From the Guidelines
For a patient with dermatomyositis who remains with active disease despite combination therapy with IVIG, rituximab, tocilizumab (Actemra), prednisone, hydroxychloroquine (Plaquenil), and mycophenolate mofetil (CellCept), I recommend considering the addition of cyclophosphamide to the current regimen, as suggested by the expert consensus for severe disease management 1.
Given the patient's severe autoimmune disease and ongoing active disease despite the current combination therapy, it is essential to consider intensifying the treatment regimen. The expert consensus recommends considering the addition of intravenous cyclophosphamide for patients with severe disease, such as major organ involvement or extensive ulcerative skin disease 1.
Before adding new medications, ensure optimal dosing of current therapies:
- IVIG at 2g/kg monthly
- rituximab at 1g every 6 months
- tocilizumab 8mg/kg monthly
- prednisone at the lowest effective dose
- hydroxychloroquine 200-400mg daily
- mycophenolate mofetil 2-3g daily in divided doses. Consider tapering prednisone if possible to minimize long-term side effects.
Key points to consider:
- The patient has been on the current regimen for 6 months with no infections or cytopenias, suggesting they may tolerate additional therapy.
- Regular monitoring of complete blood count, liver function, lipid profile, and screening for opportunistic infections remains essential with this intensive immunosuppression approach.
- The expert consensus also suggests that B cell depletion therapy (rituximab) can be considered as an adjunctive therapy for those with refractory disease, and the patient is already on this therapy 1.
- Ongoing skin disease reflects ongoing systemic disease and therefore should be treated by increasing systemic immunosuppression, which supports the consideration of adding cyclophosphamide to the current regimen 1.
From the Research
Current Treatment Regimen
The patient is currently on a combination of biologics and immunosuppression, including:
- IVIG
- Rituximab
- Actemra
- Prednisone
- Plaquenil
- Cellcept This regimen has been in place for 6 months, with no reported infections or cytopenia, but the patient still has active disease.
Treatment Options
Considering the patient's current regimen and disease activity, the following options may be considered:
- Continuing the current regimen, as some studies suggest that rituximab can take time to show its full effect 2, 3
- Adding or substituting other immunosuppressive agents, such as abatacept, which has shown promise in treating dermatomyositis 4
- Adjusting the dosing of current medications, taking into account factors such as the patient's weight, age, and other health characteristics 5
Efficacy of Rituximab
Rituximab has been shown to be effective in treating dermatomyositis, with response rates ranging from 72.5% to 75% in some studies 2, 3. However, the optimal regimen and dosing schedule for rituximab in this context are still unclear.
Future Directions
Further studies are needed to determine the best course of treatment for patients with refractory dermatomyositis, including the use of combination therapies and the optimization of dosing regimens 6. Additionally, the development of predictive models and clinical decision software may help guide treatment decisions and improve patient outcomes 5.
Key Considerations
When considering changes to the patient's treatment regimen, the following factors should be taken into account: