Catastrophic antiphospholipid syndrome masquerading as ischaemic colitis

• Published in: Rheumatology international 2003-07, Vol.23 (4), p.204-206
• Main Authors: JÜRGENSEN, Jan Steffen, KETTRITZ, Ralf, SCHNEIDER, Wolfgang, KOOP, Herbert, HILDEBRAND, Thorsten Sven, FREI, Ulrich, ECKARDT, Kai-Uwe
• Format: Article
• Language: English
• Subjects: Adrenal Cortex Hormones - therapeutic use; Adult; Anticoagulants - therapeutic use; Antiphospholipid Syndrome - complications; Antiphospholipid Syndrome - diagnosis; Antiphospholipid Syndrome - therapy; Biological and medical sciences; Colitis, Ischemic - diagnosis; Colitis, Ischemic - etiology; Colitis, Ischemic - therapy; Diagnosis, Differential; Diarrhea - etiology; Diarrhea - therapy; Female; gamma-Globulins - therapeutic use; Humans; Medical diagnosis; Medical sciences; Multiple Organ Failure - etiology; Multiple Organ Failure - therapy; Treatment Outcome
• ISSN: 0172-8172; 1437-160X
• DOI: 10.1007/s00296-003-0296-1

We describe a young woman whose initial presentation was dominated by acute diarrhoea. Life-threatening multiorgan failure rapidly ensued and necessitated mechanical ventilation and dialysis treatment. An initially elongated activated partial thromboplastin time prompted further coagulation tests that led to the detection of positive lupus anticoagulant, a highly elevated IgG-anticardiolipin (aCL) antibody titre, and prolonged dilute Russell's viper venom time. Histological examination of samples obtained during endoscopy revealed widespread intestinal thrombotic microangiopathy. In view of these serologic and histologic features, a diagnosis of the malignant variant of the antiphospholipid syndrome (APS), also termed 'catastrophic APS', was established. In spite of this syndrome's grim prognosis, the patient recovered following intensive anticoagulation and adjunct treatment with steroids and immunoglobulins.