From the Guidelines
Adult Still's disease treatment should prioritize a step-wise approach, starting with NSAIDs, followed by corticosteroids, and then biologic agents, with IL-1 inhibitors and IL-6 inhibitors being particularly effective for steroid-dependent or resistant cases. This approach is supported by the most recent evidence from the 2024 EULAR/PRES recommendations for the diagnosis and management of Still's disease 1.
Treatment Approach
The treatment of Adult Still's disease (AOSD) typically involves:
- NSAIDs like naproxen (500mg twice daily) or ibuprofen (600-800mg three times daily) for mild cases
- Corticosteroids such as prednisone (0.5-1mg/kg/day) for symptoms that persist
- Disease-modifying antirheumatic drugs (DMARDs) like methotrexate (15-25mg weekly) for steroid-dependent or resistant cases
- Biologic agents, including IL-1 inhibitors (anakinra 100mg daily subcutaneously) and IL-6 inhibitors (tocilizumab 8mg/kg IV every 2-4 weeks), which have shown particular effectiveness in managing the disease
Disease Patterns and Diagnosis
AOSD can follow three patterns: monocyclic (single episode), polycyclic (recurring episodes), or chronic (persistent symptoms). Diagnosis requires excluding other conditions and is based on clinical criteria, including:
- Fever
- Rash
- Arthritis
- Elevated inflammatory markers
- Negative tests for rheumatoid factor and antinuclear antibodies
Monitoring and Management
Regular monitoring of symptoms, inflammatory markers, and medication side effects is essential for managing AOSD effectively. Severe or life-threatening complications, such as macrophage activation syndrome (MAS) or lung disease, may develop at any point during the disease course and should be actively screened and monitored 1.
Complications Management
For patients with severe or life-threatening complications:
- MAS treatment must include high-dose glucocorticoids, and treatments including anakinra, ciclosporin, and/or IFNγ inhibitors should be considered as part of initial therapy
- Lung disease should be actively screened by searching for clinical symptoms and pulmonary function tests, and investigated by high-resolution CT scan in any patients with clinical symptoms
- The presence of risk factors for Still’s lung disease or the development of Still’s lung disease should not be considered as a contraindication to IL-1 or IL-6 inhibitors 1
From the Research
Overview of Adult Still's Disease
- Adult Still's disease (AOSD) is a rare systemic inflammatory condition characterized by spiking fever, macular rash, and polyarthritis 2.
- The clinical spectrum of AOSD ranges from self-limiting forms with mild symptoms to life-threatening cases 3.
- The pathogenesis of AOSD is only partially known, but pro-inflammatory cytokines such as tumor necrosis factor (TNF)-α, interleukin (IL)-1, IL-6, IL-18, and IFN-γ seem to play a major role in this disorder 4.
Treatment Options for AOSD
- Non-steroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids are effective in controlling clinical manifestations in the majority of AOSD patients 3.
- Conventional disease-modifying anti-rheumatic drugs (DMARDs) can be effective in some severe and steroid-dependent cases of AOSD 3, 2.
- Biologic agents, such as IL-1 and IL-6 blockers, are effective in treating systemic and articular inflammation of AOSD patients 3, 5, 4, 6.
- IL-1 blockade has an excellent safety profile and represents the first choice of biologic treatment in AOSD patients 3.
- Tocilizumab, a monoclonal anti-IL-6 antibody, may be a promising treatment option for AOSD patients refractory to conventional DMARDs and other biologic agents 6.
Biologic Agents in AOSD Treatment
- IL-1 inhibitors are more effective for systemic manifestations, while IL-6 inhibitors are effective for both joint disease and systemic features 5.
- Anti-TNF agents may be useful for patients with the pure rheumatoid subgroup of AOSD 5.
- Clinical response to biologic agents must be obtained within 2 to 3 months, and biologics with different mechanisms of action may be required for non-responders 5.