Pulmonary arterial hypertension in autoimmune diseases: an analysis of 19 cases from a medical center in northern Taiwan.
OBJECTIVE
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 >or=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.


CONCLUSIONS
PAH in autoimmune diseases could be potentially fatal with characteristic manifestations. Moreover, RVSP correlated directly with serum UA level and inversely with DLCO.
Chen
Chun-Hsiung
CH
Division of Allergy, Immunology and Rheumatology, Department of Medicine, Taipei Veterans General Hospital and National Yang-Ming University School of Medicine, Taipei, Taiwan.
Chen
Horng-An
HA
Wang
Hong-Pin
HP
Liao
Hsien-Tzung
HT
Chou
Chung-Tei
CT
Huang
De-Feng
DF
D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood; D001327:Autoimmune Diseases / Q000150:complications*; D005260:Female; D006761:Hospitals; D006801:Humans; D006942:Hypergammaglobulinemia; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000401:mortality / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011014:Pneumonia; D011928:Raynaud Disease; D013624:Taiwan; D014527:Uric Acid / Q000097:blood; D017725:Ventricular Pressure
D000974:Antibodies, Antinuclear; C026904:deoxyribonucleoprotein antibodies; D014527:Uric Acid