Systemic lupus erythematosus diagnostic criteria

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahshid Mir, M.D. [2]

Overview

Based on SLICC criteria, for an SLE diagnosis: Patient should have either at least 4 of 17 criteria, including at least 1 of the 11 clinical criteria and one of the six immunologic criteria, or that the patient has biopsy-proven nephritis compatible with SLE in the presence of antinuclear antibodies (ANA) or anti-double-stranded DNA (dsDNA) antibodies.

Diagnostic criteria

In 2012, Systemic Lupus International Collaboration Criteria (SLICC) developed a new criteria for SLE diagnosis. SLICC criteria for the classification of systemic lupus erythematosus was developed based on the old ACR criteria for the classification of systemic lupus erythematosus to address a more sensitive diagnostic criteria and also to cover weaknesses of the previous ACR criteria.

Based on SLICC criteria, diagnosis of SLE is defined as:

• At least 4 of 17 criteria, including at least 1 of the 11 clinical criteria and one of the six immunologic criteria (for each criterion, any bullet is considered as 1 clinical criteria), 

OR

• Biopsy-proven nephritis compatible with SLE in the presence of antinuclear antibodies (ANA) or anti-double-stranded DNA (dsDNA) antibodies.

	Criterion	Definition
Clinical	Acute cutaneous lupus	Lupus malar rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds Bullous lupus Toxic epidermal necrolysis variant of SLE Maculopapular lupus rash Photosensitive lupus rash (in the absence of dermatomyositis); Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation   Subacute cutaneous lupus Nonindurated psoriaform and/or annular polycyclic lesions that resolve without scarring, although occasionally with postinflammatory dyspigmentation or telangiectasias
	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 or nasal ulcers	Palate, buccal, tongue, or nasal ulcers (in the absence of other causes, such as vasculitis, Behçet's disease, infection [herpesvirus], inflammatory bowel disease, reactive arthritis, and acidic foods)
	Joint disease	Synovitis involving two or more joints, characterized by swelling or effusion Tenderness in two or more joints and at least 30 minutes of morning stiffness
	Serositis	Typical pleurisy for more than one day, pleural effusions, or pleural rub, OR Typical pericardial pain (pain with recumbency improved 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	Urine protein-to-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hours, OR Red blood cell casts
	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	Hemolytic anemia	Hemoglobin less than 12 g/dL in females and 13 g/dL in males
	Leukopenia or lymphopenia	Leukopenia (<4000/mm3 at least once) (in the absence of other known causes, such as Felty's 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
	ANA	ANA level above laboratory reference range
	Anti-dsDNA	Anti-dsDNA antibody level above laboratory reference range (or >twofold the reference range if tested by ELISA)
	Anit-SM	Presence of antibody to Sm nuclear antigen
Immunologic	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) Positive test result for anti-beta 2-glycoprotein I (IgA, IgG, or IgM)
	Low complement	Low C3 OR Low C4 OR Low CH50
	Direct Coombs' test	Direct Coombs' test in the absence of hemolytic anemia