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Primary antiphospholipid syndrome evolving into systemic lupus erythematosus
R H Derksen1, F H Gmelig-Meijling, P G de Groot
1Department of Internal Medicine, University Hospital, Utrecht, The Netherlands.
Lupus
|February 1, 1996
Summary
Primary antiphospholipid syndrome (PAPS) can evolve into systemic lupus erythematosus (SLE) over time. A positive antinuclear antibody (ANA) test in PAPS patients may indicate future SLE development.
Area of Science:
- Rheumatology
- Immunology
- Hematology
Background:
- Primary antiphospholipid syndrome (PAPS) is characterized by recurrent venous thromboembolism and the presence of antiphospholipid antibodies.
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical manifestations.
Observation:
- A young woman with a history of recurrent venous thromboembolic disease was diagnosed with PAPS based on lupus anticoagulant and high-titer IgG anticardiolipin antibodies.
- She also had a positive antinuclear antibody (ANA) test (1:1280, speckled pattern).
- Twelve years after her initial thrombotic event, she met the classification criteria for SLE, developing myocardial infarction, polyarthritis, and nephritis.
Findings:
- The patient's progression from PAPS to SLE over a decade highlights a potential long-term evolution.
- A strongly positive ANA test in PAPS patients may precede the development of SLE.
- This case supports the hypothesis that PAPS can be a precursor to SLE.
Implications:
- Early identification of patients with PAPS and positive ANA may allow for closer monitoring for SLE development.
- Understanding this evolution can refine diagnostic criteria and management strategies for both PAPS and SLE.
- This case underscores the importance of long-term follow-up in patients diagnosed with antiphospholipid syndrome.