Pulmonary embolism as the first manifestation of antiphospholipid syndrome

    Authors

    Keywords

    antiphospholipid syndrome, pulmonary embolism, thrombotic disorder, autoimmune disease

    DOI

    https://doi.org/10.15836/ccar2026.45

    Full Text

    **Introduction**: Antiphospholipid syndrome (APS) is a rare autoimmune disorder characterized by increased propensity to thrombotic events, pregnancy complications and persistent presence of antiphospholipid antibodies (1). Although relatively uncommon, APS has significant clinical importance due to potentially life-threatening complications. In APS patients, thrombotic events represent the most common syndrome manifestation. Deep vein thrombosis with pulmonary embolism is the most common manifestation (2). APS can be primary (isolated) or secondary, associated with other autoimmune diseases, most commonly systemic lupus erythematosus (SLE), where 30-40% of SLE patients have antiphospholipid antibodies (3). **Case report**: 22-year-old male patient, who presented to the Emergency Department with dyspnea during physical activity, followed by collapse and loss of consciousness. MSCT pulmonary angiography confirmed thromboembolic disease of large pulmonary artery branches bilaterally, with pericardial effusion. Treatment was immediately initiated with therapeutic-dose low-molecular-weight heparin. From the sixth day of hospitalization, peroral anticoagulant therapy with warfarin was introduced. Laboratory findings showed a positive ANA titer 1:320 with cytoplasmic and punctate pattern, elevated anti-dsDNA (31 H), and positive beta-2 glycoprotein I antibodies (37.9). Lupus anticoagulant was negative. These findings combined with clinical presentation confirmed antiphospholipid syndrome diagnosis. Multidisciplinary treatment approach included rheumatological follow-up with introduced hydroxychloroquine 200 mg daily, along with regular hematologist and cardiologist controls. Continuation of anticoagulant therapy and serological marker monitoring is recommended. Follow-up immunological workup is planned three months after initial testing to confirm antiphospholipid antibody persistence. **Conclusion**: This case illustrates APS as a significant diagnosis in young adults with unexplained pulmonary embolism. Early diagnosis and timely anticoagulant treatment are crucial for preventing recurrent thrombotic events. A multidisciplinary approach with regular monitoring enables optimal management of this complex autoimmune disorder.

    Literature

    1. Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002 April;46(4):1019–27. https://doi.org/10.1002/art.10187
    2. Sciascia S, Hunt BJ, Talavera-Garcia E, Lliso G, Khamashta MA, Cuadrado MJ. The impact of hydroxychloroquine treatment on pregnancy outcome in women with antiphospholipid antibodies. Am J Obstet Gynecol. 2016 February;214(2):273.e1–8. https://doi.org/10.1016/j.ajog.2015.09.078
    3. Tektonidou MG, Andreoli L, Limper M, Amoura Z, Cervera R, Costedoat-Chalumeau N, et al. EULAR recommendations for the management of antiphospholipid syndrome in adults. Ann Rheum Dis. 2019 October;78(10):1296–304. https://doi.org/10.1136/annrheumdis-2019-215213
    Cardiologia Croatica
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    Pulmonary embolism as the first manifestation of antiphospholipid syndrome

    Extended Abstract
    Issue1-2
    Published
    Pages45
    PDF via DOIhttps://doi.org/10.15836/ccar2026.45
    antiphospholipid syndrome
    pulmonary embolism
    thrombotic disorder
    autoimmune disease

    Authors

    Matilda Pudić*General Hospital “Dr. Josip Benčević”, Slavonski Brod, Croatia
    Ivica DunđerORCIDGeneral Hospital “Dr. Josip Benčević”, Slavonski Brod, Croatia
    Josip SilovićORCIDGeneral Hospital “Dr. Josip Benčević”, Slavonski Brod, Croatia

    *Correspondence email: matilda.pudic@gmail.com

    Full Text

    Introduction: Antiphospholipid syndrome (APS) is a rare autoimmune disorder characterized by increased propensity to thrombotic events, pregnancy complications and persistent presence of antiphospholipid antibodies (1). Although relatively uncommon, APS has significant clinical importance due to potentially life-threatening complications. In APS patients, thrombotic events represent the most common syndrome manifestation. Deep vein thrombosis with pulmonary embolism is the most common manifestation (2). APS can be primary (isolated) or secondary, associated with other autoimmune diseases, most commonly systemic lupus erythematosus (SLE), where 30-40% of SLE patients have antiphospholipid antibodies (3).

    Case report: 22-year-old male patient, who presented to the Emergency Department with dyspnea during physical activity, followed by collapse and loss of consciousness. MSCT pulmonary angiography confirmed thromboembolic disease of large pulmonary artery branches bilaterally, with pericardial effusion. Treatment was immediately initiated with therapeutic-dose low-molecular-weight heparin. From the sixth day of hospitalization, peroral anticoagulant therapy with warfarin was introduced. Laboratory findings showed a positive ANA titer 1:320 with cytoplasmic and punctate pattern, elevated anti-dsDNA (31 H), and positive beta-2 glycoprotein I antibodies (37.9). Lupus anticoagulant was negative. These findings combined with clinical presentation confirmed antiphospholipid syndrome diagnosis. Multidisciplinary treatment approach included rheumatological follow-up with introduced hydroxychloroquine 200 mg daily, along with regular hematologist and cardiologist controls. Continuation of anticoagulant therapy and serological marker monitoring is recommended. Follow-up immunological workup is planned three months after initial testing to confirm antiphospholipid antibody persistence.

    Conclusion: This case illustrates APS as a significant diagnosis in young adults with unexplained pulmonary embolism. Early diagnosis and timely anticoagulant treatment are crucial for preventing recurrent thrombotic events. A multidisciplinary approach with regular monitoring enables optimal management of this complex autoimmune disorder.

    Literature

    1. 1.
      Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002 April;46(4):1019–27.DOI
    2. 2.
      Sciascia S, Hunt BJ, Talavera-Garcia E, Lliso G, Khamashta MA, Cuadrado MJ. The impact of hydroxychloroquine treatment on pregnancy outcome in women with antiphospholipid antibodies. Am J Obstet Gynecol. 2016 February;214(2):273.e1–8.DOI
    3. 3.
      Tektonidou MG, Andreoli L, Limper M, Amoura Z, Cervera R, Costedoat-Chalumeau N, et al. EULAR recommendations for the management of antiphospholipid syndrome in adults. Ann Rheum Dis. 2019 October;78(10):1296–304.DOI