Enteroviruses in X-Linked Agammaglobulinemia: Update on Epidemiology and Therapy

J Allergy Clin Immunol Pract. 2016 Nov-Dec;4(6):1059-1065. doi: 10.1016/j.jaip.2015.12.015. Epub 2016 Feb 13.

Abstract

X-linked agammaglobulinemia (XLA) has been associated with a broad range of infections, but enteroviral disease represents one of the most damaging infections. The risk of enteroviral infection in XLA is lower now than in the setting of intramuscular immunoglobulin or in patients without immunoglobulin replacement, but the rate of infection has not declined significantly in the era of intravenous immunoglobulin replacement. Enteroviruses can cause inflammation of nearly every organ, but in XLA, infections often manifest as dermatomyositis or chronic meningoencephalitis. Difficulty and delay in recognizing symptoms and lack of specific therapy contribute to the poor outcomes. Furthermore, cerebrospinal fluid detection of enteroviruses is not very sensitive. Reluctance to perform brain biopsies can lead to significant delays. The other feature compromising outcomes is the lack of specific therapy. High-dose peripheral and intraventricular immunoglobulin have been used, but failure is still common. New antienteroviral drugs are in development and show promise for immunodeficient patients with life-threatening infections with enterovirus.

Keywords: Agammaglobulinemia; Coxsackievirus B5; Enterovirus; Meningoencephalitis; Pleconaril; Pocapavir; Polio.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Agammaglobulinemia* / diagnosis
  • Agammaglobulinemia* / drug therapy
  • Agammaglobulinemia* / epidemiology
  • Agammaglobulinemia* / virology
  • Antiviral Agents / therapeutic use
  • Brain / diagnostic imaging
  • Brain / virology
  • Child, Preschool
  • Enterovirus / genetics
  • Enterovirus / isolation & purification
  • Enterovirus Infections* / diagnosis
  • Enterovirus Infections* / drug therapy
  • Enterovirus Infections* / epidemiology
  • Enterovirus Infections* / virology
  • Genetic Diseases, X-Linked* / diagnosis
  • Genetic Diseases, X-Linked* / drug therapy
  • Genetic Diseases, X-Linked* / epidemiology
  • Genetic Diseases, X-Linked* / virology
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Phenyl Ethers / therapeutic use

Substances

  • Antiviral Agents
  • Phenyl Ethers
  • pocapavir

Supplementary concepts

  • Bruton type agammaglobulinemia