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Japanese Journal of Allergology 2006-Jul

[A case of systemic sclerosis and Sjogren's syndrome with cardiac tamponade due to steroid-responsive pericarditis].

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Takeo Sato
Shin-ya Oominami
Takamitsu Souma
Kiyofumi Hagiwara
Shouko Kobayashi
Osamu Akiyama

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Abstracto

A 75-year-old woman was admitted to our hospital because of abnormal lung shadow and necrosis of the left feet. She had a history of Raynaud's phenomenon from her twenties. On admission, she was diagnosed as having diffuse systemic sclerosis (SSc) and Sjögren's syndrome (SjS) because of scleroderma, interstitial pneumonia (IP), positive result of anti-Scl-70 and SS-A antibody, sicca, decreased tear excretion, and dysfunction of salivary glands. Seventy days after amputation of her left leg, she presented with edema, hypoxemia, chest discomfort, and fever. Blood test revealed inflammation and cardiac echography revealed pericardial effusion with a collapse sign of right atrium, thereby leading to the diagnosis of cardiac tamponade. After starting the daily dose of 20 mg of prednisolone, the pericardial effusion and cardiac tamponade sign disappeared. Pericarditis is seen in half of patients with SSc and rarely with SjS, and is usually asymptomatic. Pericarditis due to SSc has been reported unresponsive to steroid therapy, but several cases of steroid responsive pericarditis due to SSc or SjS have been reported. Clinically, they shared inflammatory responses and the presence of IP in the cases of SSc, which will be important when considering the pathogenesis and treatment of pericarditis due to SSc or SjS.

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