Immunomodulatory drugs for adult-onset Still disease

People with severe or treatment-resistant adult-onset Still disease (eg those with chronic polyarthritis, recurrent episodes of systemic disease, or persistently abnormal investigation findings suggesting organ-related disease) may require immunomodulatory drug therapy (in addition to oral corticosteroids). Refer these people back to their specialist for review.

Methotrexate is commonly used initially for people with severe or treatment-resistant adult-onset Still disease. Biological disease-modifying antirheumatic drugs or cyclophosphamide may be considered.

If a patient with adult-onset Still disease is suspected of developing a serious or life-threatening complication (eg macrophage activation syndrome (MAS), disseminated intravascular coagulation), refer them urgently to hospital for specialist management.