Related Experiment Videos
Catastrophic antiphospholipid syndrome masquerading as ischaemic colitis
Jan Steffen Jürgensen1, Ralf Kettritz, Wolfgang Schneider
1Department of Nephrology and Medical Intensive Care, Charité, Campus Virchow Klinikum, Augustenburger Platz 1, 13353, Berlin, Germany. jan_steffen.juergensen@charite.de
Rheumatology International
|April 8, 2003
Summary
A young woman with acute diarrhea developed catastrophic antiphospholipid syndrome (APS). Prompt diagnosis and intensive treatment including anticoagulation led to her recovery.
Area of Science:
- Internal Medicine
- Rheumatology
- Hematology
Background:
- Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent thrombosis and pregnancy morbidity.
- Catastrophic APS (CAPS) is a rare, life-threatening variant with rapid, widespread organ damage.
- Early recognition and aggressive management are crucial for improving outcomes in CAPS.
Observation:
- A young woman presented with acute diarrhea, rapidly progressing to multiorgan failure requiring mechanical ventilation and dialysis.
- Coagulation tests revealed lupus anticoagulant, elevated IgG-anticardiolipin antibodies, and prolonged dilute Russell's viper venom time.
- Intestinal biopsy showed widespread thrombotic microangiopathy, confirming the diagnosis.
Findings:
- The patient was diagnosed with catastrophic antiphospholipid syndrome (APS) based on clinical, serological, and histological findings.
- Despite the poor prognosis associated with CAPS, the patient showed significant improvement.
- Treatment involved intensive anticoagulation, steroids, and immunoglobulins.
Implications:
- This case highlights the importance of considering APS in patients with unexplained thrombotic events and multiorgan failure.
- Aggressive anticoagulation and multimodal immunosuppressive therapy can lead to favorable outcomes even in severe CAPS cases.
- Further research into optimal treatment strategies for catastrophic APS is warranted.