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Henoch-Schönlein purpura and Crohn's disease in a family

Daiana Cassater1, Giovanni Gambaro, Antonia Fabris

  • 1School of Medicine, University of Verona, Verona, Italy.

Journal of Nephrology
|July 29, 2006
PubMed

Insights

A teenage girl developed Henoch-Schonlein purpura (HSP) after appendectomy, with a family history of HSP and Crohn's disease. Genetic analysis suggests HSP and Crohn's may share a background, but not through known class II HLA types.

Area of Science:

  • Immunology
  • Gastroenterology
  • Genetics

Background:

  • Henoch-Schonlein purpura (HSP) is a systemic vasculitis often preceded by infection.
  • Crohn's disease is a chronic inflammatory bowel disease with a complex etiology.
  • Familial aggregation of HSP and inflammatory bowel disease suggests a potential genetic link.

Observation:

  • A 16-year-old female presented with terminal segmental ileitis and subsequently developed HSP post-appendectomy.
  • The patient's family history includes HSP in her brother and Crohn's disease in her mother.
  • Human leukocyte antigen (HLA) typing revealed the DRB1*11 allele in the patient, associated with HSP, but not in her brother.

Findings:

  • The patient's HLA DRB1*11 allele was present, but her brother, who had HSP, was negative for this allele.
  • Neither the patient nor other family members carried the HLA DRB1*01 allele, previously linked to both HSP and Crohn's disease.
  • This case supports a non-random association between Crohn's disease and HSP.

Implications:

  • The findings suggest a shared genetic background for concomitant Crohn's disease and HSP.
  • Class II HLA phenotypes do not appear to be the primary genetic factor linking these conditions.
  • Further research is needed to identify other potential genetic contributors to the co-occurrence of HSP and Crohn's disease.

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