Clinical Insights into Diffuse Alveolar Hemorrhage in Antiphospholipid Syndrome. Review uri icon

Overview

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. 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.

publication date

  • September 6, 2019

Research

keywords

  • Antiphospholipid Syndrome
  • Hemorrhage
  • Lung Diseases

Identity

PubMed Central ID

  • PMC7102334

Scopus Document Identifier

  • 85071896734

Digital Object Identifier (DOI)

  • 10.1111/jth.12363

PubMed ID

  • 31493005

Additional Document Info

volume

  • 21

issue

  • 10