Lupus (2014) 23, 1079–1084 http://lup.sagepub.com
CASE REPORT
The beneficial effect of plasmapheresis in mixed connective tissue disease with coexisting antiphospholipid syndrome P Szodoray1, A Hajas2, L Toth3, S Szakall3, B Nakken1, P Soltesz2 and E Bodolay2 1
Institute of Immunology, Rikshospitalet, University of Oslo, Oslo, Norway; 2Department of Medicine; and 3Department of Pathology, Medical and Health Science Centre, University of Debrecen, Debrecen, Hungary
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-b2-glycoprotein (antib2GPI) 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. Lupus (2014) 23, 1079–1084. Key words: Mixed connective tissue disease; antiphospholipid syndrome; plasmapheresis
Introduction
Case report
Mixed connective tissue disease (MCTD) is a systemic autoimmune disorder. The most common clinical manifestations of MCTD are polyarthritis, myositis, Raynaud’s phenomenon, sclerodactyly, swelling of the hands and fingers and pulmonary involvement.1 Digital ulcers and gangrene are relatively rare in these patients. In MCTD, anti-U1 ribonucleoprotein (anti-U1RNP) autoantibodies are present in the patients’ sera; however, other autoantibodies can also be found and modify the symptoms and disease course. Here, we report a rare case of a female MCTD patient with coexisting antiphospholipid syndrome (APS) successfully treated with corticosteroid pulse therapy, and plasma exchange in combination with synchronized cyclophosphamide. We also depict the serological findings, as well as changes in B-cell subsets during the therapy.
In 2003 a 46-year-old woman presented to our clinic with polyarthritis, Raynaud’s phenomenon, sclerodactyly and myositis. The patient had a high serum level of anti-U1RNP antibody (68 U/ml, norm.:
CASE REPORT
The beneficial effect of plasmapheresis in mixed connective tissue disease with coexisting antiphospholipid syndrome P Szodoray1, A Hajas2, L Toth3, S Szakall3, B Nakken1, P Soltesz2 and E Bodolay2 1
Institute of Immunology, Rikshospitalet, University of Oslo, Oslo, Norway; 2Department of Medicine; and 3Department of Pathology, Medical and Health Science Centre, University of Debrecen, Debrecen, Hungary
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-b2-glycoprotein (antib2GPI) 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. Lupus (2014) 23, 1079–1084. Key words: Mixed connective tissue disease; antiphospholipid syndrome; plasmapheresis
Introduction
Case report
Mixed connective tissue disease (MCTD) is a systemic autoimmune disorder. The most common clinical manifestations of MCTD are polyarthritis, myositis, Raynaud’s phenomenon, sclerodactyly, swelling of the hands and fingers and pulmonary involvement.1 Digital ulcers and gangrene are relatively rare in these patients. In MCTD, anti-U1 ribonucleoprotein (anti-U1RNP) autoantibodies are present in the patients’ sera; however, other autoantibodies can also be found and modify the symptoms and disease course. Here, we report a rare case of a female MCTD patient with coexisting antiphospholipid syndrome (APS) successfully treated with corticosteroid pulse therapy, and plasma exchange in combination with synchronized cyclophosphamide. We also depict the serological findings, as well as changes in B-cell subsets during the therapy.
In 2003 a 46-year-old woman presented to our clinic with polyarthritis, Raynaud’s phenomenon, sclerodactyly and myositis. The patient had a high serum level of anti-U1RNP antibody (68 U/ml, norm.:
