Primary biliary cirrhosis associated with mixed type autoimmune hemolytic anemia and sicca syndrome: a case report and review of literature

Am J Gastroenterol. 1997 Sep;92(9):1547-9.

Abstract

A case presenting with an unusual association of primary biliary cirrhosis and mixed type autoimmune hemolytic anemia plus sicca syndrome is described. The 49-yr-old female primary biliary cirrhosis patient had a confirmed sicca syndrome and presented with jaundice and life-threatening anemia. Laboratory tests revealed positive Coombs' test with coexisting cold and warm autoantibodies. She was successfully treated by blood transfusion with packed red cells lacking any red cell antigens corresponding to serum alloantibodies and pulse methylprednisolone therapy. The patient remained stable under maintenance treatment using oral steroids and ursodeoxycholic acid. This case is probably the first reported showing an association between primary biliary cirrhosis and mixed type autoimmune hemolytic anemia plus sicca syndrome and was probably induced by heterogenous and complicated autoimmune reactions.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Administration, Oral
  • Anemia, Hemolytic, Autoimmune / complications*
  • Anemia, Hemolytic, Autoimmune / immunology
  • Anemia, Hemolytic, Autoimmune / therapy
  • Anti-Inflammatory Agents / administration & dosage
  • Anti-Inflammatory Agents / therapeutic use
  • Antigens / immunology
  • Autoantibodies / analysis
  • Autoimmune Diseases / complications*
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / therapy
  • Blood Transfusion
  • Cholagogues and Choleretics / administration & dosage
  • Cholagogues and Choleretics / therapeutic use
  • Erythrocyte Transfusion
  • Erythrocytes / immunology
  • Female
  • Glucocorticoids / administration & dosage
  • Glucocorticoids / therapeutic use
  • Humans
  • Isoantibodies / immunology
  • Jaundice / etiology
  • Liver Cirrhosis, Biliary / complications*
  • Liver Cirrhosis, Biliary / immunology
  • Liver Cirrhosis, Biliary / therapy
  • Methylprednisolone / administration & dosage
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / immunology
  • Sjogren's Syndrome / therapy
  • Treatment Outcome
  • Ursodeoxycholic Acid / administration & dosage
  • Ursodeoxycholic Acid / therapeutic use

Substances

  • Anti-Inflammatory Agents
  • Antigens
  • Autoantibodies
  • Cholagogues and Choleretics
  • Glucocorticoids
  • Isoantibodies
  • Ursodeoxycholic Acid
  • Methylprednisolone