a poorer prognosis. Elderly onset, high FC, severe skin tightness, and multiple organ involvements were associated with a greater mortality risk.
Sections du résumé
BACKGROUND
BACKGROUND
Most Thai patients with systemic sclerosis (SSc) have diffuse cutaneous SSc (dcSSc) unlike most Caucasians and some Asians. A longitudinal cohort study among Thai dcSSc is needed.
OBJECTIVES
OBJECTIVE
We aimed to determine the overall clinical characteristics, define the clinical difference between limited cutaneous SSc (lcSSc) and dcSSc, and ascertain the mortality rate and the factors associated with mortality.
METHOD
METHODS
We conducted a cohort study including 566 Thai adult SSc patients between January 2013 and June 2019. Clinical difference between lcSSc and dcSSc was investigated using generalized estimating equations (GEE).
RESULTS
RESULTS
Females presented more than males (356 vs 210 cases). The majority of cases were dcSSc (411; 72.6%). The median duration of disease at the time of pulmonary fibrosis (PF) detection was 2.5 years, pulmonary arterial hypertension 8.1 years, and renal crisis 4.1 years. By GEE analysis, dcSSc was significantly associated with salt-and-pepper skin, hand deformity, and every 1-point increase in modified Rodnan skin score (mRSS). A greater mortality risk was associated with age at onset >60 years (hazards ratio [HR] 5.5), a World Health Organization functional class (FC) III (HR 5.1), FC IV (HR 34.8), edematous skin (HR 11.4), early onset of PF (HR 1.7), each 5-point increase in the mRSS (HR 4.5), and ≥2 internal organ involvements (HR 10.1).
CONCLUSION
CONCLUSIONS
dcSSc is a common SSc subset among Thais. PF was an early complication in SSc and earlier PF detection was associated with a poorer prognosis. Elderly onset, high FC, severe skin tightness, and multiple organ involvements were associated with a greater mortality risk.
Identifiants
pubmed: 32420701
doi: 10.1111/1756-185X.13859
doi:
Types de publication
Comparative Study
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
945-957Subventions
Organisme : Scleroderma Research Group, Faculty of Medicine, Khon Kaen University
Informations de copyright
© 2020 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.
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