J Korean Rheum Assoc.  2005 Dec;12(4):335-340.

Refractory Cases of Adult Onset Still's Disease Successfully Treated with TNF-alpha Blocker

• Park I
• Park MC
• Choi ST
• Park YB
• Lee SK

Abstract

Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology characterized by spiking fever, evanescent salmon-colored rash, polyarthritis and leukocytosis. The diagnosis of AOSD remains a challenge to clinicians and requires a high index of suspicion because of its rarity and nonspecific symptoms. Although the etiology and pathogenesis of AOSD is not elucidated clearly, the pathogenetic role of inflammatory cytokines, such as interleukin (IL)-6, IL-8, IL-18 and tumor necrosis factor (TNF)-alpha were suggested and the correlations of their levels with disease activity were also reported. These results raise a possibility that the blocking of these cytokines may provide a therapeutic benefit in controlling disease activity and relieving the symptoms of AOSD. Recently, we experienced two cases of AOSD, who were refractory to the treatment with high dose glucocorticoid and immunosuppressive agents. Both were treated with TNF- blockers and experienced remissions thereafter. These experiences might support the use of biologic agents in refractory AOSD.

Keyword

Adult onset Still's disease (AOSD); Tumor necrosis factor (TNF)-alpha; TNF-alpha blocker

MeSH Terms

Adult*
Arthritis
Biological Factors
Cytokines
Diagnosis
Exanthema
Fever
Humans
Immunosuppressive Agents
Interleukin-18
Interleukin-8
Interleukins
Leukocytosis
Still's Disease, Adult-Onset*
Tumor Necrosis Factor-alpha*
Biological Factors
Cytokines
Immunosuppressive Agents
Interleukin-18
Interleukin-8
Interleukins
Tumor Necrosis Factor-alpha