Management of Still's Syndrome after Methylprednisolone Pulse

Introduction to Steroid Management

The American College of Rheumatology recommends initiating oral prednisona at 0.5-1 mg/kg/day (maximum 60 mg/day) immediately after the pulse, with a gradual reduction over 3-6 months, and considering methotrexate as a steroid-sparing agent if maintenance treatment is required or if the disease is refractory 1, 2
The reduction of prednisona should be gradual during 3-6 months depending on the clinical response, with reductions of 5-10 mg weekly until reaching 20 mg/day, then more slowly 1
High doses of prednisona should be limited to 6 months for systemic disease refractory to NSAIDs, especially with persistent anemia, pericarditis, serositis, and elevated liver enzymes 3, 4

Methotrexate is the most effective steroid-sparing agent, with 88% response and 78% complete remission at low doses of 11.5 mg/week 4
Methotrexate allows for a reduction in daily prednisona intake by 69% (21.5 mg), and 42% of patients can discontinue prednisona completely 4
Polyarthritis is particularly susceptible to methotrexate treatment, while the effect on non-articular manifestations is less defined 4
The European League Against Rheumatism recommends considering methotrexate as a first-line steroid-sparing agent in patients with refractory disease 4

Consider IL-1 or IL-6 inhibitors as early as possible if the disease is refractory to steroids and methotrexate, as there is a therapeutic window of opportunity before 3 months of symptom onset 5
IL-1 and IL-6 inhibitors have a steroid-sparing effect and can prevent the persistent chronic course when initiated early 5
Before attempting biologic reduction, clinical inactivity should be maintained for 3-6 months without glucocorticoids 5

Monitor blood pressure and serum glucose before, during, and after each methylprednisolone infusion 1, 2, 6
Perform bone densitometry (DEXA) if ≥3 months of glucocorticoids are anticipated after pulse therapy 1, 2
Monitor renal function before the first infusion, especially in patients with comorbidities 2
Maintain a high index of suspicion for macrophage activation syndrome (MAS), which occurs in 15-20% of patients with Still's disease and can develop at any time during the course of the disease, even during biologic treatment when the disease is well-controlled 5, 2

Avoid underdosing when converting from pulse therapy to oral maintenance; consider patient-specific factors such as disease severity and comorbidities 1
Avoid chronic corticosteroids at low doses whenever possible; consider alternative maintenance therapies 1
Eight patients who received maintenance doses of 10-15 mg/day of prednisona developed severe joint destruction despite steroid treatment 3, 7
The American College of Rheumatology recommends considering high-dose glucocorticoids as the mainstay of treatment for MAS, administered as intravenous pulses of methylprednisolone (15-30 mg/kg/day, maximum dose 1 g/infusion) 5