Abstract Catastrophic antiphospholipid syndrome (CAPS) is a severe complication of antiphospholipid syndrome (APS) characterized by diffuse thrombosis and multiorgan involvement. Although CAPS is rare, occurring in approximately 1 percent of patients with APS 1, it carries a poor prognosis even with aggressive treatment. This report will describe a case of catastrophic antiphospholipid syndrome, as well as the numerous complications present through the course. A 64 year-old female presented to the medical intensive care unit for management of hemoptysis in the setting of catastrophic antiphospholipid syndrome. Medical history included triple-positive antiphospholipid syndrome (APS) on warfarin, pulmonary hypertension, heart failure with preserved ejection fraction (HFpEF), chronic kidney disease stage 4, aortic stenosis, Factor VIII deficiency, and immune thrombocytopenic purpura. Her recent history was significant in that she had undergone workup for triple-positive APS and immunosuppressive therapy with high-dose steroids, IVIG and rituximab. She initially presented for evaluation of dyspnea and right-sided chest discomfort. A computed tomography with angiography of the chest which demonstrated bilateral hilar fullness, increased interstitial markings and small pleural effusions suggesting volume overload with no evidence of pulmonary embolus (Figure 1). After several days of treatment with diuresis, the patient developed hemoptysis and underwent bronchoscopy with bronchoalveolar lavage. The findings of bloody secretions and increasingly sanguinous lavage fluid on three separate aliquots was suggestive of diffuse alveolar hemorrhage (DAH), and the patient was transferred to the MICU. In addition to DAH, the patient also demonstrated worsening renal function, heart failure with reduced ejection fraction, transaminitis and hyperbilirubinemia, schistocytes and elevated LDH, and the development of diffuse purpuric lesions. Skin biopsy revealed diffuse thrombotic angiopathy concerning for catastrophic antiphospholipid syndrome (CAPS) and warfarin failure. A management plan consisting of alternative therapeutic anticoagulation with heparin drip and factor Xa monitoring, pulse-dose methylprednisolone, and five rounds of plasma exchange therapy (with 1:1 ratio of FFP and albumin replacement) was initiated. Catastrophic antiphospholipid syndrome (CAPS) is a feared complication of antiphospholipid syndrome (APS) characterized by rapid onset of systemic thrombotic microangiopathy (TMA) which can involve both microvasculature as well as larger vessels throughout multiple organ systems. If concern for CAPS is high, a patient should be evaluated at a facility with the appropriate specialist coverage as well as the ability to provide advanced interventions. Aggressive management is necessary, and initial treatment should focus on achieving appropriate anticoagulation, immunosuppression with high-dose glucocorticoids, and the use of therapeutic plasma exchange or IVIG therapy. 2, 3 This abstract is funded by: None
Mckinney et al. (Fri,) studied this question.