What is the next step for a patient on Cellcept (Mycophenolate Mofetil), Rituximab, high-dose Intravenous Immunoglobulin (IVIG), Prednisone, and Plaquenil (Hydroxychloroquine) with persistent active inflammation, neutrophilia, and hypocomplementemia?

From the Guidelines

For a patient on Cellcept (Mycophenolate Mofetil), Rituximab, high-dose Intravenous Immunoglobulin (IVIG), Prednisone, and Plaquenil (Hydroxychloroquine) with persistent active inflammation, neutrophilia, and hypocomplementemia, I recommend considering plasmapheresis as the next step, as suggested by the American Society of Clinical Oncology clinical practice guideline 1. The patient's current regimen includes multiple immunosuppressants, but the persistence of active inflammation, neutrophilia, and hypocomplementemia indicates a need for additional therapeutic interventions.

• The current treatment regimen has not adequately controlled the patient's immune response, as evidenced by the ongoing inflammation and laboratory abnormalities.
• According to the guideline, plasmapheresis may be offered in cases of severe compromise or when symptoms and laboratory findings do not improve or worsen after 4 to 6 weeks of treatment 1.
• Other options, such as adding methotrexate, azathioprine, or adjusting the dose of mycophenolate mofetil, could also be considered, but plasmapheresis is a more direct approach to removing circulating immune complexes and autoantibodies contributing to the patient's condition.
• Regular monitoring of the patient's condition, including complete blood count, liver and kidney function, and complement levels, is crucial to assess the effectiveness of the added therapy and adjust the treatment plan as needed.
• It is also important to consider the potential risks and benefits of plasmapheresis, including the risk of infection, bleeding, and allergic reactions, and to discuss these with the patient before initiating treatment.

From the FDA Drug Label

If after a reasonable period of time there is a lack of satisfactory clinical response, PredniSONE should be discontinued and the patient transferred to other appropriate therapy

The next step for a patient on Cellcept (Mycophenolate Mofetil), Rituximab, high-dose Intravenous Immunoglobulin (IVIG), Prednisone, and Plaquenil (Hydroxychloroquine) with persistent active inflammation, neutrophilia, and hypocomplementemia is to discontinue Prednisone and transfer the patient to other appropriate therapy 2.

From the Research

Next Steps for Patient with Persistent Active Inflammation

The patient is currently on a regimen of Cellcept (Mycophenolate Mofetil), Rituximab, high-dose Intravenous Immunoglobulin (IVIG), Prednisone, and Plaquenil (Hydroxychloroquine) with persistent active inflammation, neutrophilia, and hypocomplementemia. The next steps for this patient could be:

• Re-evaluating the patient's response to the current biologic therapy and determining whether it represents a primary nonresponse or a secondary loss of response due to inadequate drug levels and/or antibody formation to the drug 3
• Considering strategies such as dose escalation or addition of an immunosuppressant to address secondary loss of response 3
• Identifying patients with ongoing histological evidence of inflammation despite clinical remission and considering a lower threshold for intervention to reduce the risk of adverse outcomes 4
• Exploring advanced therapies such as cell therapy and tissue engineering approaches to control chronic inflammation 5
• Combining pharmacological treatment with individualized and supervised exercise to reduce disease activity and improve muscle performance 6

Potential Treatment Options

Potential treatment options for the patient could include:

• Changing to a therapy targeting other mechanisms of immune modulation 3
• Using biologic agents such as rituximab, which has shown some evidence of effect in patients with certain myositis-specific autoantibodies 6
• Combining immunosuppressive treatment with exercise to reduce disease activity and improve muscle performance 6
• Using immunomodulator agents such as 5-aminosalicylates, corticosteroids, and antibiotics to control mucosal inflammation and heal mucosal ulcerations 7
• Considering a top-down approach, initiating biologic therapy at diagnosis, or using a combination therapy with a biologic and an immunomodulator 7