Systemic lupus erythematosus diagnostic criteria: Difference between revisions

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==Overview==
==Overview==
Based on SLICC criteria, for an SLE diagnosis, the patient should have either at least 4 of 17 criteria, including at least 1 of the 11 clinical criteria and one of the six [[Immunological|immunologic]] criteria (for each criterion, any bullet is considered as 1 clinical criteria), or a biopsy-proven [[nephritis]] compatible with [[SLE]] in the presence of [[Antinuclear antibodies|antinuclear antibodies (ANA)]] or [[Double stranded DNA antibody|anti-double-stranded DNA]] (dsDNA) [[antibodies]].


==Diagnostic criteria==
==Diagnostic criteria==
SLICC criteria for the classification of systemic lupus erythematosus
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.<ref name="pmid7138600">{{cite journal |vauthors=Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, Schaller JG, Talal N, Winchester RJ |title=The 1982 revised criteria for the classification of systemic lupus erythematosus |journal=Arthritis Rheum. |volume=25 |issue=11 |pages=1271–7 |year=1982 |pmid=7138600 |doi= |url=}}</ref><ref name="pmid9324032">{{cite journal |vauthors=Hochberg MC |title=Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus |journal=Arthritis Rheum. |volume=40 |issue=9 |pages=1725 |year=1997 |pmid=9324032 |doi=10.1002/1529-0131(199709)40:9&lt;1725::AID-ART29&gt;3.0.CO;2-Y |url=}}</ref>
# Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982; 25:1271.
 
# Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus (letter). Arthritis Rheum 1997; 40:1725.
Based on SLICC criteria, diagnosis of SLE is defined as:<ref name="pmid22553077">{{cite journal |vauthors=Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, Bruce IN, Isenberg D, Wallace DJ, Nived O, Sturfelt G, Ramsey-Goldman R, Bae SC, Hanly JG, Sánchez-Guerrero J, Clarke A, Aranow C, Manzi S, Urowitz M, Gladman D, Kalunian K, Costner M, Werth VP, Zoma A, Bernatsky S, Ruiz-Irastorza G, Khamashta MA, Jacobsen S, Buyon JP, Maddison P, Dooley MA, van Vollenhoven RF, Ginzler E, Stoll T, Peschken C, Jorizzo JL, Callen JP, Lim SS, Fessler BJ, Inanc M, Kamen DL, Rahman A, Steinsson K, Franks AG, Sigler L, Hameed S, Fang H, Pham N, Brey R, Weisman MH, McGwin G, Magder LS |title=Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus |journal=Arthritis Rheum. |volume=64 |issue=8 |pages=2677–86 |year=2012 |pmid=22553077 |pmc=3409311 |doi=10.1002/art.34473 |url=}}</ref>
# 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.
* Meeting at least 4 of 17 criteria, including at least 1 of the 11 clinical criteria and one of the six [[Immunological|immunologic]] criteria 
'''OR'''
* Biopsy-proven [[nephritis]] compatible with [[SLE]] in the presence of [[Antinuclear antibodies|antinuclear antibodies (ANA)]] or [[Double stranded DNA antibody|anti-double-stranded DNA]] (dsDNA) [[antibodies]]
'''A criterion is considered positive if one or more of the observations listed in the definition for the criterion are present in the patient. A criterion should only be counted once, regardless of the number of observations in the definition that the patient presents with.'''
{| class="wikitable"
{| class="wikitable"
!
! align="center" style="background: #4479BA; color: #FFFFFF; " |Category
!Criterion
! align="center" style="background: #4479BA; color: #FFFFFF; " |Criterion
!Definition
! align="center" style="background: #4479BA; color: #FFFFFF; " |Definition
|-
|-
|Clinical criteria
| rowspan="8" |<small>Clinical</small>
|Acute cutaneous lupus
! style="background: #DCDCDC; " | Acute cutaneous lupus
|
|
* Lupus malar rash (do not count if malar discoid)
* Lupus [[malar rash]]
* Bullous lupus
** Fixed [[erythema]], flat or raised, over the [[malar]] eminences, tending to spare the [[nasolabial folds]]
* Toxic epidermal necrolysis variant of SLE
* [[Bullous]] lupus
* Maculopapular lupus rash
* [[Toxic epidermal necrolysis]] variant of SLE
* 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)
* [[Maculopapular rash|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 hypopigmentation|postinflammatory dyspigmentation]] or [[Telangiectasia|telangiectasias]]
|-
|-
! style="background: #DCDCDC; " |Chronic cutaneous lupus
|
|
|Chronic cutaneous lupus
* Classic [[Discoid lupus|discoid rash]]
|
* Classic discoid rash
** Localized (above the neck)
** Localized (above the neck)
** Generalized (above and below the neck)
** Generalized (above and below the neck)
** Hypertrophic (verrucous) lupus
** [[Hypertrophic]] (verrucous) lupus
** Lupus panniculitis (profundus)
** Lupus [[panniculitis]] (profundus)
** Mucosal lupus
** [[Mucosal]] lupus
** Lupus erythematosus tumidus
** [[Lupus erythematosus]] tumidus
** Chilblains lupus OR
** Chilblains lupus OR
** Discoid lupus/lichen planus overlap
** Discoid lupus[[Lichen planus|/lichen planus]] overlap
|-
|-
! style="background: #DCDCDC; " |Nonscarring [[alopecia]]
|
|
|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]])
|-
! style="background: #DCDCDC; " |[[Oral ulcer|Oral]] or nasal ulcers
|
|
* 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)
* [[Palate]], [[buccal]], [[tongue]], or nasal [[ulcers]] (in the absence of other causes, such as [[vasculitis]], [[Behçet's disease]], [[infection]], [[inflammatory bowel disease]], [[reactive arthritis]], and acidic foods)
|-
|-
! style="background: #DCDCDC; " |[[Arthralgia|Joint disease]]
|
|
|Oral or nasal ulcers
* [[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
* 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)
|-
|-
! style="background: #DCDCDC; " |[[Serositis]]
|
|
|Joint disease
* Typical [[pleurisy]] for more than one day, [[Pleural effusion|pleural effusions]], or [[pleural]] rub
|
* 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]]
* Synovitis involving two or more joints, characterized by swelling or effusion '''OR'''
* Tenderness in two or more joints and at least 30 minutes of morning stiffness
|-
|-
! style="background: #DCDCDC; " |[[Renal]]
|
|
|Serositis
* Urine protein-to-[[creatinine]] ratio (or 24-hour urine protein) representing 500 mg protein/24 hours
|
* [[Red blood cell]] casts
* 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
|-
|-
! style="background: #DCDCDC; " |[[Neurological|Neurologic]]
|
|
|Renal
* [[Seizure|Seizures]]
|
* [[Psychosis]]
* Urine protein-to-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hours, '''OR'''
* [[Mononeuritis multiplex]] (in the absence of other known causes, such as primary [[vasculitis]])
* Red blood cell casts
* [[Myelitis]]
* [[Peripheral neuropathy|Peripheral]] or [[Neuropathy|cranial neuropathy]] (in the absence of other known causes, such as primary [[vasculitis]], [[infection]], and [[diabetes mellitus]]) 
* [[Confusion|Acute confusional state]] (in the absence of other causes, including toxic/metabolic, [[uremia]], drugs)
|-
|-
| rowspan="3" |<small>[[Hematologic]]</small>
! style="background: #DCDCDC; " |[[Hemolytic anemia]]
|
|
|Neurologic
* [[Hemoglobin]] less than 12 g/dL in females and 13 g/dL in males
|
* 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 criteria
! style="background: #DCDCDC; " |[[Leukopenia]] or [[lymphopenia]]
|Hemolytic anemia
|
|
* [[Leukopenia]] (<4000/mm3 at least once) (in the absence of other known causes, such as [[Felty's syndrome]], [[drugs]], and [[portal hypertension]])
* [[Lymphopenia]] (<1000/mm3 at least once) (in the absence of other known causes, such as [[glucocorticoids]], [[drugs]], and [[infection]])
|-
|-
! style="background: #DCDCDC; " |[[Thrombocytopenia]]
|
|
|Leukopenia or lymphopenia
* [[Thrombocytopenia]] (<100,000/mm3) at least once in the absence of other known causes, such as [[drugs]], [[portal hypertension]], and [[thrombotic thrombocytopenic purpura]]
|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)
|-
|-
| rowspan="6" |<small>[[Immunological|Immunologic]]</small>
! style="background: #DCDCDC; " |[[ANA]]
|
|
|Thrombocytopenia
* [[ANA]] level above laboratory reference range
|Thrombocytopenia (<100,000/mm3) at least once in the absence of other known causes, such as drugs, portal hypertension, and thrombotic thrombocytopenic purpura
|-
|-
! style="background: #DCDCDC; " |[[Anti-dsDNA antibody|Anti-dsDNA]]
|
|
|ANA
* [[Anti-dsDNA antibody]] level above laboratory reference range (or >twofold the reference range if tested by [[Enzyme linked immunosorbent assay (ELISA)|ELISA]])
|ANA level above laboratory reference range
|-
|-
! style="background: #DCDCDC; " |Anit-SM
|
|
|Anti-dsDNA
* Presence of [[antibody]] to [[Smith antigen|Sm nuclear antigen]]
|Anti-dsDNA antibody level above laboratory reference range (or >twofold the reference range if tested by ELISA)
|-
|-
! style="background: #DCDCDC; " |[[Antiphospholipid antibody|Antiphospholipid]]
|
|
|Anit-SM
* [[Antiphospholipid antibodies|Antiphospholipid antibody]] positivity as determined by any of the following:
|Presence of antibody to Sm nuclear antigen
** Positive test result for [[lupus anticoagulant]]
|-
** False-positive test result for [[rapid plasma reagin]]
|Immunologic criteria
** Medium- or high-titer [[anticardiolipin antibody]] level ([[IgA]], [[IgG]], or [[IgM]])
|Antiphospholipid
** Positive test result for anti-beta 2-glycoprotein I ([[IgA]], [[IgG]], or [[IgM]])
|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)
|-
|-
! style="background: #DCDCDC; " |Low [[complement]]
|
|
|Low complement
* Low [[C3 glomerular disease|C3]]
|Low C3; low C4; '''OR''' low CH50
* Low [[C4 disease|C4]]
* Low CH50
|-
|-
! style="background: #DCDCDC; " |[[Coombs' Test|Direct Coombs' test]]
|
|
|Direct Coombs' test
* [[Coombs' Test|Direct Coombs' test]] in the absence of [[hemolytic anemia]]
|Direct Coombs' test in the absence of hemolytic anemia
|}
|}


