[Case of rapid progressive interstitial pneumonia associated with primary Sjogren syndrome]

Yoshihisa Naruse, Naoki Inui, Hideki Yasui, Masato Karayama, Sawa Yamazaki, Eriko Muramatsu, Tomohiro Uto, Satoru Morita, Tomoyoshi Tsuchiya, Yutaka Nakano, Noriyuki Enomoto, Shiro Imokawa, Takafumi Suda, Kingo Chida
Nihon Kokyūki Gakkai Zasshi, the Journal of the Japanese Respiratory Society 2006, 44 (10): 721-6
A 72-year-old woman with a dry cough and dyspnea on exertion was admitted to our hospital. A chest radiograph showed reticular opacities and volume loss in both lower lung fields. She was troubled with xerostomia and her laboratory test showed positive reaction for anti SS-A and SS-B antibody. Labial biopsy led to a diagnosis of primary Sjogren's syndrome (pSjS). Lung biopsy specimens obtained by video-assisted thoracoscopic surgery (VATS) revealed interstitial pneumonia. On the sixth postoperative day, hypoxemia acutely worsened and her chest radiograph showed widespread diffuse ground-glass attenuation. A diagnosis of acute exacerbation was made, and steroid and immunosuppressive therapy was started. In spite of intensive therapy, she died due to respiratory failure. We report a rare case of interstitial pneumonia with pSjS resulting in acute exacerbation.

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