What is the next step in managing a patient with Adult-Onset Still's Disease (AOSD) who responded to initial treatment with prednisolone (corticosteroid) 1mg/kg?

Management of Adult-Onset Still's Disease After Initial Prednisolone Response

The next step in managing this patient with Adult-Onset Still's Disease (AOSD) who responded to initial prednisolone treatment should be a slow taper of prednisolone over 4-6 months while considering the addition of a steroid-sparing agent such as methotrexate.

Initial Assessment and Response Evaluation

• The patient's positive response to prednisolone 1mg/kg indicates successful initial treatment, which aligns with standard practice as most AOSD patients (76-95%) respond to corticosteroid therapy 1
• Complete resolution of fever and erythematous rash suggests good disease control, but careful monitoring is needed during the tapering phase 1

Corticosteroid Tapering Approach

• Begin tapering prednisolone slowly over a total period of 4-6 months to minimize risk of relapse 1
• The tapering schedule should be individualized based on clinical response, with careful monitoring for any signs of disease recurrence 2
• Prednisolone doses above 1 mg/kg/day are rarely needed if the patient has already shown good response 1

Steroid-Sparing Strategies

• Consider adding methotrexate as a steroid-sparing agent, particularly if:

  • The patient requires prolonged steroid therapy
  • There are concerns about steroid-related adverse effects
  • The patient has prominent articular symptoms 1, 3

• Methotrexate has shown effectiveness in AOSD management:

  • 85% of patients on methotrexate were able to taper prednisolone dose 1
  • 78% of patients with refractory AOSD achieved complete remission with low-dose methotrexate 1
  • Daily prednisone intake decreased by 69% in patients on methotrexate 1

Monitoring During Treatment

• Schedule follow-up visits every 4-6 weeks during the initial tapering phase 1
• Monitor for:
  • Recurrence of fever, rash, or arthralgia 3
  • Laboratory markers including CRP, ferritin, and liver function tests 3
  • Potential steroid-related adverse effects 1
  • If using methotrexate, monitor for potential side effects including mild rises in liver enzymes 1

Alternative Therapeutic Options

• If the patient shows signs of relapse during tapering or develops steroid dependence, consider:
  • Increasing the methotrexate dose (if already initiated) 1
  • Adding or switching to biologic agents, particularly IL-1 or IL-6 inhibitors such as tocilizumab 4, 5
  • Tocilizumab has shown efficacy in refractory AOSD with significant decrease in prednisolone dose requirements 4

Special Considerations

• Be vigilant for potential complications of AOSD, particularly macrophage activation syndrome (MAS), which can develop even during treatment 5, 3
• If MAS is suspected (persistent fever, cytopenia, hyperferritinemia, liver dysfunction), prompt intensification of immunosuppressive therapy is required 5
• For patients with prominent systemic symptoms, IL-1 inhibitors may be more effective, while those with predominant articular symptoms may respond better to IL-6 inhibitors 3

Long-term Management

• Some patients may achieve sustained remission and eventually discontinue all medications 4
• Others may require long-term maintenance therapy with a steroid-sparing agent 1, 3
• Regular follow-up is essential even after apparent remission, as disease flares can occur 3