==References==
==References==
{{reflist|2}}
{{reflist|2}}

Latest revision as of 13:53, 17 October 2017

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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, the 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 (for each criterion, any bullet is considered as 1 clinical criteria), or a 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.[1][2]

Based on SLICC criteria, diagnosis of SLE is defined as:[3]

  • Meeting at least 4 of 17 criteria, including at least 1 of the 11 clinical criteria and one of the six immunologic criteria 

OR

A criterion is considered positive if one or more of the observations listed in the definition for the criterion are present in the patient. A criterion should only be counted once, regardless of the number of observations in the definition that the patient presents with.

Category Criterion Definition
Clinical Acute cutaneous lupus
Chronic cutaneous lupus
Nonscarring alopecia
Oral or nasal ulcers
Joint disease
Serositis
Renal
Neurologic
Hematologic Hemolytic anemia
  • Hemoglobin less than 12 g/dL in females and 13 g/dL in males
Leukopenia or lymphopenia
Thrombocytopenia
Immunologic ANA
  • ANA level above laboratory reference range
Anti-dsDNA
Anit-SM
Antiphospholipid
Low complement
  • Low C3
  • Low C4
  • Low CH50
Direct Coombs' test

References

  1. ↑ Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, Schaller JG, Talal N, Winchester RJ (1982). "The 1982 revised criteria for the classification of systemic lupus erythematosus". Arthritis Rheum. 25 (11): 1271–7. PMID 7138600.
  2. ↑ Hochberg MC (1997). "Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus". Arthritis Rheum. 40 (9): 1725. doi:10.1002/1529-0131(199709)40:9&lt;1725::AID-ART29&gt;3.0.CO;2-Y. PMID 9324032.
  3. ↑ Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, Bruce IN, Isenberg D, Wallace DJ, Nived O, Sturfelt G, Ramsey-Goldman R, Bae SC, Hanly JG, Sánchez-Guerrero J, Clarke A, Aranow C, Manzi S, Urowitz M, Gladman D, Kalunian K, Costner M, Werth VP, Zoma A, Bernatsky S, Ruiz-Irastorza G, Khamashta MA, Jacobsen S, Buyon JP, Maddison P, Dooley MA, van Vollenhoven RF, Ginzler E, Stoll T, Peschken C, Jorizzo JL, Callen JP, Lim SS, Fessler BJ, Inanc M, Kamen DL, Rahman A, Steinsson K, Franks AG, Sigler L, Hameed S, Fang H, Pham N, Brey R, Weisman MH, McGwin G, Magder LS (2012). "Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus". Arthritis Rheum. 64 (8): 2677–86. doi:10.1002/art.34473. PMC 3409311. PMID 22553077.
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