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Antiphospholipid antibody syndrome due to interferon treatment for hepatitis C. Michi Shinohara, MD Pacific Dermatologic Association August 10, 2008. Case presentation. 53 yo woman with 3 week history of progressive superficial cutaneous thrombosis
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Antiphospholipid antibody syndrome due to interferon treatment for hepatitis C Michi Shinohara, MD Pacific Dermatologic Association August 10, 2008
Case presentation • 53 yo woman with 3 week history of progressive superficial cutaneous thrombosis • History of Hep C with cryoglobulinemia and cutaneous vasculitis, s/p IFN and ribavarin • IVDU > 10y ago, current skin lesions in areas of prior injection • Health otherwise stable, no dyspnea, chest pain or leg edema
Work up • Upper extremity duplex confirmed superficial clot; no DVT • Assessed for hypercoaguable state • Anticardiolipin antibodies, lupus anticoagulant, inherited thrombophilias, cryoglobulins negative • 2 glycoprotein-1 Ab (2-GP-1) titer >99th percentile • Referred to hematology, initiated on anticoagulation for antiphospholipid antibody syndrome (APS)
Antiphospholipid antibody syndrome • Primary or secondary (SLE) • Diagnosis based on antiphospholipid antibodies “aPL Abs” (LA, aCL, 2-GP-1) and clinical features (thrombosis, fetal loss) • Pathogenesis due to aPL Ab binding to phospholipid associated proteins, especially 2-GP-1 • Main antigenic target of aPL Abs • Clotting inhibitor required for binding of aCL with cardiolipin
Autoantibodies and hepatitis C • Development of autoantibodies in setting of hepatitis C is common - RF • APL Abs including aCL1, LA2 reported in up to 46% of those with hepatitis C • APS in hepatitis C rare, incidence not increased in setting of hepatitis C1,2 • aPL Abs due to infection “cofactor independent”, nonthrombogenic • No increased incidence of 2-GP-1 antibodies Ordi-Ros et al, (2000) Clin and Diag Lab Immuno 7:241-244 Amin, N (2008) Hematol Oncol Clin N Am 22:131-143
Antiphospholipid antibody syndrome and interferon • Autoantibodies and immune phenomenon common during IFN therapy • Thyroid antibodies in 22% • Appearance or exacerbation of SLE, sarcoidosis1 • Our patient had mildly elevated 2-GP-1 antibodies prior to therapy (22 SGU, ref range 0-9) which rose markedly after IFN • Also developed sarcoidosis in tattoo 1. Hauschild et al (2008) J Deutschem Dermatol ISSN: 1610-0387 (Online)
Conclusions • Numerous autoimmune phenomena attributable to hepatitis C as well as immunomodulatory treatment • Although development of APS is rare, consider screening for aPL Abs in patients with thrombosis in setting of hepatitis C or interferon treatment
Thank you Dr John Olerud, Division head; Claudia Davis ARNP, and the Pacific Derm Association