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Conversion of discoid lupus to antiphospholipid syndrome and SLE

Nature Clinical Practice Rheumatology volume 4, pages 106–110 (2008)Cite this article

Abstract

Background A 64-year-old man was admitted to hospital with increasing seizure frequency, lethargy and confusion. He had a history of discoid lupus erythematosus, complex partial seizures, cerebral thromboses associated with antiphospholipid syndrome (APS) and hypertension. After admission to hospital, he developed autoimmune hemolytic anemia, thrombocytopenia, severe hypertension, proteinuria and a fluctuating level of consciousness.

Investigations Physical examination, biochemical and hematological indices, urine dipstick, electrocardiography and chest radiography on admission, with subsequent electroencephalography, lumbar puncture, septic screen, autoimmune screen, CT of the head, MRI of the brain and renal biopsy.

Diagnosis Conversion of discoid lupus erythematosus with APS to systemic lupus erythematosus with APS.

Management Pulsed intravenous methylprednisolone and a single infusion of intravenous cyclophosphamide, followed by oral prednisolone, were initially administered for presumed cerebral lupus. When renal biopsy revealed dual pathology, another dose of intravenous cyclophosphamide was administered with intravenous heparin therapy to treat systemic lupus erythematosus and APS, respectively. Intravenous immunoglobulin was used to treat thrombocytopenia, which was unresponsive to immunosuppression and anticoagulation. Gradual improvement occurred with maximal antihypertensive therapy and supportive treatment. The patient was discharged after 9 months of hospital treatment. His medication at this stage included warfarin, clopidogrel, prednisolone, carbamazepine, ramipril, atenolol, calcium and vitamin D supplements, and alendronate.

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Figure 1: MRI scan of the brain showing a previous hemorrhage in the right frontal lobe, indicated by a solid arrow.
Figure 2: Renal histopathology in antiphospholipid syndrome.

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Acknowledgements

The authors would like to thank Dr Meryl Griffiths (Department of Histopathology, University of Cambridge, UK) and Professor Guy Neild (retired) for their assistance and critical review of this report.

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Authors and Affiliations

  1. MMA Fernando is a Clinical Research Fellow at the Section of Molecular Genetics and Rheumatology, Faculty of Medicine, Imperial College, London, UK.,

    Michelle MA Fernando

  2. DA Isenberg is the Diamond Jubilee Professor of Rheumatology in the Department of Medicine at University College London, London, UK.,

    David A Isenberg

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  1. Michelle MA Fernando
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  2. David A Isenberg
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Correspondence to David A Isenberg.

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Fernando, M., Isenberg, D. Conversion of discoid lupus to antiphospholipid syndrome and SLE. Nat Rev Rheumatol 4, 106–110 (2008). https://doi.org/10.1038/ncprheum0704

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