Primary Antiphospholipid Syndrome Presenting With Life-Threatening Diffuse Alveolar Hemorrhage

TL;DR

A 33-year-old man with a life-threatening lung condition was diagnosed with antiphospholipid syndrome and successfully treated with corticosteroids and anticoagulation.

Contribution

This case highlights APS as a rare but critical cause of diffuse alveolar hemorrhage and emphasizes the importance of early diagnosis and treatment.

Findings

The patient's symptoms and lab results confirmed antiphospholipid syndrome as the cause of diffuse alveolar hemorrhage.

High-dose corticosteroids and anticoagulation led to successful recovery and long-term remission.

Early recognition and aggressive treatment improved outcomes in this life-threatening condition.

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.

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