LAUSR.org creates dashboard-style pages of related content for over 1.5 million academic articles. Sign Up to like articles & get recommendations!

568: CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME A DIAGNOSTIC NIGHTMARE IN CRITICAL CARE SETTINGS

Photo from wikipedia

www.ccmjournal.org Critical Care Medicine • Volume 46 • Number 1 (Supplement) Learning Objectives: Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening manifestation of APS, characterized by multiple vascular occlusive events involving… Click to show full abstract

www.ccmjournal.org Critical Care Medicine • Volume 46 • Number 1 (Supplement) Learning Objectives: Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening manifestation of APS, characterized by multiple vascular occlusive events involving multiple organs developing over a short period of time. The mortality rate is almost 50%, making rapid diagnosis essential to its treatment. Methods: 46-year-old female presented with pleuritic chest pain and dyspnea. CT angiogram of the chest showed right-sided pulmonary embolism. She was diagnosed with unprovoked PE and was discharged on Rivaroxaban. Two weeks later, she presented to ED with headache and blurry vision. CT head showed tentorial subdural hematoma. No neurosurgical intervention was done. Her anticoagulation was switched to Apixaban. Two days later, she developed acute abdominal pain. CT abdomen showed b/l adrenal hemorrhage. She had high titers of beta2glycoprotein IgG/IgM; positive lupus anticoagulant with DRVVT and anticardiolipin antibody. She continued to decompensate with acute hemoglobin drop and hypoxic respiratory failure requiring oxygen therapy. She underwent bronchoscopy with increasingly bloody return on serial aliquots which is characteristic of diffuse alveolar hemorrhage (DAH). Pulse dose steroids were started with no improvement; therapeutic plasma exchange finally achieved remission. Repeat antiphospolipid antibodies remained positive albeit at a lower value four months after follow up. Results: This case highlights the importance of considering CAPS when multiple thromboembolic occlusions affecting diverse vascular beds are observed. The patient was diagnosed with CAPS as she fulfilled 3/4 diagnostic criteria. She had multiple organ involvement with subdural hematoma, b/l adrenal hemorrhages, PE and DAH all with a rapid onset in setting of positive lupus anticoagulant. This case shows how physicians focus on a narrow snapshot instead of seeing the bigger picture. An aggressive treatment strategy with a combination of immunosuppression, anticoagulation, plasma exchange, and IVIGs, is advised due to the high morbidity and mortality associated with CAPS. Anticoagulation options are limited as direct oral anticoagulants are of unproven benefit. Long term anticoagulation with Vitamin K antagonist is the preferred therapy. Conclusion: CAPS is a devastating disease process and attention should be paid to patients with acute episodes. It’s difficult to save patients without early treatment, thus an increased awareness of this disorder among clinicians is imperative.

Keywords: medicine; antiphospholipid syndrome; catastrophic antiphospholipid; critical care

Journal Title: Critical Care Medicine
Year Published: 2018

Link to full text (if available)


Share on Social Media:                               Sign Up to like & get
recommendations!

Related content

More Information              News              Social Media              Video              Recommended



                Click one of the above tabs to view related content.