"Antiphospholipid Syndrome and the Craft of Its Sequalae" by L Masood, M S. Wasim et al.
 

Antiphospholipid Syndrome and the Craft of Its Sequalae

Document Type

Conference Proceeding

Publication Date

5-2025

Publication Title

American Journal of Respiratory and Critical Care Medicine

Abstract

Introduction: Antiphospholipid Syndrome (APS) is an autoimmune disorder characterized by recurrent thrombotic events due to antiphospholipid antibodies. APS is associated with a higher risk of both venous and arterial thromboembolism, with PE representing one of the most life-threatening manifestations. Patients presenting to the critical care unit are often hard to assess given the sequalae of complications associated with APS. Appropriate recognition becomes key to management. Case: 53-yearold female with past medical history of hypertension, left leg DVT, PE status post embolectomy, positive lupus anticoagulant noncompliant with Eliquis, presented for evaluation of multiple episodes of loss of consciousness in the setting of severe coughing bouts. Labs were notable for Hb 5.9, platelets 14, PTT 29.4, INR 1.8, total bilirubin 4, LDH 593, direct bilirubin 2.36, haptoglobin less than 30, alkaline phosphatase 88, AST 28, ALT 7, creatinine 2.19, BNP 651, lactic acid 3.7 TSH 1.9. Patient's intravascular hemolysis, thrombocytopenia, increased creatinine, hypercoagulable state, were discussed among rheumatology, cardiology, pulmonology, nephrology, hematology. ADAM-TS was normal and the hemolysis was intravascular (ruling out ITP/TTP). CTA showed extensive calcified lesions in the main pulmonary artery extending into the left and right pulmonary arteries, with dense calcified mass in the right atrium extending into the right ventricular and IVC (image-1). Echocardiogram showed right ventricular systolic function depression, along with the thrombus. The conclusion was made that this is likely APLA, causing thrombocytopenia, and chronic hypercoagulable state leading to these large pulmonary emboli. The calcified extensive pulmonary embolism is causing shearing of RBCs hence hemolysis. She was transferred to facility for management of CTEPH with pulmonary endarterectomy. Discussion: This case underscores the complex interplay between antiphospholipid syndrome (APS) and pulmonary embolism (PE). Typically, intravascular hemolysis is not associated with APS, often leading physicians to rule it out as a primary diagnosis. Since APS complications can overlap with other pathologies, physicians should maintain a broad differential diagnosis, especially when encountering atypical presentations. This case also highlights a particularly rare form of large, calcified PE extending from the pulmonary artery to the inferior vena cava. Such extensive emboli are rarely observed and typically present with symptoms much earlier. Additionally, chronic thromboembolic pulmonary hypertension (CTEPH) is identified in up to 15-50% of APS cases, and often goes unrecognized. This emphasizes medication adherence and the need for close monitoring due to the, often subtle but, potentially fatal course of APS.

Volume

211

First Page

A2209

Comments

American Thoracic Society (ATS) International Conference May 16-21, 2025, San Francisco, CA

Last Page

A2209

DOI

10.1164/ajrccm.2025.211.Abstracts.A2209

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