title: Extracorporeal photopheresis as a therapy for autoimmune diseases.
abstract: Systemic autoimmune diseases (AID) have multiorgan, heterogeneous clinical presentations and are characterized by dysregulation of the immune system, immunodeficiency, irreversible organ damage and increased morbidity and mortality. Preventing or decreasing flares of AID correlate with durable disease control, significant reduction of inflammation and prevention of disability or therapy-related toxicity. There is an urgent need for better treatment of severe, therapy-refractory AID. Extracorporeal photopheresis (ECP) is a cell-based immunomodulatory treatment which has been extensively used in variety of autoimmune disorders for the last two decades. ECP treatment is FDA approved for the treatment of cutaneous T-cell lymphoma (CTCL) with particularly promising results seen in graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (HCT). Prolonged therapy is safe, well tolerated and allows reduction of systemic immunosuppression in therapy-refractory patients. Both clinical and experimental evidence suggest that ECP mechanism of action is characterized by apoptosis and phagocytosis of activated cells by antigen-presenting cells (APC), secretion of anti-inflammatory cytokines and stimulation of regulatory T cells (Tregs). The focus of this paper is to review the current evidence of ECP use in the treatment of AID. Here, we summarize the experience of nine major AID from 65 published reports. The key findings demonstrate substantial evidence of ECP feasibility, safety and in some AID also promising efficacy. However, the role of ECP in AID therapy is not established as most published studies are retrospective with limited number of patients and the trials are small or poorly standardized. The available data support future investigations of ECP as a therapeutic modality for the treatment of AID in well-designed prospective clinical studies. J
authors.affiliation: Graft-versus-Host and Autoimmunity Unit, Experimental Transplantation and Immunology Branch, National Cancer Institute NCI, National Institutes of Health, Bethesda, Maryland.
authors.forename: Zoya
authors.initials: Z
authors.lastname: Kuzmina
authors.affiliation: Cell Processing Section, Department of Transfusion Medicine, Clinical Center, National Institutes of Health, Bethesda, Maryland.
authors.forename: David
authors.initials: D
authors.lastname: Stroncek
authors.affiliation: Graft-versus-Host and Autoimmunity Unit, Experimental Transplantation and Immunology Branch, National Cancer Institute NCI, National Institutes of Health, Bethesda, Maryland.
authors.forename: Steven Z
authors.initials: SZ
authors.lastname: Pavletic
mesh_terms: D017209:Apoptosis; D001327:Autoimmune Diseases / Q000276:immunology* / Q000628:therapy*; D001781:Blood Component Removal / Q000379:methods*; D002986:Clinical Trials as Topic; D006801:Humans; D007108:Immune Tolerance; D007165:Immunosuppression Therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007249:Inflammation; D008730:Methoxsalen / Q000737:chemistry; D010587:Phagocytosis; D017893:Photopheresis / Q000379:methods*; D017319:Photosensitizing Agents / Q000627:therapeutic use; D012107:Research Design; D012189:Retrospective Studies; D014466:Ultraviolet Rays
chemical_list: D007166:Immunosuppressive Agents; D017319:Photosensitizing Agents; D008730:Methoxsalen
keywords: autoimmune disease; cellular therapy; extracorporeal; immunomodulation; photopheresis