Imaging findings in the rare catastrophic variant of the primary antiphospholipid syndrome.
We report imaging findings in a case of the rare catastrophic variant of antiphospholipid syndrome (CAPS) characterized by widespread microvascular occlusions, which may lead to multiple organ failure. We present a case of a 66-year-old woman with bone marrow necrosis, acute acalculous cholecystitis (AAC), focal liver necrosis, subtle patchy splenic infarctions, and bilateral adrenal infarction. The demonstration of multiple microvascular organ involvement (three or more) is crucial for the diagnosis of the catastrophic variant of APS. This can be performed radiologically intra-vitam. Imaging can even reveal subclinical microinfarctions, which are often only diagnosed at autopsy.
Thuerl
Christina
C
Department of Radiology, University of Freiburg, Hugstetter Strasse 55, 79106 Freiburg, Germany. thuerl@mrs1.ukl.uni-freiburg.de
Altehoefer
Carsten
C
Spyridonidis
Alexandros
A
Laubenberger
Jörg
J
D000368:Aged; D016736:Antiphospholipid Syndrome / Q000145:classification / Q000150:complications / Q000175:diagnosis*; D001853:Bone Marrow / Q000473:pathology; D002764:Cholecystitis / Q000000981:diagnostic imaging / Q000209:etiology; D005260:Female; D006801:Humans; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008107:Liver Diseases / Q000000981:diagnostic imaging / Q000209:etiology; D008279:Magnetic Resonance Imaging; D009102:Multiple Organ Failure / Q000209:etiology; D009336:Necrosis; D013131:Spine / Q000473:pathology; D013154:Spleen / Q000000981:diagnostic imaging; D013158:Splenic Diseases / Q000000981:diagnostic imaging / Q000209:etiology; D014057:Tomography, X-Ray Computed