Clinical characteristics of pulmonary artery involvement in patients with Behçet’s syndrome: single-centre experience of 61 patients
- 27 April 2021
- journal article
- research article
- Published by Springer Science and Business Media LLC in Clinical Rheumatology
- Vol. 40 (10), 4127-4134
- https://doi.org/10.1007/s10067-021-05748-x
Abstract
Objective To report the clinical characteristics of pulmonary artery involvement (PAI) in patients with Behçet’s syndrome (BS) and to define the predictors of relapses. Methods We performed retrospective analysis of BS patients with PAI who fulfilled international study group criteria. Among 460 patients with vascular Behçet’s syndrome (VBS), 66 were diagnosed with PAI. For final analyses, 61 patients with PAI were included who had at least 2 follow-up visits (72.1% male, mean age at BS diagnosis 29.34 ± 10.1 years). The patient data were recorded. Relapse was defined as the reoccurrence of vascular event in any vascular structure. Factors associated with relapse were assessed by logistic regression analysis. Results There were no differences considering demographic and clinical features of the patients with and without PAI in the VBS group, except that intracardiac thrombosis was more common in the patients with PAI (19.7% vs 0.3%). Among 61 patients, 50 (82.0%) had isolated pulmonary artery thrombosis (PAT), whereas 11 (18.0%) had pulmonary artery aneurysm with or without PAT. Twenty-four (39.3%) patients experienced vascular relapse during median follow-up of 65.9 (Q1-Q3: 20.1–109.0) months. To define the factors associated with relapses, patients with isolated PAT were analysed. On multivariable logistic regression analysis, older age at BS diagnosis and anticoagulation usage seemed to be protective (OR: 0.92, 95% CI 0.86–1.02, OR: 0.34, 95% CI 0.09–1.33, respectively). Conclusion Our results indicate a higher frequency of intracardiac thrombosis in BS patients with PAI and possible efficacy of anticoagulation usage in preventing relapses. Key Points • This study shows that intracardiac and intracranial thromboses are seen more frequently in patients with PAI and the prevalence of pulmonary artery thrombosis has been increasing in the case of PAI. Furthermore, our report indicates that anticoagulation might be effective in preventing further vascular relapses.This publication has 32 references indexed in Scilit:
- Immunosuppressants reduce venous thrombosis relapse in Behçet's diseaseArthritis & Rheumatism, 2012
- Long-Term Outcome of Arterial Lesions in Behçet DiseaseMedicine, 2012
- Pulmonary Artery Involvement and Associated Lung Disease in Behçet DiseaseMedicine, 2012
- Mortality in Behçet's diseaseArthritis & Rheumatism, 2010
- Treatment of venous thrombosis associated with Behcet’s disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulationClinical Rheumatology, 2007
- Pulmonary Involvement in Behçet’s DiseaseRespiration, 2007
- Pulmonary artery aneurysms in Behçet syndromeThe American Journal of Medicine, 2004
- The Long-Term Mortality and Morbidity of Behçet SyndromeMedicine, 2003
- PULMONARY ARTERIAL ANEURYSMS IN BEHÇET'S SYNDROME: A REPORT OF 24 CASESRheumatology, 1994
- Criteria for diagnosis of Behcet's diseaseThe Lancet, 1990