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Anticoagulant Treatment May Decrease the Relapse Rate of Pulmonary Arterial Involvement in Behçet's Disease.

Kerem Yiğit Abacar1, Ayşe Elif Boncukcuoglu2, Aysun Aksoy3

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Pulmonary arterial involvement in Behçet's disease (BD) primarily presents as thrombosis. Adding anticoagulants to immunosuppressive therapy significantly reduces relapse risk in BD patients with pulmonary arterial involvement.

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Area of Science:

  • Rheumatology
  • Cardiology
  • Pulmonology

Background:

  • Pulmonary arterial involvement (PAI) is a critical cause of mortality in Behçet's disease (BD).
  • Understanding the clinical spectrum and recurrence factors of PAI in BD is crucial for patient management.

Purpose of the Study:

  • To investigate the clinical features, disease course, and risk factors for recurrence in patients with Behçet's disease-associated pulmonary arterial involvement.
  • To identify predictors of relapse in BD-associated PAI.

Main Methods:

  • Retrospective analysis of medical records from Behçet's disease patients treated between 1990 and 2023.
  • Classification of pulmonary arterial involvement into thrombosis or aneurysm.
  • Multivariate Cox regression analysis to determine factors influencing relapse risk.

Main Results:

  • Out of 1350 BD patients, 110 (8.1%) had PAI. Pulmonary arterial thrombosis occurred in 94.5% of cases, while aneurysms were rare (6.6%).
  • Asymptomatic PAI patients were younger, while symptomatic patients showed higher rates of female gender and recurrence.
  • Approximately 28.2% of patients experienced at least one PAI relapse; not initiating anticoagulants independently increased relapse risk (HR, 4.36).

Conclusions:

  • Pulmonary arterial thrombosis is the predominant manifestation of PAI in BD, with aneurysms being infrequent.
  • Despite immunosuppressive therapy, a significant proportion of patients (around one-third) experience relapses.
  • Anticoagulant therapy, when combined with immunosuppressive treatment, substantially lowers the relapse rate in BD patients with PAI.