Arthritis: Synovitis in at least two
joints ( 34).
Neurologic: Delirium (12), psychosis ( 20), and seizure ( 34).
Serositis: Pleural or pericardial effusion ( 34) and acute pericarditis ( 38).
Hematologic: Leukopenia (12),
thrombocytopenia ( 26), and autoimmune hemolysis ( 28).
Renal: Proteinuria more than 0.5
g/24 hours ( 27), renal biopsy with
class II or V lupus nephritis (55),
and renal biopsy with class III or IV
lupus nephritis (74).
The immunologic domains
G more than 40 GPL units, an-
ti-beta2GP1 IgG more than 40
units, or lupus anticoagulant positive ( 13).
Complement proteins: Low C3 or
low C4 ( 19) and low C3 and low C4
Highly specific antibodies: Anti-dsDNA antibody ( 38) and anti-Smith antibody ( 40).
Screening 10 domains with their
attendant components may seem a
bit clunky now, Dr. Johnson noted,
but the final iteration should be
more streamlined. Plus, she said,
the system will be presented on a
computer application that makes
calculation much easier. “We’re
aiming for feasibility and simplici-
ty, but, at the same time, when you
have a complex disease, you don’t
want oversimplification. You may
lose sensitivity and specificity.”
After further streamlining, Dr.
Johnson said, the next step will be
validating in a large retrospective
patient cohort. “Right now, we are
still collecting data for the valida-
tion cohort, which will be drawn
from 36 centres. We’ll analyse sen-
sitivity and specificity, comparing
this system with the other two. We
hope to present all these data at
the ACR meeting in the fall.”
While research classification is
the system’s raison d’être, it will
undoubtedly influence diagnosis
and clinical assessment as well, Dr.
Johnson said. “ACR and EULAR are
very clear that they only support the
validation of classification criteria.
The diagnosis of SLE is still within
the hands of the physician. But, we
know that classification criteria do
inform our concept of the disease,
so it’s likely these will shift the way
we think about diagnosing lupus
as well. We do hope to identify patients with earlier disease, so they
have the opportunity to be involved
in research” that may modify their
disease course and, ultimately,
prevent permanent damage and improve quality of life.
Dr. Johnson had no disclosures
related to the development of the
The aim is to get patients into trials at earlier stages
This joint ACR/EULAR effort is very large, involving
over 40 international SLE experts and centres, including many Systemic Lupus International Collaborating
Clinics Criteria members. The
goals are to develop new criteria
that will be both sensitive and
specific for SLE, which is a very
heterogeneous and often elusive
disease, using newer rigorous
expert opinion–based and da-ta-driven methodologies (as has
been accomplished recently for
rheumatoid arthritis, scleroderma,
The new criteria will include a
point system on a continuous scale with a cutoff for
“definite SLE” decided upon by SLE expert consensus
and many cases used for validation. There is a particular interest in trying to include SLE patients at earlier
stages of disease, as there is impetus in SLE clinical
trials and studies to test strategies and medications
capable of preventing the longer-term sequelae and
complications of the disease.
This is a tall order! While genetic and cytokine-
based biomarkers are being developed for the iden-
tification of SLE, unfortunately, we were not able to
incorporate them as they are not available for routine
use at this time. The SLE classification criteria are
used worldwide for inclusion in clinical trials and
The work has been preceding in phases: phase
1, item generation; phase 2, item reduction and
definition of criteria; phase 3, multicriteria decision
analysis and threshold identification; and phase
4, validation. Phases 1-3 involved many iterative,
group discussions, data collection and review, and
novel “forced choice” methodologies for arriving at
group consensus. In phase 4 (validation), the goal
is to compare classification using these criteria with
the existing ACR and SLICC criteria, as well as SLE
expert physician diagnosis. We will see how they do
after all this effort!
Dr. Karen H. Costenbader is the lupus programme
director at Brigham and Women’s Hospital, Boston,
USA. She is a member of the classification criteria
steering committee and is the senior author on a paper describing the process to develop the draft classification criteria (Arthritis Care Res. 2017 July 10. doi: