Mediators of Inflammation (Jan 2013)

Biological Treatments: New Weapons in the Management of Monogenic Autoinflammatory Disorders

  • Antonio Vitale,
  • Donato Rigante,
  • Orso Maria Lucherini,
  • Francesco Caso,
  • Isabella Muscari,
  • Flora Magnotti,
  • Maria Giuseppina Brizi,
  • Susanna Guerrini,
  • Maria Patti,
  • Leonardo Punzi,
  • Mauro Galeazzi,
  • Luca Cantarini

DOI
https://doi.org/10.1155/2013/939847
Journal volume & issue
Vol. 2013

Abstract

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Treatment of monogenic autoinflammatory disorders, an expanding group of hereditary diseases characterized by apparently unprovoked recurrent episodes of inflammation, without high-titre autoantibodies or antigen-specific T cells, has been revolutionized by the discovery that several of these conditions are caused by mutations in proteins involved in the mechanisms of innate immune response, including components of the inflammasome, cytokine receptors, receptor antagonists, and oversecretion of a network of proinflammatory molecules. Aim of this review is to synthesize the current experience and the most recent evidences about the therapeutic approach with biologic drugs in pediatric and adult patients with monogenic autoinflammatory disorders.