This systemic lupus diagnostic criteria SLICC calculator helps the classification and diagnosis of SLE based on clinical and immunological criteria. In the text below the form there is information on the SLICC, along with instructions on how to interpret the criteria that is present.

Clinical Criteria

1Acute cutaneous lupus
2Chronic cutaneous lupus
3Oral ulcers
4Nonscarring alopecia
5Synovitis
6Serositis
7Renal manifestations
8Biopsy-proven lupus nephritis
9Neurologic manifestations
10Hemolytic anemia
11Leukopenia or Lymphopenia
12Thrombocytopenia

Immunological Criteria

1ANA above ref. range
2Anti-dsDNA above ref. range
3Anti-Sm
4Antiphospholipid antibody
5Low complement
6Direct Coombs test

How does the systemic lupus diagnostic criteria SLICC calculator work?

This health tool is based on the 2012 classification created by the Systemic Lupus International Collaborating Clinics (SLICC) which is an international group that performs SLE clinical research.

Classification criteria is generally used to ensure a constant definition of the disease, to help in diagnosis and during clinical studies.

The SLICC consists in a revision and validation of the American College of Rheumatology (ACR) SLE classification criteria. The new developments in SLE immunology have demanded an update of the lupus diagnostic criteria. It also aims to clarify and remove the possible duplication of highly correlated cutaneous lupus terms and to introduce the neurologic manifestations.

The criteria are divided into two groups: clinical and immunological. The recommendation from the original study is that the criteria are cumulative and need not be present concurrently.

SLICC clinical criteria:

Criterion Instruction
Acute cutaneous lupus

Including:

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 psoriaform and/or annular polycyclic lesions that resolve without scarring, although occasionally with postinflammatory dyspigmentation or telangiectasias).

Chronic cutaneous lupus

Including:

classical discoid rash

localized (above the neck)

generalized (above and below the neck)

hypertrophic (verrucous) lupus

lupus panniculitis (profundus)

mucosal lupus

lupus erythematosus tumidus

chillblains lupus

discoid lupus/lichen planus overlap

Oral ulcers

Palate

Buccal

Tongue

OR Nasal ulcers in the absence of other causes, such as vasculitis, Behcets, infection (herpes), inflammatory bowel disease, reactive arthritis, and acidic foods.

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.

Synovitis Involving two or more joints, characterized by swelling or effusion OR tenderness in 2 or more joints and thirty minutes or more of morning stiffness.
Serositis Typical pleurisy for more than 1 day or pleural effusions or pleural rub.

OR

Typical pericardial pain (pain with recumbence improved by sitting forward) for more than 1 day or pericardial effusion or pericardial rub or pericarditis by EKG in the absence of other causes, such as infection, uremia, and Dressler's pericarditis.

Renal manifestations Urine protein/creatinine (or 24 hr urine protein) representing 500 mg of protein/24 hr.

OR

Red blood cell casts

Biopsy-proven lupus nephritis No instruction.
Neurologic manifestations

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.

Acute confusional state in the absence of other causes, including toxic-metabolic, uremia, drugs.

Hemolytic anemia No instruction.
Leukopenia (< 4000/mm3) OR

Lymphopenia (< 1000/mm3)

Leukopenia: at least once and in the absence of other known causes such as Felty's, drugs and portal hypertension.

OR

Lymphopenia: At least once and in the absence of other known causes such as corticosteroids, drugs and infection.
Thrombocytopenia (<100,000/mm3) At least once and in the absence of other known causes such as drugs, portal hypertension and TTP.

SLICC immunological criteria:

Criterion Instruction
ANA Above laboratory reference range
Anti-dsDNA Above laboratory reference range, except ELISA: twice above laboratory reference range
Anti-Sm No instruction.
Antiphospholipid antibody Any of the following:

lupus anticoagulant

false-positive RPR

medium or high titer anticardiolipin (IgA, IgG or IgM)

anti-β2 glycoprotein I (IgA, IgG or IgM)

Low complement Low C3, C4, CH50
Direct Coombs test In the absence of hemolytic anemia

SLICC criteria is considered positive in two cases:

1. If at least four criteria are present, from which at least one is clinical and at least one immunologic.

2. Lupus nephritis as sole clinical criterion in the presence of ANA or anti-dsDNA antibodies. Lupus nephritis proved by biopsy and in the presence of antibodies was considered as sufficient as standalone clinical criterion because of its representativeness.

Compared to the ACR classification, the SLICC resulted in fewer misclassifications and showed greater sensitivity (94% compared to 86%) and similar specificity (approx. 92%).

One of the criticism of the method is that it is not very timely in application (for example, need to wait for laboratory results) and can delay diagnosis in the case of some patients or be avoided entirely by clinicians because of this.

There is also a recent (2015) revision of the SLICC, on a point based system, that needs to be put under further validation to determine whether it can perform better than the original 2012 version.

There are other indices for lupus erythematosus such as the SLE Disease Activity Index (SLEDAI), which evaluates changes in the characteristic symptoms of this immune deficiency disorder.

References

1) Petri M. et al. (2012) Derivation and Validation of Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheum; 64(8): 2677–2686.

2) Pons-Estel GJ, Wojdyla D, McGwin G, Magder LS, Petri MA, Pons-Estel BA, Alarcón GS. (2014) The American College of Rheumatology and the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus in two multiethnic cohorts: a commentary. Lupus, 23 (1): 3-9.

3) Lythgoe H, Morgan T, Heaf E, Lloyd O, Beresford MW. (2016) G260 Evaluation of the ACR and SLICC classification criteria in juvenile systemic lupus erythematosus: a longitudinal analysis. Arch Dis Child;101:A143-A144.

4) Tiao J, Feng R, Carr K, Okawa J, Werth VP. (2016) Using the American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC) criteria to determine the diagnosis of systemic lupus erythematosus (SLE) in patients with subacute cutaneous lupus erythematosus (SCLE). J Am Acad Dermatol; 74(5):862-9.

14 Dec, 2016 | 0 comments

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