Adult-onset Still's disease-pathogenesis, clinical manifestations, and new treatment options

Ann Med. 2015 Feb;47(1):6-14. doi: 10.3109/07853890.2014.971052. Epub 2015 Jan 22.

Abstract

Adult-onset Still's disease (AOSD), a systemic inflammatory disorder, is often considered a part of the spectrum of the better-known systemic-onset juvenile idiopathic arthritis, with later age onset. The diagnosis is primarily clinical and necessitates the exclusion of a wide range of mimicking disorders. AOSD is a heterogeneous entity, usually presenting with high fever, arthralgia, skin rash, lymphadenopathy, and hepatosplenomegaly accompanied by systemic manifestations. The diagnosis is clinical and empirical, where patients are required to meet inclusion and exclusion criteria with negative immunoserological results. There are no clear-cut diagnostic radiological or laboratory signs. Complications of AOSD include transient pulmonary hypertension, macrophage activation syndrome, diffuse alveolar hemorrhage, thrombotic thrombocytopenic purpura and amyloidosis. Common laboratory abnormalities include neutrophilic leukocytosis, abnormal liver function tests, and elevated acute-phase reactants (ESR, CRP, ferritin). Treatment consists of anti-inflammatory medications. Non-steroidal anti-inflammatory drugs have limited efficacy, and corticosteroid therapy and disease-modifying anti-rheumatic drugs are usually required. Recent advances have revealed a pivotal role of proinflammatory cytokines such as tumor necrosis factor-α (TNF-α), interleukin (IL)-1, IL-6, IL-8, and IL-18 in disease pathogenesis, giving rise to the development of novel targeted therapies aiming at optimal disease control. The review aims to summarize recent advances in pathophysiology and potential therapeutic strategies in AOSD.

Keywords: Adult-onset Still's disease; FUO (fever of unknown origin); retrospective study.

Publication types

  • Review

MeSH terms

  • Adult
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
  • Antirheumatic Agents / therapeutic use
  • Cytokines / therapeutic use
  • Humans
  • Still's Disease, Adult-Onset* / complications
  • Still's Disease, Adult-Onset* / physiopathology
  • Still's Disease, Adult-Onset* / therapy

Substances

  • Anti-Inflammatory Agents, Non-Steroidal
  • Antirheumatic Agents
  • Cytokines