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Volume 39, Number 2, April 2006

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
Division of Allergy, Immunology and Rheumatology, Department of Medicine, Taipei Veterans General Hospital and National Yang-Ming University School of Medicine, Taipei; and Taipei Medial University-Municipal Wan Fang Hospital, Taipei, Taiwan

Received: April 18, 2005    Revised: June 27, 2005    Accepted: July 7, 2005   

 

Corresponding author:

Dr. De-Feng Huang, Division of Allergy, Immunology and Rheumatology, Department of Medicine, Taipei Veterans General Hospital, 201, Sec.2, Shih-Pai Road, Taipei 112, Taiwan. E-mail: dfhuang@vghtpe.gov.tw This e-mail address is being protected from spam bots, you need JavaScript enabled to view it



 

Background and purpose: 

Pulmonary arterial hypertension (PAH), a serious complication of autoimmune diseases, has rarely been reported in Taiwan.
 



 

Methods:

Nineteen patients with varioius 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 <80 mm Hg died. In all of the 19 patients, the severity of RVSP was significantly correlated with serum uric acid (UA) level (r = 0.686, p=0.001). Among the PAH patients without pneumonitis, the severity of RVSP inversely correlated with the diffusion capacity of the lung for carbon monoxide (DLCO) [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.

 



 

Conclusion:

PAH in autoimmune diseases could be potentially fatal with characteristic manifestations. Moreover, RVSP correlated directly with serum UA level and inversely with DLCO.

 



 

Key words:

Autoimmune diseases, pulmonary diffusion capacity, pulmonary hypertension, Taiwan, uric acid


 



 

J Microbiol Immunol Infect 2006;39:162-168.