Pulmonary arterial hypertension in autoimmune diseases: An analysis of 19 cases from a medical center in northern Taiwan

Chun Hsiung Chen, Horng An Chen, Hong Pin Wang, Hsien Tzung Liao, Chung Tei Chou, De Feng Huang

Research output: Contribution to journalArticle

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Abstract

Background and Purpose: Pulmonary arterial hypertension (PAH), a serious complication of autoimmune diseases, has rarely been reported in Taiwan. Methods: Nineteen patients with various autoimmune diseases diagnosed with PAH at Taipei Veterans General Hospital from 2002 to 2004 were enrolled; the underlying autoimmune diseases included systemic lupus erythematosus (n = 6), primary Sjögren's syndrome (n = 5), systemic sclerosis (n = 4), adult-onset Still's disease (n = 2), and mixed connective tissue disease (n = 2). The characteristic manifestations of underlying autoimmune diseases and the clinical features of PAH were analyzed. Results: There were 16 female and 3 male patients. The median age at onset of PAH was 44 years and the mean right ventricular systolic pressure (RVSP) was 67.9 mm Hg. Patients without pneumonitis had a significantly higher RVSP value than those with pneumonitis (77.5 ± 24.3 vs 54.8 ± 18.4 mm Hg, p=0.041). Four out of 7 patients (57.1%) with RVSP ≥80 mm Hg and 1 out of 12 patients (8.3%) with RVSP LCO) [r = -0.856, p=0.003]. The characteristic manifestations of underlying autoimmune diseases included a high incidence of Raynaud's phenomenon (15/19,78.9%), a high titer of antinuclear antibody (13/17, 76.5%), positive anti-ribonucleoprotein antibody (8/15, 53.3%), hypergammaglobulinemia (15/19, 78.9%), hyperuricemia(13/19, 68.4%), and less renal involvement. Conclusions: PAH in autoimmune diseases could be potentially fatal with characteristic manifestations. Moreover, RVSP correlated directly with serum UA level and inversely with DLCO.

Original languageEnglish
Pages (from-to)162-168
Number of pages7
JournalJournal of Microbiology, Immunology and Infection
Volume39
Issue number2
Publication statusPublished - Apr 2006

Fingerprint

Taiwan
Pulmonary Hypertension
Autoimmune Diseases
Ventricular Pressure
Blood Pressure
Pneumonia
Adult-Onset Still's Disease
Mixed Connective Tissue Disease
Hypergammaglobulinemia
Veterans Hospitals
Hyperuricemia
Raynaud Disease
Ribonucleoproteins
Systemic Scleroderma
Antinuclear Antibodies
Age of Onset
General Hospitals
Systemic Lupus Erythematosus
Anti-Idiotypic Antibodies
Kidney

Keywords

  • Autoimmune diseases
  • Pulmonary diffusion capacity
  • Pulmonary hypertension
  • Taiwan
  • Uric acid

ASJC Scopus subject areas

  • Microbiology (medical)
  • Immunology and Allergy
  • Immunology and Microbiology(all)

Cite this

Pulmonary arterial hypertension in autoimmune diseases : An analysis of 19 cases from a medical center in northern Taiwan. / Chen, Chun Hsiung; Chen, Horng An; Wang, Hong Pin; Liao, Hsien Tzung; Chou, Chung Tei; Huang, De Feng.

In: Journal of Microbiology, Immunology and Infection, Vol. 39, No. 2, 04.2006, p. 162-168.

Research output: Contribution to journalArticle

Chen, Chun Hsiung ; Chen, Horng An ; Wang, Hong Pin ; Liao, Hsien Tzung ; Chou, Chung Tei ; Huang, De Feng. / Pulmonary arterial hypertension in autoimmune diseases : An analysis of 19 cases from a medical center in northern Taiwan. In: Journal of Microbiology, Immunology and Infection. 2006 ; Vol. 39, No. 2. pp. 162-168.
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abstract = "Background and Purpose: Pulmonary arterial hypertension (PAH), a serious complication of autoimmune diseases, has rarely been reported in Taiwan. Methods: Nineteen patients with various autoimmune diseases diagnosed with PAH at Taipei Veterans General Hospital from 2002 to 2004 were enrolled; the underlying autoimmune diseases included systemic lupus erythematosus (n = 6), primary Sj{\"o}gren's syndrome (n = 5), systemic sclerosis (n = 4), adult-onset Still's disease (n = 2), and mixed connective tissue disease (n = 2). The characteristic manifestations of underlying autoimmune diseases and the clinical features of PAH were analyzed. Results: There were 16 female and 3 male patients. The median age at onset of PAH was 44 years and the mean right ventricular systolic pressure (RVSP) was 67.9 mm Hg. Patients without pneumonitis had a significantly higher RVSP value than those with pneumonitis (77.5 ± 24.3 vs 54.8 ± 18.4 mm Hg, p=0.041). Four out of 7 patients (57.1{\%}) with RVSP ≥80 mm Hg and 1 out of 12 patients (8.3{\%}) with RVSP LCO) [r = -0.856, p=0.003]. The characteristic manifestations of underlying autoimmune diseases included a high incidence of Raynaud's phenomenon (15/19,78.9{\%}), a high titer of antinuclear antibody (13/17, 76.5{\%}), positive anti-ribonucleoprotein antibody (8/15, 53.3{\%}), hypergammaglobulinemia (15/19, 78.9{\%}), hyperuricemia(13/19, 68.4{\%}), and less renal involvement. Conclusions: PAH in autoimmune diseases could be potentially fatal with characteristic manifestations. Moreover, RVSP correlated directly with serum UA level and inversely with DLCO.",
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AU - Huang, De Feng

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AB - Background and Purpose: Pulmonary arterial hypertension (PAH), a serious complication of autoimmune diseases, has rarely been reported in Taiwan. Methods: Nineteen patients with various autoimmune diseases diagnosed with PAH at Taipei Veterans General Hospital from 2002 to 2004 were enrolled; the underlying autoimmune diseases included systemic lupus erythematosus (n = 6), primary Sjögren's syndrome (n = 5), systemic sclerosis (n = 4), adult-onset Still's disease (n = 2), and mixed connective tissue disease (n = 2). The characteristic manifestations of underlying autoimmune diseases and the clinical features of PAH were analyzed. Results: There were 16 female and 3 male patients. The median age at onset of PAH was 44 years and the mean right ventricular systolic pressure (RVSP) was 67.9 mm Hg. Patients without pneumonitis had a significantly higher RVSP value than those with pneumonitis (77.5 ± 24.3 vs 54.8 ± 18.4 mm Hg, p=0.041). Four out of 7 patients (57.1%) with RVSP ≥80 mm Hg and 1 out of 12 patients (8.3%) with RVSP LCO) [r = -0.856, p=0.003]. The characteristic manifestations of underlying autoimmune diseases included a high incidence of Raynaud's phenomenon (15/19,78.9%), a high titer of antinuclear antibody (13/17, 76.5%), positive anti-ribonucleoprotein antibody (8/15, 53.3%), hypergammaglobulinemia (15/19, 78.9%), hyperuricemia(13/19, 68.4%), and less renal involvement. Conclusions: PAH in autoimmune diseases could be potentially fatal with characteristic manifestations. Moreover, RVSP correlated directly with serum UA level and inversely with DLCO.

KW - Autoimmune diseases

KW - Pulmonary diffusion capacity

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KW - Uric acid

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