Table 3

EULAR recommendations for the management of LVV—2018 update

LoESoRFV (%)LoA (0–10)
Overarching principles
APatients with LVV should be offered best care which must be based on a shared decision between the patient and the rheumatologist, considering efficacy, safety and costsn.a.n.a.n.a.9.7±0.7
BPatients should have access to education focusing on the impact of LVV, it’s key warning symptoms and its treatment (including treatment-related complications)n.a.n.a.n.a.9.7±0.7
C Patients with LVV should be screened for treatment-related and cardiovascular comorbidities. We recommend prophylaxis and life-style advice to reduce cardiovascular risk and treatment-related complicationsn.a.n.a.n.a.9.8±0.7
Recommendations
1All patients presenting with signs and symptoms suggestive of GCA should be urgently referred to a specialist team for further multidisciplinary diagnostic work-up and management2bC919.2±2.1
2All patients presenting with signs and symptoms suggestive of TAK should be referred to a specialist team for multidisciplinary diagnostic work-up and management5D1009.6±0.9
3A suspected diagnosis of LVV should be confirmed by imaging (ultrasound* or MRI§ for temporal or other cranial arteries, ultrasound, CT, PET-CT or MRI for the aorta/extracranial arteries#) or histology (TAB*)*1b
§2b
#3
*A
§B
#C
*100
§100
#100
9.5±0.9
9.3±1.2
9.6±0.8
4High dose glucocorticoid (GC) therapy (40–60 mg/day prednisone-equivalent) should be initiated immediately for induction of remission in active GCA& or TAK+
Once disease is controlled, we recommend tapering the GC dose to a target dose of 15–20 mg/day within 2–3 months and after 1 year to ≤5 mg/day (for GCA) and to ≤10 mg/day (for TAK)
&4
+5
5
&C
+D
D
&100
+100
87
9.8±0.6
9.8±0.5
9.5±0.9
5Adjunctive therapy should be used in selected patients with GCA (refractory or relapsing disease, the presence or an increased risk of GC related adverse effects or complications) using tocilizumab**. Methotrexate may be used as an alternative§§ **1b
§§1a-
**A
§§A
**100
§§100
9.4±0.8
9.4±0.8
6Non-biologic disease modifying agents should be given in combination with GC in all patients with TAK#. Tocilizumab or TNF-inhibitors can be considered in case of relapsing or refractory disease despite conventional DMARD therapy# 4C1009.4±1.2
7In case of major relapse (either with signs or symptoms of ischaemia or progressive vascular inflammation) we recommend reinstitution or dose escalation of GC therapy as recommended for new onset disease.## For minor relapses we recommend an increase in GC dose at least to the last effective dose.* Initiation or modification of adjunctive therapy should be considered particularly after recurrent disease relapses&& ##2b
&&1b
##C
&&A
##95
&&95
9.5±1.0
9.6±1.0
8Antiplatelet or anticoagulant therapy should not be routinely used for treatment of LVV unless it is indicated for other reasons (eg, coronary heart disease or cerebrovascular disease etc). In special situations such as vascular ischaemic complications or high risk of cardiovascular disease, these might be considered on an individual basis4C1009.4±0.8
9In LVV, elective endovascular interventions or reconstructive surgery should be performed in phases of stable remission. However, arterial vessel dissection or critical vascular ischaemia requires urgent referral to a vascular team4C959.8±0.5
10Regular follow-up and monitoring of disease activity in patients with LVV is recommended, primarily based on symptoms, clinical findings and ESR/CRP levels3bC1009.6±0.6
  • The LoE was determined for different parts of each recommendation (referred to with different signs such as * or §). The level of agreement was computed on a 0–10 scale.

  • DMARD, disease modifying anti-rheumatic drug; FV, final vote (% of expert panel members that agreed to the recommendation); LVV, large vessel vasculitis; LoA, level of agreement; LoE, level of evidence; NA, not applicable; SoR, strength of recommendation; TAB, temporal artery biopsy; TAK, Takayasu arteritis; TNF, tumour necrosis factor.