EULAR recommendations for the management of large vessel vasculitis

Abstract

Objectives: To develop European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis. Methods: An expert group (10 rheumatologists, 3 nephrologists, 2 immunolgists, 2 internists representing 8 European countries and the USA, a clinical epidemiologist and a representative from a drug regulatory agency) identified 10 topics for a systematic literature search through a modified Delphi technique. In accordance with standardised EULAR operating procedures, recommendations were derived for the management of large vessel vasculitis. In the absence of evidence, recommendations were formulated on the basis of a consensus opinion. Results: Seven recommendations were made relating to the assessment, investigation and treatment of patients with large vessel vasculitis. The strength of recommendations was restricted by the low level of evidence and EULAR standardised operating procedures. Conclusions: On the basis of evidence and expert consensus, management recommendations for large vessel vasculitis have been formulated and are commended for use in everyday clinical practice.The large vessel vasculitides affect the aorta and its branches and include giant cell arteritis and Takayasu arteritis, which are anatomically, epidemiologically and clinically distinct conditions. The estimated incidence of giant cell arteritis in Europe, in individuals aged over 50 years of age varies between 32 and 290/million/year, making it the commonest primary systemic vasculitis in adulthood.

A rare case of Takayasu's arteritis in pregnancy

“…5 EULAR recommends the starting dose of glucocorticoids 1mg/kg body weight for four weeks and then to taper them off. 5 We followed the same protocol in our case.…”

“…4 Whereas, steroids with tapering doses are the mainstay of medical treatment; the refractory cases require additional therapy. 5 Reconstructive vascular surgery is limited to the severe and irreversible stenotic lesions. 3 Here we describe the course and management of pregnancy in a woman with Takayasu's arteritis.…”

Acute upper limb ischemia, a rare presentation of giant cell arteritis

“…2 The European League Against Rheumatism guidelines for the management of GCA recommend a temporal artery biopsy in all patients whenever a diagnosis is suspected (level of evidence 3, strength of recommendation C). 5 Nevertheless, Brack et al reported negative temporal artery biopsy in 42% of patients with large-vessel GCA. 6 Assie et al also observed a lower rate of positive temporal artery biopsy in patients with temporal artery GCA compared to those with involvement of upper and lower limb arteries (69% vs. 95%).…”

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

“…The recent British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BSR and BHPR) guidelines for GCA management and EULAR recommendations for the management of large vessel vasculitides provide a thorough description of available induction and maintenance treatment options [121,122]. Initial treatment consists of high oral dose steroids, which must be slowly tapered over months (grade 3C).…”

“…Takayasu arteritis is usually treated similarly to GCA, with high-oral dose steroids (1 mg/kg/day), followed by slow tapering and steroid-sparing agents, such as MTX, AZA, and cyclophosphamide (grade 3C) [122]. An open-label study and a retrospective review, along with some case reports and series, have shown promising results of anti-TNF treatment, but randomized trials are still lacking [126,127].…”