First line therapy
Includes :
- Corticosteriods (often with alternate day prednisone - 1 mg/kg/day divided BID)
- Cyclosporin (Calcineurin inhibitor)
Corticosteroids:
- Individuals often need prolonged course of corticosteroids lasting 3-12 months.
- Corticosteriods are an important part of first line therapy.
- Multiple earlier trials have compared steroids alone to combinations of steroids plus cyclophosphamide, mycophenolate mofetil (MMF), procarbazine, anti-thymocyte globulin, and azathioprine did not show improved outcomes.
- Many patients develop long-term complications of corticosteroids:
- Cushingoid appearance
- Bone mineral density loss
- Diabetes
- Cataracts
Use of immunosuppression increases the long-term risk of infection.
Antibiotic prophylaxis should be given for encapsulated organisms (e.g. Penicillin VK) and Pneumocystis carini (jiroveci) (e.g. Septra) should be initiated with therapy.
Second Line Therapy
Attempts to decrease corticosteroids because of side effects or because cGvHD is refractory, lead to a number of second-line agents for cGvHD. There is no accepted second line therapy for cGvHD.
Second-line therapy for steroid-refractory cGvHD may include:
- Mycophenolate Mofetil (MMF)
- Sirolimus
- Hydroxychloroquine
- Rituximab
- Chimeric monoclonal antibody against protein CD29
- Protein CD20 is found on the surface of B cells
- Used in the treatment of many lymphomas, leukemias and transplant rejection
- Thalidomide
- Pentostatin
- Extracorporeal Photopheresis
- Lymphocytes are treated with psoralen and UV light
Skin involvement of chronic GVHD can respond to psoralen and UV light therapy or topical tacrolimus
