The first recommendations from
a rheumatology society for managing patients with Sjögren’s syndrome are nearing finalisation by a
EULAR task force, and they divide
the treatment targets into sicca
syndrome and systemic manifestations of the disease.
“In Sjögren’s, we always have
two subtypes of patients: those
who have sicca syndrome only, and
those with sicca syndrome plus systemic disease,” explained Prof. Soledad Retamozo, who presented the
current version of the recommendations at the Congress. “We wanted
to highlight that there are two types
of patients,” said Prof. Retamozo, a
rheumatologist at the University of
Córdoba (Argentina). “It’s hard to
treat patients with sicca syndrome
plus fatigue and pain because there
is no high-level evidence on how to
do this; all we have is expert opin-ion,”she said in an interview.
In fact, roughly half of the rec-
ommendations have no supporting
evidence base, as presented by
Prof. Retamozo. That starts with all
three general recommendations
• Patients with Sjögren’s should
be managed at a centre of ex-
pertise using a multidisciplinary
approach, which she said should
include ophthalmologists and
dentists to help address the
mouth and ocular manifestations
of sicca syndrome.
• Patients with sicca syndrome
should receive symptomatic relief
with topical treatments.
• Systemic treatments – glucocorticoids, immunosuppressants, and
biologicals – can be considered
for patients with active systemic
The statement’s specific recommendations start with managing oral dryness, beginning with
measuring salivary gland (SG)
dysfunction. The document next
interventions for mild SG dysfunction, pharmacological stimulation
for moderate SG dysfunction, and
a saliva substitute for severe SG
dysfunction. All three recommendations are evidence based, relying on
results from either randomised trials
or controlled studies.
The second target for topical
treatments is ocular dryness, which
starts with artificial tears, or ocular
gels or ointments, recommendations based on randomised trials.
Refractory or severe ocular dryness
should receive eye drops that contain a nonsteroidal anti-inflamma-tory drug or a glucocorticoid, based
on controlled study results, or au-tologous serum eye drops, a strategy tested in a randomised trial.
The recommendations then shift
to dealing with systemic manifestations, starting with fatigue and pain,
offering the expert recommendation
to evaluate the contribution of co-morbid diseases and assess their severity with tools such as the EULAR
Sjögren’s Syndrome Patient-Reported Index (ESSPRI) (Ann Rheum Dis.
2011 Jun;70[ 6]:968-72), the Profile of
Fatigue, and the Brief Pain Inventory.
Using evidence from randomised
trials, the recommendations tell
clinicians to consider treatment
with analgesics or pain-modifying
agents for musculoskeletal pain by
weighing the potential benefits and
adverse effects from this treatment.
For other forms of systemic dis-
ease, the recommendations offer
the expert opinion to tailor treat-
ment to the organ-specific severity
using the ESSPRI definitions. If
using glucocorticoids to treat sys-
temic disease, they should be giv-
en at the minimum effective dose
and for the shortest period of time
needed to control active systemic
disease, a recommendation based
on retrospective or descriptive
studies. Expert opinion called for
using immunosuppressive treat-
ments as glucocorticoid-sparing
options for systemic disease, and
this recommendation adds that
no particular immunosuppressive
agent stands out as best, com-
pared with all available agents.
Finally, for systemic disease
the recommendations cited evidence from controlled studies that
B-cell–targeted therapies, such as
rituximab and belimumab, may be
considered in patients with severe,
refractory systemic disease. An additional expert opinion was that the
systemic, organ-specific approach
should sequence treatments by using glucocorticoids first, followed
by immunosuppressants, and finally biological drugs.
The recommendations finish with
an entry that treatment of B-cell
lymphoma be individualised based
on the specific histopathologic subtype involved and the level of disease extension, an approach based
on results from retrospective or
The recommendations must still
undergo final EULAR review and
endorsement, Prof. Retamozo said.
EULAR nears first recommendations for
managing Sjögren’s syndrome
BY MITCHEL L. ZOLER
Prof. Soledad Retamozo