JOURNAL OF HOSPITAL GENERAL MEDICINE
Online ISSN : 2436-018X
Case Reports
A case of IgG4-related disease with periaortitis/ periarteritis that progressed to unstable angina pectoris during multidisciplinary treatment
Toru SuzukiKazuyoshi KanekoWataru KatawakiKenji TakahashiMasato HoshikawaHiroya Ohtake
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ジャーナル フリー

2022 年 4 巻 5 号 p. 243-250

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IgG4-related disease (IgG4-RD) is an autoimmune disorder in which lymphocytes and IgG4- positive plasma cells infiltrate organs and tissues throughout the body that may occur asynchronously, presenting various clinical symptoms. Steroid therapy is known to be effective. Vascular lesions in IgG4-RD include periaortitis/arteritis and aneurysms. We encountered a case of IgG4-RD-related autoimmune pancreatitis (AIP) and interstitial nephritis complicated by periaortitis in the abdominal aorta and periarteritis in the coronary artery. Steroid therapy improved the AIP and interstitial nephritis, and IgG4 levels showed gradual improvement to normal. But, abdominal periaortitis was only slightly improved on periodic ultrasound examinations (US). Furthermore, when it came to the coronary arteries, the patient eventually developed unstable angina despite multidisciplinary treatment. Therefore, steroid therapy may be limited in the treatment of IgG4-RD-related periaortitis/periarteritis, and it is important that extensive, careful follow-up be done with periodic evaluation of periaortitis/periarteritis, even if amelioration is seen in other organs.
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© 2022 JAPAN SOCIETY OF HOSPITAL GENERAL MEDICINE

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