Management of autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus

Autoimmun Rev. 2011 May;10(7):432-7. doi: 10.1016/j.autrev.2011.01.006. Epub 2011 Jan 18.

Abstract

Autoimmune neutropenia, caused by neutrophil-specific autoantibodies is a common phenomenon in autoimmune disorders such as Felty's syndrome and systemic lupus erythematosus. Felty's syndrome is associated with neutropenia and splenomegaly in seropositive rheumatoid arthritis which can be severe and with recurrent bacterial infections. Neutropenia is also common in systemic lupus erythematosus and it is included in the current systemic lupus classification criteria. The pathobiology of the autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus is complex, and it could be a major cause of morbidity and mortality due to increased risk of sepsis. Treatment should be individualized on the basis of patient's clinical situation, and prevention or treatment of the infection. Recombinant human granulocyte colony-stimulating factor is a safe and effective therapeutic modality in management of autoimmune neutropenia associated with Felty's syndrome and systemic lupus erythematosus, which stimulates neutrophil production. There is a slight increased risk of exacerbation of the underlying autoimmune disorder, and recombinant human granulocyte colony-stimulating factor dose and frequency should be adjusted at the lowest effective dose.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases / complications*
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / therapy*
  • Felty Syndrome / complications*
  • Felty Syndrome / physiopathology
  • Felty Syndrome / therapy*
  • Granulocyte Colony-Stimulating Factor / therapeutic use
  • Humans
  • Lupus Erythematosus, Systemic / complications*
  • Lupus Erythematosus, Systemic / physiopathology
  • Lupus Erythematosus, Systemic / therapy
  • Neutropenia / etiology*
  • Neutropenia / physiopathology
  • Neutropenia / therapy*
  • Recombinant Proteins

Substances

  • Recombinant Proteins
  • Granulocyte Colony-Stimulating Factor