Adult-onset Still's disease: can recent advances in our understanding of its pathogenesis lead to targeted therapy?
Adult-onset Still's disease is a rare systemic inflammatory disease of unknown etiology, characterized by daily high, spiking fevers, evanescent rash, and arthritis. There is no single diagnostic test for adult-onset Still's disease; rather, the diagnosis is based on clinical criteria and necessitates the exclusion of infectious, neoplastic, and other 'autoimmune' diseases. Proinflammatory cytokines such as interleukin (IL)-1, IL-6, and IL-18, interferon-gamma, tumor necrosis factor, and macrophage colony-stimulating factor are elevated in patients with adult-onset Still's disease and are thought to have a major role in the pathogenesis of the disease. Treatment consists of nonsteroidal anti-inflammatory drugs, corticosteroids, immunosuppressants (methotrexate, gold, azathioprine, leflunomide, cyclosporin, and cyclophosphamide), intravenous immunoglobulin, and cytokine (tumor necrosis factor, IL-1 and IL-6) inhibitors. Recent advances in basic immunology have enhanced our ability to hinder the pathogenic mechanisms associated with adult-onset Still's disease and have led to a paradigm shift where targeted treatments have an increasingly important role.
Efthimiou
Petros
P
Division of Allergy, Immunology, and Rheumatology, New Jersey Medical School, and Rheumatology Clinic, University Hospital, New Jersey Medical School, University of Medicine and Dentistry of New Jersey, Newark, NJ 07103, USA. efthimpv@umdnj.edu
Kontzias
Apostolos
A
Ward
Carla M
CM
Ogden
Neeta S
NS
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D016207:Cytokines / Q000187:drug effects / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016706:Still's Disease, Adult-Onset / Q000188:drug therapy* / Q000503:physiopathology*
D000305:Adrenal Cortex Hormones; D018501:Antirheumatic Agents; D016207:Cytokines; D007166:Immunosuppressive Agents