title: Polymyositis and pemphigus vulgaris in a patient: successful treatment with rituximab.
abstract: Rituximab has been documented to be an effective treatment for autoimmune diseases with contribution of B cells. We report a case of antisynthetase syndrome with a history of EBV-induced lymphoma which developed a pemphigus vulgaris. Rituximab was effective both on polymyositis and on pemphigus. Fifteen months later, the patient died from a septic shock after the first cyclophosphamide infusion for amyloidosis while the B cell population remained depleted. Rituximab may be a good alternative to immunosuppressive drugs in polymyositis and pemphigus especially in lymphoma-risk patients. However, it did not prevent progression to secondary amyloidosis and the fatal infection developed in this patient raises the question of whether a prolonged B cell depletion with rituximab contributes to a greater risk of infection.
authors.affiliation: CHU Clermont-Ferrand, Rheumatology Department, G. Montpied Hospital, Place H Dunant, BP 69, 63003 Clermont-Ferrand, France. atournadre@chu-clermontferrand.fr
authors.forename: Anne
authors.initials: A
authors.lastname: Tournadre
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Amarger
authors.forename: Pascal
authors.initials: P
authors.lastname: Joly
authors.forename: Michel
authors.initials: M
authors.lastname: d'Incan
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Ristori
authors.forename: Martin
authors.initials: M
authors.lastname: Soubrier
mesh_terms: D000686:Amyloidosis / Q000150:complications / Q000473:pathology; D000974:Antibodies, Antinuclear / Q000276:immunology; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007674:Kidney Diseases / Q000150:complications / Q000473:pathology; D008025:Ligases / Q000276:immunology; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D017285:Polymyositis / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D000069283:Rituximab; D012772:Shock, Septic; D013577:Syndrome
chemical_list: D000974:Antibodies, Antinuclear; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D000069283:Rituximab; D008025:Ligases; D011241:Prednisone