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

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.