title: The beneficial effect of plasmapheresis in mixed connective tissue disease with coexisting antiphospholipid syndrome.
abstract: The authors report a rare case of a female patient with mixed connective tissue disease (MCTD) with coexisting antiphospholipid syndrome (APS). Five years after the diagnosis of MCTD high concentrations of anticardiolipin (anti-CL) and anti-β2-glycoprotein (anti-β2GPI) autoantibodies were present in the patient's serum without thrombotic events. Epstein-Barr virus (EBV) reactivation provoked APS, with the clinical manifestations of livedo reticularis, digital gangrene and leg ulcers. Skin biopsy from the necrotic area showed multiple fibrin microthrombi in the superficial vessels. Corticosteroid pulse therapy, and plasma exchange in combination with synchronized cyclophosphamide was administered, which led to improvement of the digital gangrenes, while no new lesions developed. The number of CD27high plasma cells decreased, and the previous high levels of autoantibodies also normalized in the peripheral blood. In the case of MCTD with coexisting APS combination therapy, including plasmapheresis has beneficial effects.
authors.affiliation: Institute of Immunology, Rikshospitalet, University of Oslo, Oslo, Norway szodoray@gmail.com.
authors.forename: P
authors.initials: P
authors.lastname: Szodoray
authors.affiliation: Department of Medicine.
authors.forename: A
authors.initials: A
authors.lastname: Hajas
authors.affiliation: Department of Pathology, Medical and Health Science Centre, University of Debrecen, Debrecen, Hungary.
authors.forename: L
authors.initials: L
authors.lastname: Toth
authors.affiliation: Department of Pathology, Medical and Health Science Centre, University of Debrecen, Debrecen, Hungary.
authors.forename: S
authors.initials: S
authors.lastname: Szakall
authors.affiliation: Institute of Immunology, Rikshospitalet, University of Oslo, Oslo, Norway.
authors.forename: B
authors.initials: B
authors.lastname: Nakken
authors.affiliation: Department of Medicine.
authors.forename: P
authors.initials: P
authors.lastname: Soltesz
authors.affiliation: Department of Medicine.
authors.forename: E
authors.initials: E
authors.lastname: Bodolay
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D017152:Antibodies, Antiphospholipid / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000276:immunology / Q000628:therapy*; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy*; D010956:Plasmapheresis; D020551:Pulse Therapy, Drug; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D017152:Antibodies, Antiphospholipid; D015415:Biomarkers; D007166:Immunosuppressive Agents
keywords: Mixed connective tissue disease; antiphospholipid syndrome; plasmapheresis