IgG4-related disease coexisting with autoimmune haemolytic anaemia

BMJ Case Rep. 2018 Jul 6:2018:bcr2018224814. doi: 10.1136/bcr-2018-224814.

Abstract

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.

Keywords: biliary intervention; endoscopy; pancreas and biliary tract.

Publication types

  • Case Reports

MeSH terms

  • Aged, 80 and over
  • Anemia, Hemolytic, Autoimmune / complications*
  • Anti-Inflammatory Agents / administration & dosage
  • Bile Ducts, Intrahepatic / diagnostic imaging
  • Bile Ducts, Intrahepatic / pathology
  • Cholangiopancreatography, Endoscopic Retrograde
  • Cholangitis / blood*
  • Cholangitis / complications*
  • Cholangitis / drug therapy
  • Exanthema / etiology
  • Humans
  • Hypergammaglobulinemia / complications*
  • Immunoglobulin G / blood*
  • Male
  • Methylprednisolone / administration & dosage
  • Pancreas / diagnostic imaging
  • Pancreas / pathology
  • Tomography, X-Ray Computed

Substances

  • Anti-Inflammatory Agents
  • Immunoglobulin G
  • Methylprednisolone