Table 1.
Patient and diagnosis | Age (years) | Extramuscular organ involvement | Previous therapy | Concomitant medications | Treatment response |
---|---|---|---|---|---|
1 DM | 45 | Skin | Prednisone 40 mg/day, methotrexate 15 mg/week, azathioprine 150 mg/day, cyclosporine 200 mg/day, IVIG 2 g/kg/month, rituximab 375 mg/m2, 2 doses 1 week apart, then every 3 months for 12 months | Prednisone 20 mg/day, IVIG 2 g/kg/month | Resolution of skin involvement; improved proximal leg strength; able to get out of chair and walk independently |
2 JDM | 25 | Skin, calcinosis | Prednisone 100 mg/day, IVIG 2 g/kg/month, cyclosporine 200 mg/day, azathioprine 200 mg/day, rituximab 375 mg/m2, 2 doses 1 week apart, then every 3 months for 12 months, MMF 1500 mg BID, pulse methylprednisolone 1 g/week for 8 weeks | IVIG 2 g/kg/month, azathioprine 200 mg/day, cyclosporine 200 mg/day | Increased leg strength; improvement in skin involvement; return to independent ambulation and work |
3 DM | 43 | Dysphagia, joint pains | Prednisone 60 mg/day, pulse methylprednisolone 1 g/day for 5 days, IVIG 50 g/month to 2 g/kg/month | Prednisone 30 mg/day, IVIG 2 g/kg/month | Improved strength; decreased pain; able to hold her baby; independence with ADLs |
4 PM | 55 | Extraocular muscles | Prednisone 60 mg/day, IVIG 2 g/kg/month, MMF 1500 mg BID | MMF 1500 mg BID | Improved proximal muscle strength; no improvement in diplopia; able to participate in activities with grandchildren |
5 PM | 68 | None | Pulse methylprednisolone 1 g/week for 8 weeks, pulse cyclophosphamide 1 g/m2/month for 6 months, IVIG 2 g/kg/month, MMF 1500 mg BID, rituximab 375 mg/m2, 2 doses 1 week apart, then every 3 months for 12 months | MMF 1500 mg BID | Improved strength; return to independent ambulation |
Abbreviations: ADLs, activities of daily living; BID, twice daily; DM, dermatomyositis; IVIG, intravenous immunoglobulins; JDM, juvenile dermatomyositis; MMF, mycophenolate mofetil; PM, polymyositis.