sirolimus and Sarcoidosis--Pulmonary

Topics: Anti-Bacterial Agents; Anti-Inflammatory Agents, Non-Steroidal; Doxycycline; Enzyme Inhibitors; Humans; Idiopathic Pulmonary Fibrosis; Immunosuppressive Agents; Indoles; Lung Diseases, Interstitial; Lymphangioleiomyomatosis; Pyridones; Sarcoidosis, Pulmonary; Sirolimus; Smoking; Tomography, X-Ray Computed; Tumor Necrosis Factor-alpha

As our understanding of the pathobiology and natural history of the various forms of interstitial lung disease (ILD) has evolved, so have our approaches to treating this heterogeneous group of lung disorders. The earliest pharmacologic agents used to treat various forms of ILD were corticosteroids, and corticosteroids are currently the mainstay of therapy for many forms of ILD. However, it has become clear that corticosteroids and other anti-inflammatory agents lack efficacy for many forms of ILD, such as idiopathic pulmonary fibrosis (IPF), and newer therapies that are in clinical trials target the fibrogenic process and/or secondary pulmonary hypertension (PH) that is present in various forms of fibrotic lung disease. Novel therapies, such as the use of biologic agents (antibodies and cell cycle inhibitors) or stem cell therapies will undoubtedly evolve as new research is performed and clinical trials are undertaken. Lung transplantation remains an option for advanced lung disease that is progressive and unresponsive to non-surgical therapies.

Topics: Clinical Trials as Topic; Cyclophosphamide; Glucocorticoids; Humans; Idiopathic Pulmonary Fibrosis; Immunosuppressive Agents; Lung Diseases, Interstitial; Macrolides; Risk Assessment; Sarcoidosis, Pulmonary; Sirolimus

Topics: Asthma; Biomarkers; Humans; Sarcoidosis; Sarcoidosis, Pulmonary; Sirolimus

Topics: Bile Duct Diseases; Biopsy; Constriction, Pathologic; Cyclosporine; Humans; Liver; Liver Cirrhosis; Liver Failure; Liver Transplantation; Male; Middle Aged; Postoperative Complications; Sarcoidosis; Sarcoidosis, Pulmonary; Sirolimus; Time Factors; Treatment Outcome

Rapamycin is a new immunosuppressive agent that has been shown to be effective in acute heart allograft rejection. This case documents a patient suffering from cardiac sarcoidosis who was bridged to transplantation for 90 days with ongoing rejection after allograft implantation. Rejection did not abate despite treatment with antithymocyte globulin (ATG), FK506, a mycophenolate switch and courses of multiple apheresis. Initiation of rapamycin treatment resulted in a rapid resolution of cardiac rejection and reduction of concomitant immunosuppressive agents with few side-effects. Most notably was the reduction of panel reactive antibodies within a few weeks after the rapamycin initiation. This case illustrates that the utilization of rapamycin ceased ongoing rejection in a patient with a clear hyperimmune state despite prior extensive utilization of a variety of immunosuppressive strategies after heart transplantation.

Topics: Adult; Graft Rejection; Heart Transplantation; Heart-Assist Devices; Humans; Immunosuppression Therapy; Immunosuppressive Agents; Male; Salvage Therapy; Sarcoidosis, Pulmonary; Sirolimus; Time; Treatment Outcome