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Classification criteria for systemic lupus erythematosus

ACR criteria for the classification of SLICC criteria for the classification
systemic lupus erythematosus [1,2] of systemic lupus erythematosus [3]
(4 of 11 criteria)* (4 of 17 criteria, including at least one clinical
criterion and one immunologic criterion; ¶ OR
biopsy-proven lupus nephritis Δ)

Criterion Definition Criterion Definition

Clinical criteria

Malar rash Fixed erythema, flat or Acute cutaneous Lupus malar rash (do not
raised, over the malar lupus count if malar discoid);
eminences, tending to spare bullous lupus; toxic
the nasolabial folds epidermal necrolysis variant
of SLE; maculopapular lupus
rash; photosensitive lupus
rash (in the absence of
dermatomyositis); OR
subacute cutaneous lupus
Photosensitivity Skin rash as a result of (nonindurated psoriaform
unusual reaction to sunlight, and/or annular polycyclic
by patient history or clinician lesions that resolve without
observation scarring, although
occasionally with
postinflammatory
dyspigmentation or
telangiectasias)

Discoid rash Erythematosus raised patches Chronic cutaneous Classic discoid rash; localized
with adherent keratotic lupus (above the neck);
scaling and follicular plugging; generalized (above and
atrophic scarring may occur below the neck);
in older lesions hypertrophic (verrucous)
lupus; lupus panniculitis
(profundus); mucosal lupus;
lupus erythematosus
tumidus; chilblains lupus; OR
discoid lupus/lichen planus
overlap

Nonscarring Diffuse thinning or hair


alopecia fragility with visible broken
hairs (in the absence of other
causes, such as alopecia
areata, drugs, iron
deficiency, and androgenic
alopecia)

Oral ulcers Oral or nasopharyngeal Oral or nasal Palate, buccal, tongue, OR


ulceration, usually painless, ulcers nasal ulcers (in the absence
observed by a clinician of other causes, such as
vasculitis, Behçet's disease,

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infection [herpesvirus],
inflammatory bowel disease,
reactive arthritis, and acidic
foods)

Arthritis Nonerosive arthritis involving Joint disease Synovitis involving two or


two or more peripheral joints, more joints, characterized by
characterized by tenderness, swelling or effusion OR
swelling, or effusion
Tenderness in two or more
joints and at least 30 minutes
of morning stiffness

Serositis Pleuritis – Convincing history Serositis Typical pleurisy for more


of pleuritic pain or rubbing than one day, pleural
heard by a clinician or effusions, or pleural rub, OR
evidence of pleural effusion
OR

Pericarditis – Documented by Typical pericardial pain (pain


EKG, rub, or evidence of with recumbency improved
pericardial effusion by sitting forward) for more
than one day, pericardial
effusion, pericardial rub, or
pericarditis by
electrocardiography in the
absence of other causes, such
as infection, uremia, and
Dressler's syndrome

Renal disorder Persistent proteinuria greater Renal Urine protein-to-creatinine


than 500 mg/24 hours or ratio (or 24-hour urine
greater than 3+ if protein) representing 500 mg
quantitation not performed protein/24 hours, OR
OR

Cellular casts – May be red Red blood cell casts


cell, hemoglobin, granular,
tubular, or mixed

Neurologic Seizures OR psychosis – In Neurologic Seizures; psychosis;


disorder the absence of offending mononeuritis multiplex (in
drugs or known metabolic the absence of other known
derangements (uremia, causes, such as primary
ketoacidosis, or electrolyte vasculitis); myelitis;
imbalance) peripheral or cranial
neuropathy (in the absence
of other known causes, such
as primary vasculitis,
infection, and diabetes
mellitus); OR acute
confusional state (in the
absence of other causes,
including toxic/metabolic,
uremia, drugs)

Hematologic Hemolytic anemia – With Hemolytic anemia Hemolytic anemia


disorder reticulocytosis OR
Leukopenia or Leukopenia (<4000/mm 3 at
Leukopenia – Less than lymphopenia least once) (in the absence of
4000/mm 3 total on two or other known causes, such as

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more occasions OR Felty's syndrome, drugs, and


Lymphopenia – Less than portal hypertension), OR
1500/mm 3 on two or more Lymphopenia (<1000/mm 3
occasions OR at least once) (in the absence
Thrombocytopenia – Less of other known causes, such
than 100,000/mm 3 (in the as glucocorticoids, drugs, and
absence of offending drugs) infection)

Thrombocytopenia Thrombocytopenia
(<100,000/mm 3) at least
once in the absence of other
known causes, such as drugs,
portal hypertension, and
thrombotic thrombocytopenic
purpura

Immunologic criteria

ANA An abnormal titer of ANA by ANA ANA level above laboratory


immunofluorescence or an reference range
equivalent assay at any point
in time and in the absence of
drugs known to be associated
with "drug-induced lupus"
syndrome

Immunologic Anti-DNA – Antibody to native Anti-dsDNA Anti-dsDNA antibody level


disorders DNA in abnormal titer OR above laboratory reference
Anti-Sm – Presence of range (or >twofold the
antibody to Sm nuclear reference range if tested by
antigen OR ELISA)

Positive finding of Anti-Sm Presence of antibody to Sm


antiphospholipid antibody nuclear antigen
based on an abnormal serum
level of IgG or IgM Antiphospholipid Antiphospholipid antibody
anticardiolipin antibodies, on positivity as determined by
a positive test result for lupus any of the following: Positive
anticoagulant using a test result for lupus
standard method, or on a anticoagulant; false-positive
false-positive serologic test test result for rapid plasma
for syphilis known to be reagin; medium- or high-titer
positive for at least six anticardiolipin antibody level
months and confirmed by (IgA, IgG, or IgM); or
Treponema pallidum positive test result for
immobilization or fluorescent anti-beta 2-glycoprotein I
treponemal antibody (IgA, IgG, or IgM)
absorption test

Low complement Low C3; low C4; OR low


CH50

Direct Coombs' Direct Coombs' test in the


test absence of hemolytic anemia

ACR: American College of Rheumatology; SLICC: Systemic Lupus International Collaborating Clinics; SLE:
systemic lupus erythematosus; EKG: electrocardiogram; ANA: antinuclear antibodies; Anti-Sm: anti-Smith
antibody; IgG: immunoglobulin G; IgM: immunoglobulin M; Anti-dsDNA: anti-double-stranded DNA; ELISA:
enzyme-linked immunosorbent assay; IgA: immunoglobulin A.
* For the ACR criteria, no distinction is made between clinical and immunologic criteria in determining

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whether the required number has been met. The classification is based upon 11 criteria. For the purpose of
identifying patients in clinical studies, a person is said to have SLE if any 4 or more of the 11 criteria are
present, serially or simultaneously, during any interval of observation.
¶ For the SLICC criteria, criteria are cumulative and need not be presently concurrently. A patient is classified
as having SLE if he or she satisfies four of the clinical and immunologic criteria used in the SLICC classification
criteria, including at least one clinical criterion and one immunologic criterion.
Δ Alternatively, according to the SLICC criteria, a patient is classified as having SLE if he or she has biopsy-
proven nephritis compatible with SLE in the presence of ANAs or anti-dsDNA antibodies.

References:
1. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus
erythematosus. Arthritis Rheum 1982; 25:1271.
2. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of
systemic lupus erythematosus (letter). Arthritis Rheum 1997; 40:1725.
3. Petri M, Orbai AM, Alarcón GS, et al. Derivation and validation of the Systemic Lupus International
Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012;
64:2677.

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