Primary Antiphospholipid Syndrome Presenting With Life-Threatening Diffuse Alveolar Hemorrhage

Antiphospholipid syndrome (APS)-associated diffuse alveolar hemorrhage (DAH) results from antiphospholipid-induced endothelial dysfunction, microvascular thrombosis, and complement activation. Management requires balancing thrombosis prevention and hemorrhage control. High-dose corticosteroids are the first-line therapy. We report a case of a 33-year-old male presenting with progressive dyspnea, hemoptysis, and hypoxemia. Initial evaluation revealed thrombocytopenia, anemia, elevated inflammatory markers, and diffuse bilateral ground-glass opacities on a thoracic computed tomography, consistent with DAH. Autoimmune serology confirmed persistent positivity for lupus anticoagulant, anti-cardiolipin IgM, and anti-β2-glycoprotein-I IgM, fulfilling APS classification criteria. The patient developed respiratory failure, requiring mechanical ventilation and venovenous extracorporeal membrane oxygenation (VV-ECMO). High-dose corticosteroids were initiated, leading to gradual improvement. He was successfully extubated and discharged on long-term anticoagulation with warfarin. At four years of follow-up, he remains free of APS-related complications. This case highlights the importance of recognizing APS as a cause of DAH, particularly in patients with unexplained pulmonary hemorrhage. Early diagnosis and aggressive therapy can improve outcomes.

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Journal
Cureus
Published
2025-10-04
DOI
https://doi.org/10.7759/cureus.93814
Primary Topic
Systemic Lupus Erythematosus Research
Type
article
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article

Primary Antiphospholipid Syndrome Presenting With Life-Threatening Diffuse Alveolar Hemorrhage

M. Costa, João Casanova Pinto, Beatriz Fernandes, Carlos Ramalheira
Cureus
Systemic Lupus Erythematosus Research
article

Primary Antiphospholipid Syndrome Presenting With Life-Threatening Diffuse Alveolar Hemorrhage

M. Costa, João Casanova Pinto, Beatriz Fernandes, Carlos Ramalheira
article en

Abstract

Antiphospholipid syndrome (APS)-associated diffuse alveolar hemorrhage (DAH) results from antiphospholipid-induced endothelial dysfunction, microvascular thrombosis, and complement activation. Management requires balancing thrombosis prevention and hemorrhage control. High-dose corticosteroids are the first-line therapy. We report a case of a 33-year-old male presenting with progressive dyspnea, hemoptysis, and hypoxemia. Initial evaluation revealed thrombocytopenia, anemia, elevated inflammatory markers, and diffuse bilateral ground-glass opacities on a thoracic computed tomography, consistent with DAH. Autoimmune serology confirmed persistent positivity for lupus anticoagulant, anti-cardiolipin IgM, and anti-β2-glycoprotein-I IgM, fulfilling APS classification criteria. The patient developed respiratory failure, requiring mechanical ventilation and venovenous extracorporeal membrane oxygenation (VV-ECMO). High-dose corticosteroids were initiated, leading to gradual improvement. He was successfully extubated and discharged on long-term anticoagulation with warfarin. At four years of follow-up, he remains free of APS-related complications. This case highlights the importance of recognizing APS as a cause of DAH, particularly in patients with unexplained pulmonary hemorrhage. Early diagnosis and aggressive therapy can improve outcomes.

CureusVol. 17(10)
Hospital de São José (PT), Universitat de Barcelona (ES), Universidade Nova de Lisboa (PT)
Openalex Percentile: Top 60%
Systemic Lupus Erythematosus Research
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