# EULAR 2018 — Vascular Behçet, Immunosuppression and Anticoagulation

_Last updated: 2026-06-21_

## Citation
- **Title:** 2018 update of the EULAR recommendations for the management of Behçet's syndrome.
- **Authors:** Gulen Hatemi; Robin Christensen; Dongsik Bang; Bahram Bodaghi; Aykut Ferhat Celik; Farida Fortune; Julien Gaudric; Ahmet Gul; Ina Kötter; Pietro Leccese; Alfred Mahr; Robert Moots; et al.
- **Journal:** Annals of the Rheumatic Diseases, 2018
- **PubMed:** https://pubmed.ncbi.nlm.nih.gov/29625968/
- **DOI:** 10.1136/annrheumdis-2018-213225
- **Guideline Central summary:** https://www.guidelinecentral.com/guideline/5147305/

## Key EULAR vascular Behçet points
- Vascular Behçet is treated primarily as **inflammation-driven vascular disease**.
- **Immunosuppressives should be continued as maintenance.**
- **Anticoagulants may be added** if:
  - bleeding risk is low, and
  - coexistent **pulmonary artery aneurysms are ruled out**.
- In cerebral venous sinus thrombosis with vision-threatening intracranial hypertension, urgent surgical/interventional options should be considered.

## Why the pulmonary artery aneurysm point matters
In Behçet, arterial aneurysms — especially pulmonary artery aneurysms — can bleed catastrophically. Anticoagulation can be dangerous if such aneurysms are present. Therefore, anticoagulation decisions should be linked to imaging/risk assessment, not only to whether thrombosis occurred.

## Implication for Eliquis/Apixaban discussion
EULAR does **not** provide a simple rule to stop anticoagulation once immunosuppression starts. The safer interpretation:
- Immunosuppression is the disease-control core.
- Anticoagulation is individualized.
- Reducing/stopping Eliquis should require objective stability and explicit clinician agreement.

## Practical monitoring before considering anticoagulation reduction
- No new DVT/SVT/thrombophlebitis for an agreed interval.
- CRP/BSG and clinical inflammation controlled.
- Venous imaging stable/improved where relevant.
- Arterial/pulmonary aneurysm status known.
- Clear plan from rheumatology + angiology/hämostaseology.

## Caveat
The EULAR recommendation is a guideline-level synthesis but acknowledges limited high-quality trial evidence in vascular Behçet. Newer studies after 2018 increasingly suggest potential benefit of **IS + AC** in selected patients.