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Published on: May 23, 2025
Catastrophic antiphospholipid syndrome
Agnieszka Furmańczyk1, Ewa Komuda-Leszek, Wioletta Gadomska
1Department of Transplantation Medicine and Nephrology, Transplantation Institute, Medical University of Warsaw, Warszawa, Poland. afurmanczyk@gmail.com
Catastrophic Antiphospholipid Syndrome (CAPS) treatment challenges were addressed using plasma exchange and intravenous immunoglobulin therapy. This case study shows successful management of a patient with severe APS, improving renal function and clinical status.
Area of Science:
- Internal Medicine
- Rheumatology
- Immunology
Background:
- Antiphospholipid Syndrome (APS) is an autoimmune disorder characterized by thrombosis and antiphospholipid antibodies (APLA).
- Catastrophic Antiphospholipid Syndrome (CAPS) is a rare, life-threatening variant with rapid multi-organ failure, often caused by small vessel thrombosis.
Observation:
- A 39-year-old male patient presented with worsening renal function secondary to APS.
- Diagnostic findings included positive lupus anticoagulant, high anticardiolipin, and anti-beta 2-glycoprotein I antibodies.
- The patient met criteria for CAPS, necessitating aggressive treatment.
Findings:
- The patient received treatment with low-molecular-weight heparin, glucocorticosteroids, plasmapheresis, and intravenous immunoglobulin (IVIg).
- Following IVIg administration (1 g/kg), gradual clinical improvement was observed.
- Renal function stabilized, with serum creatinine levels reaching 1.5 mg/dl.
Implications:
- Plasma exchange and IVIg can be effective in managing severe CAPS cases.
- This case highlights the importance of timely and aggressive treatment strategies for CAPS.
- Further research into optimal treatment protocols for CAPS is warranted to improve patient outcomes.
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