A 15 years old caucasian girl was diagnosed with systemic juvenile idiopathic arthritis (sJIA) by the age of four and was initially treated with nonsteroidal anti-inflammatory drugs and oral corticosteroids (prednisolone 1mg/kg/day). Although there was some clinical improvement, the disease progressed with 1–2 articular and systemic exacerbations a year and, in the beginning of the year 2007, when she was 7 years old, she started weekly methotrexate (10mg/m2).

Despite methotrexate treatment escalation (up to 25mg/m2/week subcutaneously) the patient had persistent disease activity and developed severe left coxitis (Fig. 1) in the beginning of 2009, which did not respond to ultrasound guided joint injection with triamcinolone hexacetonide. Due to the persistence of arthritis and elevated inflammatory markers she was started on Anakinra (1mg/kg/day) in July 2009.

Fig. 1.

Anteroposterior (Panel A) and frog-leg lateral (Panel B) radiographs of the pelvis showing left hip joint space narrowing, subchondral cysts of the left femoral head and acetabular sclerosis (*).

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Since then there has been sustained improvement with resolution of clinical symptoms, including complete imagiological regression of coxitis (Fig. 2), which allowed discontinuation of methotrexate and corticosteroids. The patient is in clinical remission on medication with Anakinra 100mg/day (2–2.5mg/kg/day) since 2011.

Fig. 2.

Anteroposterior (Panel A) and frog-leg lateral (Panel B) radiographs of the pelvis showing normal left hip joint space with discrete acetabular sclerosis.

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sJIA is an autoinflammatory rheumathologic disease that accounts for 5–10% of all patients classified as JIA.1 Hip involvement in sJIA is relatively common and is a cause of significant functional impairment and a marker of poor prognosis.2,3 A significant number of patients with sJIA has persistent disease despite the treatments used.

Anakinra is a recombinant form of human IL-1 receptor antagonist (IL-1Ra) that is recommended as first line disease-modifying therapy in sJIA selected patients and in refractory disease.4 In this case, the authors report the therapeutic success with Anakinra in a patient with refractory systemic and articular disease, emphasizing the regression of structural damage of the hip joint, which is rarely reported in these cases.

The authors declare that there are no conflicts of interest.