Adult Still's Disease (Still's Disease)

Adult Still’s Disease – Treatment Options

Introduction
Adult Still’s disease (ASD) is a rare systemic inflammatory disorder of unknown etiology, characterized by high spiking fevers, evanescent salmon-pink rash, arthritis/arthralgia, sore throat, lymphadenopathy, hepatosplenomegaly, and elevated inflammatory markers. It is considered an autoinflammatory condition, and its course may be monocyclic (self-limiting), polycyclic (relapsing), or chronic (persistent arthritis). Treatment is guided by disease severity and organ involvement, aiming to control systemic inflammation, relieve symptoms, prevent long-term joint damage, and reduce the risk of complications such as macrophage activation syndrome (MAS).

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1. Non-Steroidal Anti-Inflammatory Drugs (NSAIDs)

• Used in mild cases or as initial therapy.

• Provide symptomatic relief of fever, arthralgia, and inflammation.

• Commonly used agents: naproxen, indomethacin, ibuprofen.

• Often insufficient as monotherapy, especially in moderate-to-severe disease.

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2. Glucocorticoids

• Mainstay for acute and systemic disease flares.

• Prednisone/prednisolone:

  • Initial dose: 0.5–1 mg/kg/day orally, tapered gradually based on clinical response.

• Intravenous methylprednisolone pulses may be required in life-threatening complications or macrophage activation syndrome.

• Effective in controlling fever, systemic symptoms, and joint inflammation.

• Long-term use is limited by side effects (osteoporosis, diabetes, hypertension, infections).

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3. Conventional Disease-Modifying Anti-Rheumatic Drugs (DMARDs)

• Indicated for steroid-sparing effect or refractory arthritis.

• Methotrexate: Most widely used (15–25 mg weekly), often combined with folic acid.

• Leflunomide, azathioprine, cyclosporine – alternatives for patients intolerant or resistant to methotrexate.

• Help achieve long-term disease control and reduce steroid dependence.

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4. Biologic Therapies (Targeted Agents)

• Used in patients with refractory or severe systemic disease.

• IL-1 inhibitors:

  • Anakinra (100 mg SC daily), canakinumab, rilonacept – highly effective for systemic features and MAS prevention.

• IL-6 inhibitors:

  • Tocilizumab, sarilumab – beneficial for both systemic and articular manifestations.

• TNF inhibitors (etanercept, infliximab, adalimumab): Less effective compared to IL-1/IL-6 inhibitors, reserved for selected cases with arthritis-predominant disease.

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5. Management of Macrophage Activation Syndrome (MAS)

• MAS is a potentially fatal complication characterized by severe hyperinflammation, cytopenias, coagulopathy, and liver dysfunction.

• Requires high-dose IV corticosteroids (methylprednisolone pulses).

• Cyclosporine or IL-1 blockade (anakinra) may be added in refractory cases.

• Aggressive supportive care in an intensive care setting is often necessary.

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6. Supportive and Long-Term Care

• Analgesics and physical therapy to preserve joint function and mobility.

• Bone protection strategies (calcium, vitamin D, bisphosphonates) during prolonged steroid use.

• Vaccinations and infection prevention in immunosuppressed patients.

• Regular monitoring of blood counts, liver function, and inflammatory markers to guide therapy.