Related Experiment Videos
Primary antiphospholipid syndrome (PAPS). Two case reports and therapeutic implications
1Department of Rheumatology and Clinical Immunology, University of Freiburg, Federal Republic of Germany.
Rheumatology International
|January 1, 1991
Summary
Primary antiphospholipid syndrome (PAPS) involves clotting disorders. Aggressive immunosuppression with prednisone, azathioprine, or cyclophosphamide effectively prevented thrombo-embolism and lowered antibody titers in two PAPS patients.
Area of Science:
- Immunology
- Hematology
- Rheumatology
Background:
- Antiphospholipid antibodies (aPL) disrupt coagulation, leading to thrombosis and related disorders.
- Primary antiphospholipid syndrome (PAPS) is defined by recurrent thrombosis, embolism, and fetal loss, excluding systemic lupus erythematosus (SLE).
Observation:
- Two patients with PAPS were treated with immunosuppressive therapy including prednisone and either azathioprine or cyclophosphamide.
- The study monitored IgG- and IgM-anticardiolipin antibody (aCL) titers over 18 months.
Findings:
- Immunosuppressive therapy was effective in preventing recurrent thrombo-embolism and other PAPS complications.
- Cyclophosphamide normalized aCL titers in one patient; azathioprine reduced titers in the other.
- Aggressive immunosuppression is proposed for severe, recurrent PAPS-associated thrombo-embolic events.
Implications:
- This approach may offer a new treatment strategy for severe primary antiphospholipid syndrome.
- Further research into the efficacy of specific immunosuppressants for managing aPL-related complications is warranted.
- Understanding the role of immunosuppression in managing aPL antibody titers and clinical outcomes is crucial.