What is the recommended initial glucocorticoid (steroid) dose and tapering schedule for an adult with newly diagnosed adult‑onset Still’s disease?

Glucocorticoid Dosing for Adult-Onset Still's Disease

For newly diagnosed adult-onset Still's disease with high disease activity, initiate high-dose glucocorticoids at ≥1 mg/kg/day of prednisone equivalent (or intravenous methylprednisolone followed by oral prednisone), combined with IL-1 or IL-6 inhibitors as first-line therapy, and begin tapering glucocorticoids as soon as fever resolves and active joints decrease by 50%. 1

Initial Dosing Strategy

High Disease Activity Presentation

• High-dose glucocorticoids are defined as ≥1 mg/kg/day of prednisone equivalent in adults (maximum typically 60-80 mg/day). 1
• Intravenous methylprednisolone pulse therapy followed by oral prednisone is appropriate for severe presentations with high spiking fever, widespread polyarthritis, pericarditis, or impending macrophage activation syndrome. 1
• Real-world data from China shows that 47.2% of patients required 30-60 mg/day of prednisone (approximately 0.5-1 mg/kg) to achieve clinical remission. 2

Moderate Disease Activity

• Low-to-intermediate dose glucocorticoids (≤0.1 mg/kg/day prednisone equivalent) combined with IL-1 or IL-6 inhibitors can be used for patients without high disease activity features. 1

Critical Context: Glucocorticoids Are No Longer First-Line Monotherapy

The treatment paradigm has fundamentally shifted—IL-1 inhibitors (anakinra) or IL-6 receptor inhibitors (tocilizumab) are now recommended as first-line therapy alongside glucocorticoids, not as rescue therapy. 1, 3, 4

• Historical data showed that 76-95% of patients responded to glucocorticoids, but 88-89% eventually required additional immunosuppressive therapy. 1, 3
• NSAID monotherapy controls disease in only 7-15% of patients. 1, 3
• Maintenance prednisone at 10-15 mg/day for arthritic symptoms can lead to severe joint destruction despite steroid treatment. 1, 4

Tapering Schedule

When to Begin Tapering

• Start glucocorticoid tapering as soon as the first intermediate target is reached: no fever and 50% reduction in active joints. 1
• This represents a major shift from older recommendations that required 4-6 weeks of high-dose therapy before tapering. 1

Tapering Timeline

• Progressive glucocorticoid tapering should occur over several months while maintaining biologic therapy. 1
• The ultimate goal is clinically inactive disease (CID) off glucocorticoids by 6 months. 1, 3
• Low-dose glucocorticoids are defined as ≤0.1 mg/kg/day prednisone equivalent in adults. 1

Treatment Targets and Monitoring

Sequential Targets

• Day 7: Resolution of fever and CRP reduction >50%. 4
• Week 4: No fever, active joint count reduced by 50%, normal CRP, and physician/patient global assessment <20 on 0-100 VAS. 4
• Month 3: Clinically inactive disease. 1
• Month 6: Clinically inactive disease off glucocorticoids. 1, 3

Definition of Clinically Inactive Disease

• No fever, no rash, no active arthritis, no serositis, normal inflammatory markers, and physician global assessment indicating inactive disease. 1

Special Considerations

Impending Macrophage Activation Syndrome

• Use high-dose anakinra (>100 mg twice daily in adults) combined with high-dose glucocorticoids for patients with impending or active MAS (elevated liver function tests and/or markedly elevated ferritin). 1, 3, 4

Predictors of Poor Response

• Baseline white blood cell count ≥13,050/μL predicts poor treatment response to initial glucocorticoid therapy (60% poor outcome vs. 23.5% with lower WBC). 5
• Patients with skin rash, pericarditis, splenomegaly, or delayed diagnosis require higher glucocorticoid doses for remission. 2

Critical Pitfalls to Avoid

• Never rely on glucocorticoids alone as long-term therapy—85-93% of patients will require biologic therapy. 3, 4
• Do not delay biologic therapy beyond 3 months of symptom onset—early initiation prevents persistent chronic disease course. 3, 4
• Avoid prolonged high-dose glucocorticoid monotherapy (>16 weeks)—this increases toxicity without improving outcomes. 1
• Do not maintain prednisone at 10-15 mg/day for chronic arthritis—this leads to joint destruction without adequate disease control. 1, 4

Algorithm Summary

1. Confirm AOSD diagnosis (fever ≥39°C, transient rash, arthralgia/arthritis, elevated ferritin, neutrophilic leukocytosis). 4
2. Assess disease activity: High (widespread polyarthritis, pericarditis, VAS >6-7/10) vs. moderate. 1
3. Initiate combination therapy immediately:
   • High disease activity: High-dose glucocorticoids (≥1 mg/kg/day) + IL-1 or IL-6 inhibitor 1
   • Moderate disease activity: Low-dose glucocorticoids (≤0.1 mg/kg/day) + IL-1 or IL-6 inhibitor 1
4. Begin tapering when fever resolves and joints improve by 50%. 1
5. Target CID off glucocorticoids by 6 months. 1, 3
6. If no response by 3 months, rotate between IL-1 and IL-6 inhibitors while continuing glucocorticoid taper. 1