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Clinical characteristics and outcomes of 566 Thais with systemic sclerosis: A cohort study

Chingching Foocharoen1, Udomlack Peansukwech2, Ajanee Mahakkanukrauh1

  • 1Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.

International Journal of Rheumatic Diseases
|May 19, 2020
PubMed

Insights

Diffuse cutaneous systemic sclerosis (dcSSc) is common in Thai patients. Early pulmonary fibrosis detection and factors like older age, high functional class, and organ involvement increase mortality risk in systemic sclerosis (SSc).

Area of Science:

  • Rheumatology
  • Dermatology
  • Pulmonology

Background:

  • Systemic sclerosis (SSc) predominantly presents as diffuse cutaneous SSc (dcSSc) in Thai patients, differing from Caucasian and some Asian populations.
  • A longitudinal study of Thai dcSSc patients is crucial for understanding disease progression and outcomes.

Purpose of the Study:

  • To characterize the clinical features of Thai SSc patients.
  • To differentiate between limited cutaneous SSc (lcSSc) and dcSSc.
  • To determine SSc mortality rates and associated risk factors.

Main Methods:

  • A cohort study of 566 adult Thai SSc patients was conducted from January 2013 to June 2019.
  • Generalized estimating equations (GEE) were used to analyze clinical differences between lcSSc and dcSSc.

Main Results:

  • The majority of patients were female (72.6%) and had dcSSc (72.6%).
  • Pulmonary fibrosis (PF) was detected early (median 2.5 years), with pulmonary arterial hypertension and renal crisis occurring later.
  • dcSSc correlated with specific skin manifestations (salt-and-pepper, deformity) and higher modified Rodnan skin scores (mRSS).
  • Mortality risk factors included older age at onset, high World Health Organization functional class (FC III/IV), edematous skin, early PF, higher mRSS, and multiple organ involvement.

Conclusions:

  • Diffuse cutaneous systemic sclerosis (dcSSc) is the predominant subtype in Thailand.
  • Pulmonary fibrosis (PF) is an early complication of SSc, and its early detection indicates a poorer prognosis.
  • Factors such as advanced age at onset, severe functional limitation, extensive skin involvement, and multi-organ disease significantly increase mortality risk in SSc patients.
Abstract

Keywords:
clinical trialcohort studymortalitysclerodermasystemic sclerosis

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