Cytomegalovirus-related necrotising vasculitis mimicking Henoch-Schönlein syndrome

Clin Exp Rheumatol. 2014 May-Jun;32(3 Suppl 82):S73-5. Epub 2014 May 16.

Abstract

Viral vasculitides have been previously reported in the literature, the role of infections in their pathogenesis ranging from direct cause to trigger event. Here we report the case of a 3-year-old immunocompetent girl who developed a systemic vasculitis leading to ileal perforation, mimicking a full blown picture of Henoch-Schönlein purpura. High dosage steroid treatment was started, with good response. The anatomopathological examination of the resected gastrointestinal tract showed features of necrotising vasculitis and cytomegalovirus (CMV)-related inclusion bodies in the endothelial cells, with direct correlation to vascular damage. The causative role of viral infection was revealed by the presence of CMV DNA in patient's blood and positive IgG titer against the virus. Steroid therapy was then tapered: the patient achieved clinical remission, which still persists after a six-months follow-up. Our report suggests that CMV vasculitis is probably more frequent than previously thought, even in immunocompetent patients, with a protean clinical presentation, mimicking other types of vasculitides.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Cytomegalovirus / isolation & purification
  • Cytomegalovirus Infections / complications*
  • Cytomegalovirus Infections / physiopathology
  • Cytomegalovirus Infections / virology
  • Diagnosis, Differential
  • Digestive System Surgical Procedures / methods*
  • Drug Administration Schedule
  • Female
  • Glucocorticoids / administration & dosage
  • Humans
  • IgA Vasculitis / diagnosis*
  • Ileal Diseases / diagnosis
  • Ileal Diseases / etiology*
  • Ileal Diseases / physiopathology
  • Ileal Diseases / surgery
  • Ileum* / pathology
  • Ileum* / surgery
  • Intestinal Perforation / diagnosis
  • Intestinal Perforation / etiology*
  • Intestinal Perforation / physiopathology
  • Intestinal Perforation / surgery
  • Methylprednisolone / administration & dosage*
  • Monitoring, Immunologic
  • Remission Induction
  • Systemic Vasculitis* / diagnosis
  • Systemic Vasculitis* / drug therapy
  • Systemic Vasculitis* / etiology
  • Systemic Vasculitis* / physiopathology
  • Treatment Outcome

Substances

  • Glucocorticoids
  • Methylprednisolone