肺高血圧症を伴ったSjögren症候群の1例

A case of Sjögren's syndrome associated with pulmonary hypertension

　Sjögren症候群にみられる肺病変の出現頻度は，Lie—bowら（1973）^1）の報告では1.9％と少ないものとされていたが，その後Strimlan^2）らは9％と報告し，そのほかSilbigcrら^3）は42例中20例（42％），わが国の平木^4）らも59例中28例（47％）に胸部X線上何らかの異常を呈していたと報告している。

　このようにSjögren症候群における肺病変は，かつて考えられていたよりも高率なものと思われる。

A case of Sjögren's syndrome associated with pulmonary hypertension of a 29-years old house wife was reported.

She was admitted to our hospital for dry mouth, dry eye, palpitation, and exertional dyspnea.

Chest X-ray film and pulmonary function were very similar to interstitial pneumonia.

The electrocardiogram showed a right ventric-ular overload, and sialography was consistent with Sjögren's syndrome.

Righ-sided cardiac catheterization revealed the pulmonary arterial pressure of 55/30mmHg (mean pressure of 40mmHg).

Finally, she died of right ventricular failure ac-celerated for progression of the pulmonary hyper-tension.

The most impressive findings in the histopathol-ogical investigation was closely resembled one to primary pulmonary hypertension.

The correlation between primary pulmonary hypertension and collagen disease including of Sjö-gren's syndrome has been discussed.