Can Rinvoq (upadacitinib) help a patient with dermatomyositis who is already taking IVIG (Intravenous Immunoglobulin)?

Rinvoq (Upadacitinib) for Dermatomyositis in Patients Already on IVIG

Rinvoq (upadacitinib) is not currently recommended as an established treatment for dermatomyositis in patients already receiving IVIG therapy, as there is insufficient evidence supporting its use in this specific scenario.

Current Treatment Approach for Dermatomyositis

First-Line Therapies

• Corticosteroids: High-dose corticosteroids (oral or IV) remain the cornerstone of initial treatment for dermatomyositis 1
  • Prednisone 0.5-1 mg/kg/day is typically recommended as first-line therapy
  • For severe cases, IV methylprednisolone 1-2 mg/kg may be considered

Second-Line/Adjunctive Therapies

• Methotrexate: Recommended as first-line steroid-sparing agent 1

  • Typically started at 15-20 mg/m²/week (maximum 40 mg/week) 2

• IVIG (Intravenous Immunoglobulin): Strong evidence supports its use

  • Particularly effective for skin manifestations of dermatomyositis 3
  • The ProDERM study demonstrated significant improvement in both muscle and skin symptoms with IVIG treatment 3, 4
  • Typical dosing: 2 g/kg divided over 2-5 days, repeated every 4 weeks 1
  • IVIG has been shown to allow for steroid sparing in dermatomyositis patients 5

Additional Treatment Options

• Other immunosuppressants:

  • Azathioprine (1-2.5 mg/kg/day) 2
  • Mycophenolate mofetil (0.5-1g twice daily) 2
  • Cyclosporine A 2

• Anti-TNF therapies: May be considered in refractory cases 2

  • Infliximab or adalimumab are preferred over etanercept

Evaluation of Rinvoq (Upadacitinib) for Dermatomyositis

Upadacitinib (Rinvoq) is a JAK inhibitor that has been approved for several autoimmune conditions. However:

1. Lack of evidence: There are no published clinical trials or guidelines supporting the use of upadacitinib specifically for dermatomyositis

2. Current guidelines: The most recent myositis management guidelines do not mention JAK inhibitors like upadacitinib as established treatment options 1

3. Treatment algorithm: For patients with inadequate response to steroids and first-line immunosuppressants, the recommended progression is:

   • Increase or optimize IVIG therapy
   • Consider rituximab for refractory cases 1
   • Consider TNF-α or IL-6 antagonists in selected cases 1

Management Recommendations for Patients on IVIG for Dermatomyositis

For patients already receiving IVIG therapy for dermatomyositis:

1. Optimize current therapy:

   • Ensure optimal IVIG dosing (2 g/kg every 4 weeks) 3, 4
   • Evaluate steroid dosing and consider appropriate tapering schedule
   • Assess compliance and response to current immunosuppressive agents

2. If inadequate response to IVIG:

   • Consider increasing IVIG frequency or dose
   • Add or optimize steroid-sparing agents (methotrexate, azathioprine, mycophenolate mofetil)
   • Consider rituximab for refractory cases 1
   • TNF-α inhibitors may be considered for refractory cases 2

3. Monitoring response:

   • Regular assessment of muscle strength
   • Serial creatine kinase (CK) measurements
   • Evaluation of skin manifestations using validated tools like CDASI
   • Assessment of functional status improvement

Caveats and Considerations

• Novel therapies: While JAK inhibitors like upadacitinib show promise in various autoimmune conditions, their role in dermatomyositis remains investigational

• Individual assessment: Factors such as disease severity, comorbidities, and previous treatment responses should be considered

• Multidisciplinary approach: Management should involve rheumatology, dermatology, and possibly neurology expertise

• Research participation: Patients with refractory disease may benefit from participation in clinical trials evaluating novel therapies

In conclusion, while upadacitinib (Rinvoq) may have theoretical benefits in dermatomyositis based on its mechanism of action, there is currently insufficient evidence to recommend its use in patients already receiving IVIG therapy. Optimizing current evidence-based treatments remains the preferred approach.