Background/Purpose:

Adult onset Still’s disease (AOSD) is a rare disorder of unknown etiology and systemic inflammatory disorder characterized by spiking fever, arthritis, evanescent rash and leukocytosis. Up to 80% of patients with AOSD were controlled with non-steroidal anti-inflammatory drugs, corticosteroid, disease-modifying antirheumatic drugs (DMARDs), particularly methotrexate. Some AOSD patients do not response to conventional DMARDs or biologic therapy including tumor necrosis factor-α inhibitor. Abatacept, cytotoxic lymphocyte antigen 4 (CTLA4) immunoglobulin, is a biologic agent which blocks the co-stimulation of T-lymphocytes and currently is approved for use in rheumatoid arthritis. Therefore we report the use of abatacept in the treatment of five patients with AOSD manifested by severe polyarthritis unresponsive to treatment with corticosteroid, methotrexate, TNF-α inhibitors or tocilizumab.

Subjects & Methods:

Four female and one male patients with the diagnosis of AOSD according to the Yamagushi criteria of 1992 were treated with abatacept. Median disease duration of those patients was 5.7 years. They do not response to glucocorticoid, disease-modifying antirheumatic drugs including methotrexate, and TNF-α inhibitor treatment before abatacept. Two patients of them were treated with tocillizumab but, we discontinued the agents due to the uneffectiveness or infusion reaction. After administering two 500mg infusions of abatacept 2 weeks apart initially, abatacept was administered every 4 week. Clinical efficacy and adverse events, changes in the values of routine laboratory parameters were evaluated at each visit.

Results:

The major symptom is the polyarthritis in four patients and fever and myalgia in one.One patient improved remarkably in tender joint counts and swollen joints (17à6, 9à 0) after 6 months treatment with abatacept. Another two patients showed no change in joint counts but, there is the improvement of systemic symptoms. Also in one patient with systemic symptom, fever and myalgia resolved after abatacept treatment.ESR and CRP were declined in three patients, but did not changed in the other two patients. AOSD patients have received abatacept treatment for between seven and thirteen months. Throughout this period all patients have no serious adverse event including infection and some benefits from this treatment, with improvement in their clinical symptoms, joint counts, and serological disease activity.

Conclusion:

Abatacept was administered in five patients with AOSD failing traditional DMARDs, anti-TNF-α and anti-IL-6 therapies, with some beneficial outcome. These our data suggest the potential therapeutic benefit of abatacept treatment in AOSD.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/treatment-of-refractory-adult-onset-stills-disease-with-abatacept-report-of-five-cases/