How can we help patients with refractory chronic graft versus host disease- single centre experience

Chronic graft-versus-host disease (cGVHD) is a major cause of morbidity and mortality in long-term survivors of allogeneic haematopoietic stem cell transplantation (alloHSCT). Ocular involvement as well as dermal sclerosis, joint contractures and pathological changes in oral cavity are often refractory to treatment. This kind of patients require complex aggressive immunosuppressive therapy. We are still waiting for drugs against cGVHD, characterized by decreased infectious complications, encouraging efficacy and rare and reversible side effects. We describe eight patients who developed extensive chronic graft versus host disease with eye involvement after alloHSCT. All had ocular manifestations, which were refractory to the first and second line of systemic immunosuppressive therapy. All patients responded to the topical cyclosporine therapy, but clinical improvement was seen only since the fifth month of starting treatment. Topical cyclosporine was well tolerated. Other four patients with sclerodermoid type of skin changes, refractory to second line systemic immunosuppressive therapy, were treated with clofazimine. Clofazimine is a drug used to treat leprosy. Because of its anti-inflammatory effects, clofazimine is used also as a second or third line therapy for various skin disorders including: pyoderma gangrenosum, lupus erythematosus, palmoplantar pustulosis and chronic graft versus host disease. All patients,who received clofazimine due to dermal sclerosis, joint contractures and oral manifestations, achieved partial or complete responses and were able to reduce other immunosuppressive drugs. Clofazimine was generally well tolerated.