Table 1.

Patient characteristics and response to 12 weeks of ACTH gel treatment

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.