CHEST Guidelines-A-36-Year-Old-Woman-Presenting-With-Left-Upper-Qua

A-36-Year-Old-Woman-Presenting-With-Left-Upper-Qua

Pdf Summary

A 36-year-old woman with a history of hypertension and alcoholism presented with left upper quadrant pain and jaundice, quickly progressing to somnolence and hypoxia, requiring intubation. Upon examination, she displayed jaundice, respiratory distress, and mottled extremities. Diagnostic imaging identified diffuse pulmonary infiltrates, thrombus in the abdominal aorta and portal vein, and ischemic changes in the brain. Laboratory tests revealed hyperammonemia and elevated anticardiolipin IgM levels, suggesting catastrophic antiphospholipid syndrome (CAPS).<br /><br />CAPS is a severe variant of antiphospholipid syndrome (APS) characterized by rapid multi-organ failure due to widespread thrombosis, occurring in less than a week. It is typically associated with the presence of antiphospholipid antibodies, such as lupus anticoagulant, anticardiolipin, or anti-beta2-glycoprotein. The syndrome often necessitates swift intervention due to a high mortality rate.<br /><br />Despite lack of histopathologic confirmation, this patient's clinical progression and laboratory findings supported a diagnosis of probable CAPS. Initial treatment included anticoagulation with heparin, necessary for mitigating further thrombosis. The patient was concurrently treated with antibiotics for suspected pulmonary infection and norepinephrine for hemodynamic support. Following anticoagulation, her condition improved rapidly, with better mentation, reduced abdominal pain, and decreasing ammonia levels. She was weaned from ventilatory and circulatory support, and transitioned to long-term anticoagulation therapy with warfarin.<br /><br />Treatment of CAPS emphasizes prompt anticoagulation, potentially complemented by steroids, plasma exchange, or intravenous immunoglobulin. In refractory cases, monoclonal antibodies like rituximab or eculizumab may be employed. This case illustrates the importance of prompt recognition and treatment of CAPS to improve outcomes.

Keywords

catastrophic antiphospholipid syndrome

CAPS

antiphospholipid antibodies

hyperammonemia

heparin

warfarin

thrombosis

anticoagulation

pulmonary infiltrates

ischemic changes