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題名 | Pulmonary Arterial Hypertension in Autoimmune Diseases: An Analysis of 19 Cases from a Medical Center in Northern Taiwan=肺動脈高血壓在自體免疫疾病之表現於北臺灣 |
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作 者 | 陳俊雄; 陳宏安; 王宏斌; 廖顯宗; 周昌德; 黃德豐; | 書刊名 | 微免與感染雜誌 |
卷期 | 39:2 民95.04 |
頁次 | 頁162-168 |
分類號 | 415.382 |
關鍵詞 | 肺動脈高血壓; 自體免疫疾病; Autoimmune diseases; Pulmonary diffusion capacity; Pulmonary hypertension; Taiwan; Uric acid; |
語文 | 英文(English) |
英文摘要 | 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. 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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