Clinical Features of 10 Patients with IgG4-related Retroperitoneal Fibrosis
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概要
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Objective To elucidate the clinical characteristics of IgG4-related retroperitoneal fibrosis (RF). Methods IgG4-related RF was diagnosed when all of the following three criteria were fulfilled: retroperitoneal soft tissue masses surrounding the aorta and/or adjacent tissues, elevation of the serum IgG4 levels, and abundant infiltration of IgG4-positive plasma cells in at least one organ or site. Ten patients were diagnosed as having IgG4-related RF. Results The mean age at diagnosis was 70.1 years, and the male-to-female ratio was 1:0.6. Only two patients had initial symptoms predominantly related to RF (back pain and edema of the lower extremities), while the remaining eight patients reported initial symptoms due to associated diseases. On laboratory examination, a severe inflammatory reaction was observed in one patient. Elevation of the levels of serum IgG and IgE, eosinophilia and positivity of antinuclear antibodies were detected in seven, five, two and seven patients, respectively. The retroperitoneal masses were detected primarily in the left renal hilus in four patients, in the periaortic region in five patients and in both regions in one patient. Hydronephrosis was present in five patients. The histological diagnosis was confirmed in the retroperitoneal masses (resection, n=1 biopsy, n=2) and extraretroperitoneal lesions (n=7). Twenty-four other IgG4-related diseases were found to be associated with IgG4-related RF in nine patients (autoimmune pancreatitis (n=2), sialadenitis (n=4), dacryoadenitis (n=5), lymphadenopathy (n=9), pulmonary pseudotumor (n=1) and pituitary pseudotumor (n=1)). Seven patients underwent steroid therapy, all of whom responded well and showed no instances relapse. Conclusion IgG4-related RF has several clinical characteristic features. Our diagnostic criteria may be helpful in obtaining a correct diagnosis.
著者
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Fujiwara Junko
Department Of Endoscopy Tokyo Metropolitan Komagome Hospital
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Kamisawa Terumi
Department Of Internal Medicine Tokyo Metropolitan Komagome Hospital
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Kuwata Go
Department Of Internal Medicine Municipal And County Hospital Of Miyazaki Medical Association
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Momma Kumiko
Department Of Endoscopy Tokyo Metropolitan Komagome General Hospital
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Arakawa Takeo
Department Of Internal Medicine Tokyo Metropolitan Komagome Hospital
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Koizumi Koichi
Department Of Gastroenterology Tokyo Metropolitan Komagome Hospital
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Setoguchi Keigo
Department Of Allergy And Immunological Diseases Tokyo Metropolitan Komagome Hospital
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Shinohara Mitsuru
Department Of Urology Tokyo Metropolitan Komagome Hospital
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Egashira Hideto
Department Of Internal Medicine Tokyo Metropolitan Komagome Hospital
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Tabata Taku
Department Of Internal Medicine Tokyo Metropolitan Komagome Hospital
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Fujiwara Takashi
Department Of Electrical And Electronic Systems Engineering Graduate School Of Information Science A
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Chiba Kazuro
Department Of Internal Medicine Tokyo Metropolitan Komagome Hospital
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Setoguchi Keigo
Department of Rheumatology, Tokyo Metropolitan Komagome Hospital, Japan
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Hara Seiichi
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Japan
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Kuruma Sawako
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Japan
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Koizumi Satomi
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Japan
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Kuruma Sawako
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital
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Fujiwara Takashi
Department of Cardiology, Iwakuni Clinical Center
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Shinohara Mitsuru
Department of Urology, Tokyo Metropolitan Komagome Hospital, Japan
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