title: Immunoadsorption in pemphigus.
abstract: The principle of extracorporal immunoadsorption (IA) is based on affinity adsorption of pathogenic (auto-)antibodies and circulating immune complexes (CIC) which reversibly bind to an immobilized ligand of the adsorber. In pemphigus, a blistering autoimmune disease affecting skin and mucous membranes, autoantibodies, mainly of the IgG subclass are directed against desmosomal adhesion molecules and other non-desmosomal antigens on the surface of epidermal keratinocytes, such as acetylcholine receptors. The pathogenicity of these autoantibodies has been shown in various in vitro and in vivo systems. Recently, IA was applied in severe pemphigus demonstrating that a rapid and dramatic decline in desmoglein (Dsg)-reactive autoantibodies is accompanied by clinical remission of mucocutaneous blisters and erosions. As an adjuvant treatment, IA was combined with systemic immunosuppressive medication and current protocols initially apply treatment cycles of 3-4 IAs on consecutive days followed by immunoapheresis once a week or repeating the initial cycle in 4 week intervals depending on the disease activity. IA in pemphigus is generally safe and well tolerated.
authors.affiliation: Department of Dermatology and Allergology, University Hospital, Philipps University, Marburg, Germany. eming@med.uni-marburg.de
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Eming
authors.forename: Michael
authors.initials: M
authors.lastname: Hertl
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000378:metabolism*; D051184:Desmoglein 3 / Q000276:immunology / Q000378:metabolism; D006801:Humans; D007163:Immunosorbent Techniques; D007164:Immunosorbents / Q000378:metabolism*; D010392:Pemphigus / Q000188:drug therapy / Q000276:immunology* / Q000378:metabolism / Q000628:therapy*; D017455:Receptors, IgE / Q000276:immunology / Q000378:metabolism
chemical_list: D001323:Autoantibodies; C071894:DSG3 protein, human; D051184:Desmoglein 3; C475664:FCER1A protein, human; D007164:Immunosorbents; D017455:Receptors, IgE