From the Guidelines
The treatment for Adult-Onset Still's Disease (AOSD) should prioritize the use of IL-1 or IL-6 inhibitors as early as possible, given their high efficacy and favorable safety profile, as recommended by the most recent guidelines 1.
Treatment Approach
The treatment approach for AOSD typically involves a stepwise strategy based on disease severity.
- Initial therapy may include nonsteroidal anti-inflammatory drugs (NSAIDs) like naproxen or ibuprofen for mild cases.
- Glucocorticoids such as prednisone are often necessary for more significant symptoms, particularly during flares.
- For patients requiring steroid-sparing agents or with refractory disease, conventional disease-modifying antirheumatic drugs (DMARDs) like methotrexate may be added.
Biologic Agents
In cases resistant to these treatments, biologic agents targeting specific inflammatory pathways have shown effectiveness.
- IL-1 inhibitors (anakinra, canakinumab) and IL-6 inhibitors (tocilizumab) are preferred due to their high efficacy in controlling disease activity and limiting exposure to glucocorticoids 1.
- TNF inhibitors (such as etanercept) may also be considered, although their use is less favored compared to IL-1 and IL-6 inhibitors in the context of AOSD.
Treatment Duration and Monitoring
Treatment duration varies based on clinical response, with some patients requiring long-term therapy while others may achieve remission.
- Regular monitoring for medication side effects and disease activity is essential, with treatment adjustments made according to clinical response.
- The choice of therapy depends on disease manifestations, with systemic symptoms often responding better to IL-1 or IL-6 inhibitors, reflecting the central role of these cytokines in AOSD pathogenesis 1.
Recent Guidelines
Recent guidelines from EULAR/PRES emphasize the importance of early initiation of IL-1 or IL-6 inhibitors and the use of a treat-to-target approach, aiming for clinically inactive disease without glucocorticoids 1.
- These guidelines also highlight the need for patient education and counseling to manage the disease effectively and recognize signs of relapse.
From the Research
Treatment Options for Adult-Onset Still's Disease
The treatment for Adult-Onset Still's Disease (AOSD) typically involves a combination of medications to manage symptoms and prevent complications. Some of the treatment options include:
- Non-steroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids, which are effective in controlling clinical manifestations in the majority of AOSD patients 2
- Conventional disease-modifying anti-rheumatic drugs (DMARDs), which can be effective in some severe and steroid-dependent cases of AOSD 2
- Biologic agents, such as anakinra, which blocks interleukin-1 (IL-1) and has been shown to be effective in treating systemic and articular inflammation of AOSD patients 2, 3, 4
- Interleukin-6 (IL-6) blockers, which have also been shown to be effective in treating AOSD 2
Biologic Agents for AOSD
Biologic agents, such as anakinra, have been shown to be effective in treating AOSD, particularly in patients with severe and recalcitrant clinical manifestations. Some of the benefits of biologic agents include:
- High rate of complete remission 3
- Corticosteroid-sparing effect, which can reduce the risk of long-term side effects 3
- Favorable safety profile 3, 4
- Effective in treating life-threatening complications, such as macrophage activation syndrome 5, 6
Management of Complications
AOSD can be associated with several complications, including reactive hemophagocytic lymphohistiocytosis, coagulation disorders, fulminant hepatitis, cardiac or pulmonary complications, and amyloid A amyloidosis. The management of these complications typically involves:
- Early recognition and prompt treatment 5
- High-dose corticosteroids and supportive measures as first-line treatment 5
- Combination with IL-1 or IL-6 blockers in case of inadequate response 5
- Cyclosporine A and etoposide as alternative treatment options 5
- Plasma exchange in case of thrombotic microangiopathy 5