Inflammatory Pseudotumors in Multiple Organs Associated with Elevated Serum IgG4 Level: Recovery by Only a Small Replacement Dose of Steroid
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- Tsuboi Hiroto
- Department of Allergy and Immunological Diseases, Tokyo Metropolitan Komagome Hospital
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- Inokuma Shigeko
- Department of Allergy and Immunological Diseases, Tokyo Metropolitan Komagome Hospital
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- Setoguchi Keigo
- Department of Allergy and Immunological Diseases, Tokyo Metropolitan Komagome Hospital
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- Shuji Sumitomo
- Department of Allergy and Immunological Diseases, Tokyo Metropolitan Komagome Hospital
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- Hagino Noboru
- Department of Allergy and Immunological Diseases, Tokyo Metropolitan Komagome Hospital
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- Tanaka Yoshiaki
- Department of Allergy and Immunological Diseases, Tokyo Metropolitan Komagome Hospital
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- Yoshida Nozomi
- Department of Allergy and Immunological Diseases, Tokyo Metropolitan Komagome Hospital
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- Hishima Tsunekazu
- Department of Pathology, Tokyo Metropolitan Komagome Hospital
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- Kamisawa Terumi
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital
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抄録
A 62-year-old man developed a fever, fatigue, anorexia and arthralgia. Central hypocorticoidism and central hypothyroidism were observed, and a low serum antidiuretic hormon level without symptoms of diabetes insipidus, as well. Images showed swelling of pituitary stalk, mediastinal and hilar lymphnodes and pancreas, pulmonary infiltrates and retroperitoneal mass. Serum CRP level was 20.6 mg/dL, and IgG4 level was 292 mg/dL. Lung biopsy revealed pseudotumor containing IgG4-positive plasmacytes, and obliterative vasculitis both in arterioles and venules. These features were similar to those of reported IgG4-related autoimmune disease. However, replacement steroid therapy for hypocorticoidism brought about almost complete recovery except that diabetes insipidus got apparent. This is the first report on the efficacy of only a small dose of steroid, and on features of pituitary stalk involvement and central hypocorcicoidism.<br>
収録刊行物
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- Internal Medicine
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Internal Medicine 47 (12), 1139-1142, 2008
一般社団法人 日本内科学会