EULAR overhauls large-vessel vasculitis
“thankfully rare” form of APS that was
first described in the early 1990s.
Although fewer than 1% of the
APS adult population have CAPS
(Arthritis Rheum. 2002;46[ 4]:1019-
27), it’s a condition in which several
thrombotic events occur simultaneously, affecting multiple systems or
organs and which can be life threatening if not treated quickly.
New treatment guidelines
for catastrophic APS
During a separate clinical science
session at the Congress, Dr. Cervera
discussed the development of treat-
ment guidelines for CAPS, noting that
this had been one of the focus points
of the McMaster RARE-Bestpractices
project group in 2016. The group se-
lected CAPS for a pilot exercise in
guideline development for a rare dis-
ease and published their recommen-
dations in 2018 (J Thromb Haemost.
2018;16:1656-64). Ten recommenda-
tions were developed, most of which
were conditional, Dr. Cervera said,
because of the lack of, or very low
certainty of, the evidence.
The new EULAR 2019 adult APS
recommendations now include CAPS
and recommendation number 12 is
split into two parts. The first, part A,
states that prompt treatment of infec-
tions is needed in all patients positive
for aPL antibodies and that antico-
agulation should have minimal inter-
ruption or be used at level to help
prevent the development of CAPS.
The second, part B, states that the
first-line treatment of CAPS should
be a triple combination therapy of
glucocorticoids, heparin, and plasma
exchange, or intravenous immunoglobulins, rather than single-agent
treatment. Plus, it says that any triggering factor should be treated accordingly.
“Finally,” Dr. Cervera said, “in pa-
tients with refractory CAPS, B-cell
depletion with rituximab or comple-
ment inhibitors, for example eculi-
zumab, may be considered.”
Dr. Tektonidou and Dr. Cervera re-
ported having no relevant conflicts of
Continued from // previous page
Ten years after they were last published, a EULAR expert task force has revamped guidance on how to manage patients
with large-vessel vasculitis.
The “substantial revision” of the
2009 recommendations (Ann Rheum
Dis. 2009;68[ 3]:310-7) was based
on two new systematic literature reviews, focusing on general management and treatments, respectively.
These were performed “without
limits,” task force member Prof. Bernhard Hellmich said at the European
Congress of Rheumatology.
The reason for starting from scratch
was the amount of “high-impact”
data that have been published in
the intervening years, including the
results of several randomised clinical
trials, and also the fact that EULAR
had released guidance on imaging in
large-vessel vasculitis (LVV) in 2018
(Ann Rheum Dis. 2018;77[ 5]:636-43).
The new recommendations, which
are published in Annals of the Rheumatic Diseases (Ann Rheum Dis.
2019 Jul 3. doi: 10.1136/annrheum-
dis-2019-215672), now include three
overarching principles, said Prof.
Hellmich, who is the chief physician
of the Clinic for Internal Medicine,
Rheumatology and Immunology at
Medius Kliniken in Kirchheim unter
The first overarching principle says
that patients with LVV “should be of-
fered best care which must be based
on a shared decision between the
patient and the rheumatologist, con-
sidering, of course, efficacy, safety,
and costs,” he stated.
“Second, patients should have access to education focusing on the
impact of LVV, its key warning symptoms, and its treatment, including
treatment-related complications,” he
“Third, patients with large-vessel
vasculitis should be screened for
treatment-related comorbidities and
also cardiovascular comorbidities, and
we recommend prophylaxis and life-
style advice to reduce cardiovascular
risks and treatment-related complica-
Another key change is that there
are 10 rather than 15 recommenda-
tions. These include new recom-
mendations on early diagnosis,
management, and the treatment of
The first two recommendations
highlight the need for specialist referral and multidisciplinary management of giant cell arteritis (GCA) and
Takayasu arteritis (TAK).
Recommendation 3 offers advice
on confirming a diagnosis of LVV by
Prof. Bernhard Hellmich
Continued // 34