ACR criteria[1,2] | SLICC criteria[3] | ||
(4 of 11 criteria)* | (4 of 17 criteria, including at least 1 clinical criterion and 1 immunologic criterion;¶ OR biopsy-proven lupus nephritisΔ) | ||
Criterion | Definition | Criterion | Definition |
Clinical criteria | |||
Malar rash | Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds | Acute cutaneous lupus | Lupus malar rash (do not count if malar discoid); bullous lupus; toxic epidermal necrolysis variant of SLE; maculopapular lupus rash; photosensitive lupus rash (in the absence of dermatomyositis); OR subacute cutaneous lupus (nonindurated psoriasiform and/or annular polycyclic lesions that resolve without scarring, although occasionally with postinflammatory dyspigmentation or telangiectasias) |
Photosensitivity | Skin rash as a result of unusual reaction to sunlight, by patient history or clinician observation | ||
Discoid rash | Erythematosus raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions | Chronic cutaneous lupus | Classic discoid rash; localized (above the neck); generalized (above and below the neck); hypertrophic (verrucous) lupus; lupus panniculitis (profundus); mucosal lupus; lupus erythematosus tumidus; chilblains lupus; OR discoid lupus/lichen planus overlap |
Nonscarring alopecia | Diffuse thinning or hair 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 ulceration, usually painless, observed by a clinician | Oral or nasal ulcers | Palate, buccal, tongue, OR nasal ulcers (in the absence of other causes, such as vasculitis, Behçet syndrome, infection [herpesvirus], inflammatory bowel disease, reactive arthritis, and acidic foods) |
Arthritis | Nonerosive arthritis involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion | Joint disease | Synovitis involving 2 or more joints, characterized by swelling or effusion OR |
Tenderness in 2 or more joints and at least 30 minutes of morning stiffness | |||
Serositis | Pleuritis – Convincing history of pleuritic pain or rubbing heard by a clinician or evidence of pleural effusion OR | Serositis | Typical pleurisy for more than 1 day, pleural effusions, or pleural rub, OR |
Pericarditis – Documented by ECG, rub, or evidence of pericardial effusion | Typical pericardial pain (pain with recumbency improved by sitting forward) for more than 1 day, pericardial effusion, pericardial rub, or pericarditis by electrocardiography in the absence of other causes, such as infection, uremia, and Dressler syndrome | ||
Renal disorder | Persistent proteinuria greater than 500 mg/24 hours or greater than 3+ if quantitation not performed OR | Renal | Urine protein-to-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hours, OR |
Cellular casts – May be red cell, hemoglobin, granular, tubular, or mixed | Red blood cell casts | ||
Neurologic disorder | Seizures OR psychosis – In the absence of offending drugs or known metabolic derangements (uremia, ketoacidosis, or electrolyte imbalance) | Neurologic | Seizures; psychosis; mononeuritis multiplex (in the absence of other known causes, such as primary vasculitis); myelitis; 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 disorder | Hemolytic anemia – With reticulocytosis OR Leukopenia – Less than 4000/mm3 total on 2 or more occasions OR Lymphopenia – Less than 1500/mm3 on 2 or more occasions OR Thrombocytopenia – Less than 100,000/mm3 (in the absence of offending drugs) | Hemolytic anemia | Hemolytic anemia |
Leukopenia or lymphopenia | Leukopenia (<4000/mm3 at least once) (in the absence of other known causes, such as Felty syndrome, drugs, and portal hypertension), OR | ||
Lymphopenia (<1000/mm3 at least once) (in the absence of other known causes, such as glucocorticoids, drugs, and infection) | |||
Thrombocytopenia | Thrombocytopenia (<100,000/mm3) 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 immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with "drug-induced lupus" syndrome | ANA | ANA level above laboratory reference range |
Immunologic disorders | Anti-DNA – Antibody to native DNA in abnormal titer OR Anti-Sm – Presence of antibody to Sm nuclear antigen OR Positive antiphospholipid antibody on:
| Anti-dsDNA | Anti-dsDNA antibody level above laboratory reference range (or >2-fold the reference range if tested by ELISA) |
Anti-Sm | Presence of antibody to Sm nuclear antigen | ||
Antiphospholipid | Antiphospholipid antibody positivity as determined by any of the following: Positive test result for lupus anticoagulant; false-positive test result for rapid plasma reagin; medium- or high-titer anticardiolipin antibody level (IgA, IgG, or IgM); or positive test result for anti-beta 2-glycoprotein I (IgA, IgG, or IgM) | ||
Low complement | Low C3; low C4; OR low CH50 | ||
Direct Coombs test | Direct Coombs test in the absence of hemolytic anemia |
ACR: American College of Rheumatology; SLICC: Systemic Lupus International Collaborating Clinics; SLE: systemic lupus erythematosus; ECG: 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 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 4 of the clinical and immunologic criteria used in the SLICC classification criteria, including at least 1 clinical criterion and 1 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.Do you want to add Medilib to your home screen?