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[Thoracic manifestations of Behcet's disease]
A Ghembaza1, S Boussouar2, D Saadoun1
1Sorbonne Université, Pitié-Salpêtrière University Hospital, Paris, France; Department of Internal Medicine and Clinical Immunology, Paris, France; Centre de référence des maladies auto-immunes systémiques rares, centre de référence des maladies auto-inflammatoires et de l'amylose inflammatoire, AP-HP, 75013 Paris, France; Institut national de la santé et de la recherche médicale, Paris, France; Inserm, UMR_S 959, 75013, Paris, France; RHU IMAP, CNRS, FRE3632, 75005 Paris, France.
Revue Des Maladies Respiratoires
|June 6, 2022
Summary
Behcet
Area of Science:
- Vascular Medicine
- Rheumatology
- Immunology
Background:
- Behcet's disease (BD) is a multisystemic vasculitis affecting various blood vessels.
- While joint and skin issues are common, vascular involvement is the primary cause of mortality.
- Arterial manifestations, including aneurysms and thrombosis, occur in 5-10% of BD patients.
Purpose of the Study:
- To review the arterial manifestations of Behcet's disease.
- To discuss diagnostic considerations and treatment strategies for arterial involvement in BD.
- To highlight the prognosis and long-term management of severe arterial manifestations.
Main Methods:
- Literature review of Behcet's disease with a focus on vascular and arterial manifestations.
- Analysis of clinical presentations, diagnostic approaches, and therapeutic interventions.
- Evaluation of prognosis and relapse rates associated with arterial involvement.
Main Results:
- Arterial aneurysms are often multiple, commonly affecting pulmonary arteries, aorta, and lower limb arteries.
- Pulmonary artery aneurysms carry a severe prognosis (up to 26% mortality), though outcomes improve with early diagnosis and immunosuppressants.
- Severe arterial manifestations require high-dose corticosteroids, cyclophosphamide, or anti-TNF agents, potentially with anticoagulation and endovascular treatment.
Conclusions:
- Vascular involvement, particularly arterial aneurysms, is a critical and potentially fatal aspect of Behcet's disease.
- Prompt diagnosis and aggressive immunosuppressive therapy, including endovascular interventions for severe cases, are crucial for improving outcomes.
- Long-term maintenance therapy is essential due to a significant risk of relapse in patients with severe arterial manifestations.
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