MIRZA ALI, MD
University of Illinois College of Medicine
PEORIA, Illinois
Disclosure information not submitted.
Shujaa Faryad, MD
DR
University of Illinois College of Medicine
PEORIA, Illinois, United States
Disclosure information not submitted.
Title: Catastrophic Antiphospholipid syndrome and COVID-19 – A Clinical Conundrum
Introduction: Catastrophic antiphospholipid syndrome (CAPS) is distinguished from its normal counterpart by the presence of end-organ damage as a result of thrombotic complications resulting in multiorgan failure. Clinical presentation and clinical courses are extremely variable and warrant increased threshold of suspicion to diagnose and treat.
Case Presentation: 65-year old female with past medical history of antiphospholipid syndrome, DVT/PE on chronic anticoagulation presented to the hospital for acute encephalopathy and acute hypoxemic respiratory failure. Labs were significant for elevated titers for anti-lupus anticoagulant, anticardiolipin, anti-beta1 glycoprotein 1. She was found to be positive for COVID-19 infection by PCR. Initial CT head was negative. MRI revealed progressive white matter disease without acute CVA. Encephalopathy and respiratory status continued to worsen and repeat MRI several days later showed multiple ischemic and hemorrhagic CVA for which she was transferred to tertiary care center. Respiratory failure was managed with heated-high flow. She was given high dose IV methylprednisolone, rituximab, was given 2 doses of IVIG and underwent 5 sessions of plasma exchange. Patient was discharged to SNF in stable condition after resolution of encephalopathy and respiratory failure.
Discussion: CAPS is diagnosed by clinical, laboratory, and histopathologic criteria. Patients must have new thromboses, multiorgan involvement within a short amount of time, and positive titers for antibodies to be diagnosed. CAPS is frequently triggered by sepsis (22%) or surgical procedures (10%). Pulmonary and neurologic complications are common, 64% and 62% respectively. Pulmonary complications include ARDS, PE, and diffuse alveolar hemorrhage. Neurologic complications include encephalopathy, CVA, and seizures. Management involves a combination of anticoagulation, corticosteroids, plasma exchange, cyclophosphamide, and IVIG. Analysis of CAPS registry data shows that 44% of patients die due to a CAPS event. Data suggests that patients treated with anticoagulation plus corticosteroids in addition to plasma exchange or IVIG have a survival rate of 70%. Due to the uncommon nature and variable clinical presentation of this disease, high index of suspicion and initiation of aggressive therapy is warranted.