Prednisolone-responsive primary sclerosing cholangitis with autoimmune hemolytic anemia: a case report and review of the
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CASE REPORT
Prednisolone‑responsive primary sclerosing cholangitis with autoimmune hemolytic anemia: a case report and review of the literature Toshihiro Kawaguchi1 · Teruko Arinaga‑Hino1 · Satoshi Morishige2 · Shinji Mizuochi3 · Mitsuhiko Abe4 · Kazuya Kunitake1 · Tomoya Sano1 · Keisuke Amano1 · Reiichiro Kuwahara1 · Tatsuya Ide1 · Koji Nagafuji2 · Takuji Torimura1 Received: 23 June 2020 / Accepted: 24 September 2020 © Japanese Society of Gastroenterology 2020
Abstract We report a case of primary sclerosing cholangitis (PSC) with autoimmune hemolytic anemia (AIHA). A 47-year-old woman was diagnosed with PSC. One year later, she was admitted to our hospital for jaundice and fatigue. Magnetic resonance cholangiopancreatography (MRCP) showed worsening of the biliary stricture, and rapidly progressive anemia developed simultaneously. Based on the various laboratory findings, she was diagnosed with AIHA. The administration of prednisolone improved not only the anemia but also the biliary stricture. This case is impactful, as there are few case reports of PSC with AIHA. In addition, we were able to observe the changes in imaging findings using MRCP over time. Keywords Primary sclerosing cholangitis · Autoimmune hemolytic anemia · Prednisolone Abbreviations AIH Autoimmune hepatitis AIHA Autoimmune hemolytic anemia AIP Autoimmune pancreatitis CT Computed tomography ERCP Endoscopic retrograde cholangiopancreatography EUS Endoscopic ultrasonography IDUS Intraductal ultrasonography IgG4 Immunoglobulin G4 IgG4-SC IgG4 sclerosing cholangitis IHBD Intrahepatic bile duct MRCP Magnetic resonance cholangiopancreatography * Toshihiro Kawaguchi [email protected]‑u.ac.jp 1
Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, 67, Asahi‑machi, Kurume, Fukuoka 830‑0011, Japan
2
Division of Hematology and Oncology, Department of Medicine, Kurume University School of Medicine, Kurume, Japan
3
Department of Pathology, Kurume University School of Medicine, Kurume, Japan
4
Department of Gastroenterology, Nishinihon Hospital, Kumamoto, Japan
PSC Primary sclerosing cholangitis PSL Prednisolone UDCA Ursodeoxycholic acid
Introduction Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease characterized by diffuse inflammation and fibrosis involving the intrahepatic and extrahepatic biliary ducts. The cause of PSC is unknown, and there is no effective treatment other than liver transplantation. The disease has a poor prognosis, and it often progresses to cirrhosis, cholangiocarcinoma, and liver failure. Autoimmune hemolytic anemia (AIHA) is characterized by the production of antibodies to autologous red blood cells. AIHA is a very rare disorder, with an estimated incidence of one to three cases per 100,000 persons per year. However, AIHA must be considered in the differential diagnosis of hemolytic anemia, especially if the patient has an autoimmune disease, a lymphoproliferative disorder, or a viral or mycoplasma infection [1]. Concom
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