Biological treatment in adult-onset Still's disease

Best Pract Res Clin Rheumatol. 2012 Aug;26(4):477-87. doi: 10.1016/j.berh.2012.07.002.

Abstract

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder that is characterised by high spiking fever, arthritis or arthralgia, and evanescent rash. Many other systemic manifestations may occur. Pathogenesis of AOSD remains partially unknown but a major role has been recently attributed to pro-inflammatory Th1 cytokines, including tumour necrosis factor-α (TNF-α), interleukin (IL)-1, IL-6 and IL-18. Despite limited evidence, mainly based on observational studies and the extrapolation to AOSD of the results of a few controlled studies that have been conducted in children with systemic juvenile idiopathic arthritis, biological agents represent a major therapeutic advances for patients with AOSD refractory to conventional treatment or presenting life-threatening manifestations. Both IL-1 and IL-6 blockade may be more effective than TNF-α blockers. Although debatable, therapeutic strategies are proposed.

Publication types

  • Review

MeSH terms

  • Adalimumab
  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Antirheumatic Agents / therapeutic use*
  • Cytokines / therapeutic use
  • Etanercept
  • Exanthema / complications
  • Exanthema / pathology
  • Humans
  • Immunoglobulin G / therapeutic use
  • Infliximab
  • Interleukin 1 Receptor Antagonist Protein / therapeutic use
  • Interleukin-1 / antagonists & inhibitors
  • Interleukin-18 / antagonists & inhibitors
  • Interleukin-6 / antagonists & inhibitors
  • Methotrexate / therapeutic use
  • Rare Diseases / drug therapy*
  • Rare Diseases / etiology
  • Rare Diseases / pathology
  • Receptors, Tumor Necrosis Factor / therapeutic use
  • Still's Disease, Adult-Onset / drug therapy*
  • Still's Disease, Adult-Onset / etiology
  • Still's Disease, Adult-Onset / pathology
  • Tumor Necrosis Factor-alpha / antagonists & inhibitors

Substances

  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Humanized
  • Antirheumatic Agents
  • Cytokines
  • Immunoglobulin G
  • Interleukin 1 Receptor Antagonist Protein
  • Interleukin-1
  • Interleukin-18
  • Interleukin-6
  • Receptors, Tumor Necrosis Factor
  • Tumor Necrosis Factor-alpha
  • Infliximab
  • Adalimumab
  • Etanercept
  • Methotrexate