Anakinra in Patients with Treatment-Resistant Adult-Onset Still's Disease

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Anakinra in Patients with Treatment-Resistant Adult-Onset Still's Disease:

Four Case Reports with Serial Cytokine Measurements and a Review of the

Literature

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Objective

To determine the efficacy of the interleukin (IL)-1-receptor antagonist

(IL-1RA) anakinra in patients with adult-onset Still's disease (AOSD)

refractory to standard treatments such as glucocorticosteroids (GC),

immunosuppressive drugs, and tumor necrosis factor (TNF)-antagonists; to

verify disease remission objectively by serial cytokine measurements; and to

review the current literature on anakinra for this indication.

Methods

Four patients with AOSD-2 with acute flares of the chronic form of the

disease and 2 with intermittent disease-were treated with prednisolone and

methotrexate. One was also treated with several other immunosuppressive

drugs including etanercept and infliximab. One patient had life-threatening

symptoms (toxic megacolon, pneumonitis, disseminated intravascular

coagulation) despite high-dose prednisolone. Treatment with anakinra 100

mg/d subcutaneously was initiated. White blood cells (WBC), C-reactive

protein (CRP) levels, erythrocyte sedimentation rate (ESR), liver enzymes,

ferritin levels, and serum cytokines were analyzed. The current literature

on the efficacy of anakinra for AOSD is reviewed.

Results

Patients with chronic AOSD quickly responded to anakinra treatment (1 day to

3 days). GC could be tapered. ESR, CRP, WBC, ferritin, and liver enzymes

returned to normal. Serum cytokine measurements revealed moderately elevated

IL-1B levels and highly elevated IL-18 levels in active disease, which

normalized with anakinra. TNF-a and IL-6 were moderately elevated only in

the 2 patients with chronic AOSD. In the literature, 17 similar cases have

been reported to date.

Conclusions

Anakinra is effective in treatment-resistant and in life-threatening AOSD.

IL-18 serum levels, in addition to CRP, ESR, liver enzymes, ferritin, and

WBC, may be helpful in assessing disease activity and response to treatment.