BMJ Case Reports 2018; doi:10.1136/bcr-2018-224814
  • Findings that shed new light on the possible pathogenesis of a disease or an adverse effect

IgG4-related disease coexisting with autoimmune haemolytic anaemia

Open Access
  1. Chang-Youh Tsai1,2
  1. 1Division of Allergy, Immunology and Rheumatology, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan
  2. 2Faculty of Medicine, National Yang-Ming University, Taipei, Taiwan
  1. Correspondence to Professor Chang-Youh Tsai, cytsai{at}
  • Accepted 11 May 2018
  • Published 6 July 2018


An 85-year-old man presented with a pale appearance and generalised pruritic papules. Laboratory investigations disclosed eosinophilia, autoimmune haemolytic anaemia, mixed hyperbilirubinaemia, cholestasis and elevated serum IgG4 levels. Abdominal sonography and CT showed progressive dilatation of biliary trees, with diffuse pancreatic enlargement and a subtle capsule-like low-density rim around the pancreatic head and body. Endoscopic retrograde cholangiopancreatography found no stone-related biliary obstruction, while endoscopic transpapillary biopsy demonstrated chronic inflammation only. Nevertheless, the diagnosis of IgG4-related disease with coexisting autoimmune haemolytic anaemia was presumed. The clinical picture and laboratory abnormalities improved after administration of moderate dose of methylprednisolone.


  • Contributors K-CW took care of the patient, collected the pertinent data and wrote the manuscript. H-TL amended the manuscript, and C-YT verified all the data as well as supervised the whole study.

  • Funding Taipei Veterans General Hospital (V107D37-002-MY3-1).

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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