PReS-FINAL-2127: Sclerodermatous graft versus host disease

Results A 7 year old girl was referred to rheumatology 2 years after maternal HLA matched HSCT for acute lymphoblastic leukaemia. Mild cutaneous GVHD was noted post transplant. She subsequently presented with severe sclerodermatous changes on both lower legs. She had marked loss of range of movement (ROM) of her ankles, and the disease rapidly progressed to involve the skin of her thighs and left arm, with significant joint contractures. She was treated with methylprednisolone, methotrexate and physiotherapy (PT) with good effect: improved ROM, softening of the skin and no further progression. A 4 year old girl was referred to rheumatology 3 years after having 2 matched sibling HSCT for MHC class 2 deficiency. Very mild cutaneous GVHD had been noted post transplant. She presented with rapidly worsening contractures affecting her hands and lower limbs with diffuse swelling, erythema, thickness and tethering of skin and tendons. She was treated with PT, occupational therapy (OT) and splinting, together with prednisolone and methotrexate. Clinical improvement was maintained over the following year. A 6 year old girl with beta thalassaemia major was referred to rheumatology 2 years post HSCT. Buccal GVHD was noted 4 months post transplant, treated initially with steroids and ciclosporin followed by extracorporeal photophoresis. Despite some initial response to this there was a profound deterioration of hand function with severely restricted wrists and fingers bilaterally and reduced grip strength. She has been treated with an increased dose of prednisolone, the addition of methotrexate, PT and OT.