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Clinical Insights into Diffuse Alveolar Hemorrhage in Antiphospholipid Syndrome

  • Sarah Abramson Stoots
  • Lindsay Lief
  • Doruk ErkanEmail author
Antiphospholipid Syndrome (S Zuily, Section Editor)
  • 49 Downloads

Abstract

Purpose of Review

Diffuse alveolar hemorrhage (DAH) is a rare but devastating manifestation of antiphospholipid syndrome (APS) patients with or without other systemic autoimmune diseases. Data regarding diagnosis and treatment are limited to case series. We review diagnostic and therapeutic strategies employed in APS patients with DAH and discuss our experience in managing these complex patients.

Recent Findings

Pulmonary capillaritis likely contributes to the pathogenesis, however is only observed in half of the biopsies. Corticosteroids induce remission in the majority of patients, however almost half recur and require a steroid-sparing immunosuppressive to maintain remission. Cyclophosphamide- or rituximab-based regimens achieve the highest remission rates (50%); other strategies include intravenous immunoglobulin, plasmapheresis, mycophenolate mofetil, and/or azathioprine.

Summary

Given the rarity of DAH in APS, treatment is guided by interdisciplinary experience. Why certain patients achieve full remission with corticosteroids while others require immunosuppressive agents is unknown; future research should focus on the pathophysiology and optimal management.

Keywords

Antiphospholipid syndrome Antiphospholipid antibodies Alveolar hemorrhage Pulmonary hemorrhage Bleeding Capillaritis 

Notes

References

Papers of particular interest, published recently, have been highlighted as: •• Of major importance

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Sarah Abramson Stoots
    • 1
  • Lindsay Lief
    • 2
  • Doruk Erkan
    • 3
    Email author
  1. 1.Department of Medicine, Weill Cornell MedicineHospital for Special SurgeryNew YorkUSA
  2. 2.Department of Medicine, Division of Pulmonary and Critical CareWeill Cornell MedicineNew YorkUSA
  3. 3.Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Cornell MedicineNew YorkUSA

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