Efficacy of cyclosporine to reduce steroids in patients with idiopathic pulmonary fibrosis before lung transplantation.

Lung transplantation today is considered an effective option for patients with severe idiopathic pulmonary fibrosis. The standard medical treatment for this disease consists of high-dose steroids alone or combined with other immunosuppressive drugs. Unfortunately, pretransplantation administration of steroids may jeopardize the healing of the airway anastomosis and cause other complications; therefore it is considered a relative contraindication to lung transplantation. For this reason we try to reduce the dose of prednisone to 15 to 20 mg/day or less before the transplantation, but this creates many difficulties and is sometimes impossible in severely ill patients. Therefore we used cyclosporine (4 to 7 mg/kg/day) in 10 patients who were receiving high-dose prednisone (> or = 50 mg/day) therapy, but who were otherwise suitable candidates for lung transplantation. In seven cases prednisone could be tapered to 20 mg/day or less, allowing acceptance in our program. These patients had a CRP score of 60 or more before entering our trial and remained stable at this level after conversion to cyclosporine. The 6-minute walk test showed a mild improvement in five cases (71.5%). Three patients underwent single lung transplantation; two patients are on our waiting list after 3.5 and 4 months of treatment with cyclosporine and prednisone (10 mg/day), and two patients died while awaiting a suitable organ 6 and 7.5 months after starting cyclosporine therapy. Combined administration of cyclosporine and prednisone may extend the waiting time while receiving low-dose steroids and allow more patients with idiopathic pulmonary fibrosis to qualify for lung transplantation while reducing the risk of steroid-induced complications.