Introduction: Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening form of antiphospholipid syndrome defined by the presence of various thromboses leading to multiorgan failure over a brief period of time. Because of its multiorgan involvement, presentations can vary drastically. If not recognized promptly, CAPS will lead to devastating outcomes. Our case explores a unique early misdiagnosis.
Description: A previously healthy 24 year-old male was seen at a standalone emergency department for a week of fatigue, fever, abdominal pain, nausea, and vomiting. Work up there demonstrated leukocytosis, thrombocytopenia, transaminitis, and elevated INR. A CT abdomen/pelvis showed hepatosplenomegaly with multiple hypoattenuating areas scattered throughout the liver concerning for abscesses or malignancy.
He was transferred to another facility and treated with IV antibiotics and vitamin K without improvement. An MRCP showed multifocal rim-enhancing liver lesions with central fluid attenuation suggestive of multifocal abscess. He underwent CT-guided aspiration and hepatic biopsy. The sample was grossly bloody with negative gram stain and cultures, while the liver biopsy demonstrated sinusoidal dilation and congestion. The patient worsened and was transferred to our facility with concerns for acute liver failure.
At our facility, his outside images were reevaluated and felt to be more concerning for thromboses within the liver vasculature with associated liver infarcts. Repeat CT imaging showed an increased number of thromboses, increased areas of liver infarct, and new adrenal infarcts. The patient also developed chest pain with troponemia and petechial rash over his torso. His presentation met the criteria for definite CAPS. Thus antibiotics were stopped and the patient was started on high intensity heparin. He underwent 5 sessions of plasmapheresis and 5 sessions of IVIG with improvement in his symptoms and end organ function. He was bridged to warfarin and discharged on steroids.
Discussion: CAPS presentations can vary dramatically and mimic other diseases. It is important to reevaluate available data and maintain CAPS in the differential of patients with coagulopathy and multiorgan involvement. Prompt diagnosis and a treatment are important to avoid marked morbidity and mortality.
