Clinical characteristics and survival of japanese patients with connective tissue disease and pulmonary arterial hypertension

A single-centre cohort

Yuichiro Shirai, Hidekata Yasuoka, Yutaka Okano, Tsutomu Takeuchi, Toru Satoh, Masataka Kuwana

Research output: Contribution to journalArticle

32 Citations (Scopus)

Abstract

Objective. To clarify the characteristics, survival and predictors of mortality in Japanese patients with pulmonary arterial hypertension (PAH) associated with CTD.Methods. This single-centre cohort study enrolled 70 consecutive patients with PAH-CTD who visited a tertiary referral centre in Japan between 1970 and 1990 (n = 30, historical group) and between 2000 and 2009 (n = 40, recent group). Baseline clinical features, haemodynamic parameters and ANA profiles were recorded. The Cox proportional hazards regression model was used to determine independent factors associated with an increased risk of mortality.Results. MCTD and SLE were the major underlying CTDs, comprising 43% and 29% of PAH patients respectively, whereas SSc was less common (19%). Anti-U1RNP antibody was the most prevalent ANA (61%). The cumulative survival rate was significantly better in the recent group in comparison with the historical group (76% vs 26% at 3 years; P < 0.001). When both groups were combined, World Health Organization functional class III or IV at diagnosis was identified as an independent predictor of mortality, whereas modern PAH drug use was associated with a favourable outcome.Conclusion. The major PAH-CTD population in Japan suffers from MCTD or SLE with anti-U1RNP antibody, in contrast to PAH-CTD patients in the USA and Europe. Modern PAH treatment has improved survival rates, but long-term outcomes are still unsatisfactory. Independent predictors of mortality indicate that early diagnosis and the prompt use of PAH drugs should improve survival.

Original languageEnglish
Article numberkes140
Pages (from-to)1846-1854
Number of pages9
JournalRheumatology (United Kingdom)
Volume51
Issue number10
DOIs
Publication statusPublished - 2012 Oct

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Connective Tissue Diseases
Pulmonary Hypertension
Survival
Mixed Connective Tissue Disease
Mortality
Anti-Idiotypic Antibodies
Japan
Survival Rate
Proportional Hazards Models
Tertiary Care Centers
Pharmaceutical Preparations
Early Diagnosis
Cohort Studies
Hemodynamics

Keywords

  • Anti-U1RNP antibody
  • Mixed connective tissue disease
  • Pulmonary arterial hypertension
  • Scleroderma
  • Systemic lupus erythematosus

ASJC Scopus subject areas

  • Rheumatology
  • Pharmacology (medical)

Cite this

Clinical characteristics and survival of japanese patients with connective tissue disease and pulmonary arterial hypertension : A single-centre cohort. / Shirai, Yuichiro; Yasuoka, Hidekata; Okano, Yutaka; Takeuchi, Tsutomu; Satoh, Toru; Kuwana, Masataka.

In: Rheumatology (United Kingdom), Vol. 51, No. 10, kes140, 10.2012, p. 1846-1854.

Research output: Contribution to journalArticle

Shirai, Yuichiro ; Yasuoka, Hidekata ; Okano, Yutaka ; Takeuchi, Tsutomu ; Satoh, Toru ; Kuwana, Masataka. / Clinical characteristics and survival of japanese patients with connective tissue disease and pulmonary arterial hypertension : A single-centre cohort. In: Rheumatology (United Kingdom). 2012 ; Vol. 51, No. 10. pp. 1846-1854.
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