収縮性心膜炎を来したIgG4関連心膜炎の1例

A Case of IgG4-related Pericarditis with Constrictive Pericarditis

要旨　症例は73歳男性．1カ月以上続く労作時呼吸困難感および両下肢浮腫を自覚し精査加療目的で受診．胸部CTにて心胸膜肥厚を，PET-CTでは肥厚した心胸膜に異常集積を認めた．また，心エコーでは収縮性心膜炎所見を認めた．胸膜生検ではIgG4陽性形質細胞の浸潤が認められた．血清IgG4値は高値であり，心膜にはPET-CTで胸膜と同程度に異常集積を認めたことから，収縮性心膜炎を来したIgG4関連心膜炎と診断した．ステロイド治療を開始，速やかに自覚症状の改善を認め，その後ステロイド減量でも再燃所見を認めず，経過は良好である．

　IgG4関連疾患について多くの症例報告がなされているなかで，心膜炎合併例の報告は少ないが，臓器病変の好発部位とされる膵臓や胆管・涙腺・唾液腺・後腹膜腔などの臓器病変を認めない例や心膜炎単独症例の報告も認めている．心膜炎の鑑別疾患の中にIgG4関連疾患も加える必要があると考えられた．

　A 73-year-old man came to our hospital in chief complaint for exertional dyspnea and both leg edema for more than one month. We checked the pericardium and pleural thickening findings in chest CT, and findings of the abnormal accumulation to the thickened pericardium and pleura in PET-CT. And more, echocardiography findings showed evidence of constrictive pericarditis. We tried the pleural biopsy, and this findings show IgG4-positive plasma cells infiltration. We diagnosed IgG4-related pericarditis with constrictive pericarditis. His symptom improved shortly after that we started steroid treatment. Afterward, we reduced the steroid administration, He is doing well without any signs of recurrence.

　There are few reports of IgG4-related pericarditis with constrictive pericarditis,

　While many cases are described in many organ systems such as pancreas, bile duct, lacrimal gland, salivary gland and/or retroperitoneum. This is rare case of IgG4-related pericarditis with constrictive pericarditis. IgG4-related diseases should be included in the differential diagnosis of pericardial diseases.