抄録
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.