Detection of lupus anticoagulant and successful anticoagulation in familial Sneddon syndrome
- 1Section of Rheumatology, Department of Medicine, State University of New York Upstate Medical University, Syracuse, New York, USA
- 2Department of Pathology, State University of New York Upstate Medical University, Syracuse, New York, USA
- 3Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases, Lerner College of Medicine, Cleveland Clinic, Cleveland, Ohio, USA
- Correspondence to Professor Andras Perl, 750 East Adams Street, Syracuse, NY 13210, USA;
- Accepted 19 May 2009
We report the cases of two sisters with systemic autoimmune disease accompanied by recurrent transient ischaemic attacks (TIAs), antiphospholipid antibodies (aPL) and livedo reticularis (LR). Both were thought to have central nervous system (CNS) vasculitis but immunosuppression failed. Subsequently, they were diagnosed with Sneddon's syndrome (SnS) and both experienced dramatic improvement following anticoagulation.
A 47-year-old woman had experienced recurrent dizziness, confusion, headaches, alternating muscle spasms and weakness, and falls since 1996. She was initially diagnosed with systemic lupus erythematosus (SLE) based on the presence of oral ulcers, malar rash, antinuclear antibodies, leucopenia and presumed lupus cerebritis. She also had LR and low C4. MRI of the brain showed white matter foci of increased signal intensity. …