title: Current management strategies in paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome).
abstract: Paraneoplastic pemphigus (PNP) or paraneoplastic autoimmune multiorgan syndrome (PAMS) is a life-threatening autoimmune blistering disease commonly associated with lymphoproliferative neoplasms. This article focuses on current management strategies in PNP/PAMS, and reported instances of their treatment successes and failures. Due to the rarity of the condition and the high rates of treatment failure, no randomized control trials exist to guide the evidence-based treatment of this condition; all evidence to date on the efficacy of therapeutic modalities has been gained from individual case reports, small case series, and expert recommendations.
authors.affiliation: St George Hospital, Gray Street, Kogarah, Sydney, NSW 2217, Australia.
authors.forename: John W
authors.initials: JW
authors.lastname: Frew
authors.forename: Dédée F
authors.initials: DF
authors.lastname: Murrell
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000074323:Alemtuzumab; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000912:Antibodies, Neoplasm / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000628:therapy; D001379:Azathioprine / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use; D003879:Dermatologic Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D009102:Multiple Organ Failure / Q000188:drug therapy*; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D010257:Paraneoplastic Syndromes / Q000188:drug therapy* / Q000628:therapy; D010392:Pemphigus / Q000188:drug therapy* / Q000628:therapy; D010956:Plasmapheresis; D000069283:Rituximab; D016559:Tacrolimus / Q000627:therapeutic use
chemical_list: D000305:Adrenal Cortex Hormones; D061067:Antibodies, Monoclonal, Humanized; D058846:Antibodies, Monoclonal, Murine-Derived; D000912:Antibodies, Neoplasm; D003879:Dermatologic Agents; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000074323:Alemtuzumab; D000069283:Rituximab; D016572:Cyclosporine; D003520:Cyclophosphamide; D009173:Mycophenolic Acid; D001379:Azathioprine; D016559:Tacrolimus