Catastrophic antiphospholipid syndrome masquerading as ischaemic colitis.
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.
Jürgensen
Jan Steffen
JS
Department of Nephrology and Medical Intensive Care, Charité, Campus Virchow Klinikum, Augustenburger Platz 1, 13353, Berlin, Germany. jan_steffen.juergensen@charite.de
Kettritz
Ralf
R
Schneider
Wolfgang
W
Koop
Herbert
H
Hildebrand
Thorsten Sven
TS
Frei
Ulrich
U
Eckardt
Kai-Uwe
KU
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D017091:Colitis, Ischemic / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy; D003937:Diagnosis, Differential; D003967:Diarrhea / Q000209:etiology / Q000628:therapy; D005260:Female; D006801:Humans; D009102:Multiple Organ Failure / Q000209:etiology / Q000628:therapy; D016896:Treatment Outcome; D005719:gamma-Globulins / Q000627:therapeutic use
D000305:Adrenal Cortex Hormones; D000925:Anticoagulants; D005719:gamma-Globulins