Systemic lupus erythematosus diagnostic criteria: Difference between revisions
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{{CMG}}; {{AE}}{{MIR}} | {{CMG}}; {{AE}}{{MIR}} | ||
==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 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<1725::AID-ART29>3.0.CO;2-Y |url=}}</ref> | 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<1725::AID-ART29>3.0.CO;2-Y |url=}}</ref> | ||
Based on SLICC criteria, | 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> | ||
* | * 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''' | '''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 | ||
|- | |- | ||
| rowspan="8" |Clinical | | rowspan="8" |<small>Clinical</small> | ||
|Acute cutaneous lupus | ! style="background: #DCDCDC; " | Acute cutaneous lupus | ||
| | | | ||
* Lupus malar rash | * Lupus [[malar rash]] | ||
** Fixed erythema, | ** Fixed [[erythema]], flat or raised, over the [[malar]] eminences, tending to spare the [[nasolabial folds]] | ||
* Bullous lupus | * [[Bullous]] lupus | ||
* Toxic epidermal necrolysis variant of SLE | * [[Toxic epidermal necrolysis]] variant of SLE | ||
* Maculopapular lupus rash | * [[Maculopapular rash|Maculopapular]] lupus [[rash]] | ||
* Photosensitive lupus rash (in the absence of dermatomyositis); | * [[Photosensitive]] lupus [[rash]] (in the absence of [[dermatomyositis]]); | ||
** Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation | ** Skin [[rash]] as a result of unusual reaction to sunlight, by patient history or physician observation | ||
* Subacute cutaneous lupus | * Subacute cutaneous lupus | ||
** Nonindurated psoriaform and/or annular polycyclic lesions that resolve without scarring, although occasionally with postinflammatory dyspigmentation or telangiectasias | ** Nonindurated psoriaform and/or annular polycyclic [[lesions]] that resolve without [[scarring]], although occasionally with [[Postinflammatory hypopigmentation|postinflammatory dyspigmentation]] or [[Telangiectasia|telangiectasias]] | ||
|- | |- | ||
|Chronic cutaneous lupus | ! style="background: #DCDCDC; " |Chronic cutaneous lupus | ||
| | | | ||
* Classic discoid rash | * Classic [[Discoid lupus|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 | ||
|- | |- | ||
|Nonscarring alopecia | ! style="background: #DCDCDC; " |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) | * 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 | ! style="background: #DCDCDC; " |[[Oral ulcer|Oral]] or nasal ulcers | ||
| | | | ||
* Palate, buccal, tongue, or nasal ulcers (in the absence of other causes, such as vasculitis, Behçet's disease, infection | * [[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) | ||
|- | |- | ||
|Joint disease | ! style="background: #DCDCDC; " |[[Arthralgia|Joint disease]] | ||
| | | | ||
* Synovitis involving two or more joints, characterized by swelling or | * [[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 | * [[Tenderness]] in two or more [[joints]] and at least 30 minutes of morning stiffness | ||
|- | |- | ||
|Serositis | ! style="background: #DCDCDC; " |[[Serositis]] | ||
| | | | ||
* Typical pleurisy for more than one day, pleural effusions, or pleural rub | * 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 | * 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 | ! style="background: #DCDCDC; " |[[Renal]] | ||
| | | | ||
* Urine protein-to-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hours | * Urine protein-to-[[creatinine]] ratio (or 24-hour urine protein) representing 500 mg protein/24 hours | ||
* Red blood cell casts | * [[Red blood cell]] casts | ||
|- | |- | ||
|Neurologic | ! style="background: #DCDCDC; " |[[Neurological|Neurologic]] | ||
| | | | ||
* Seizures | * [[Seizure|Seizures]] | ||
* Psychosis | * [[Psychosis]] | ||
* Mononeuritis multiplex (in the absence of other known causes, such as primary vasculitis) | * [[Mononeuritis multiplex]] (in the absence of other known causes, such as primary [[vasculitis]]) | ||
* Myelitis | * [[Myelitis]] | ||
* Peripheral or cranial neuropathy (in the absence of other known causes, such as primary vasculitis, infection, and diabetes mellitus) | * [[Peripheral neuropathy|Peripheral]] or [[Neuropathy|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) | * [[Confusion|Acute confusional state]] (in the absence of other causes, including toxic/metabolic, [[uremia]], drugs) | ||
|- | |- | ||
| rowspan=" | | rowspan="3" |<small>[[Hematologic]]</small> | ||
|Hemolytic anemia | ! style="background: #DCDCDC; " |[[Hemolytic anemia]] | ||
| | | | ||
* [[Hemoglobin]] less than 12 g/dL in females and 13 g/dL in males | |||
|- | |- | ||
|Leukopenia or lymphopenia | ! style="background: #DCDCDC; " |[[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) | * [[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) | * [[Lymphopenia]] (<1000/mm3 at least once) (in the absence of other known causes, such as [[glucocorticoids]], [[drugs]], and [[infection]]) | ||
|- | |- | ||
|Thrombocytopenia | ! style="background: #DCDCDC; " |[[Thrombocytopenia]] | ||
| | | | ||
* Thrombocytopenia (<100,000/mm3) at least once in the absence of other known causes, such as drugs, portal hypertension, and thrombotic thrombocytopenic purpura | * [[Thrombocytopenia]] (<100,000/mm3) at least once in the absence of other known causes, such as [[drugs]], [[portal hypertension]], and [[thrombotic thrombocytopenic purpura]] | ||
|- | |- | ||
|ANA | | rowspan="6" |<small>[[Immunological|Immunologic]]</small> | ||
! style="background: #DCDCDC; " |[[ANA]] | |||
| | | | ||
* ANA level above laboratory reference range | * [[ANA]] level above laboratory reference range | ||
|- | |- | ||
|Anti-dsDNA | ! style="background: #DCDCDC; " |[[Anti-dsDNA antibody|Anti-dsDNA]] | ||
| | | | ||
* Anti-dsDNA antibody level above laboratory reference range (or >twofold the reference range if tested by ELISA) | * [[Anti-dsDNA antibody]] level above laboratory reference range (or >twofold the reference range if tested by [[Enzyme linked immunosorbent assay (ELISA)|ELISA]]) | ||
|- | |- | ||
|Anit-SM | ! style="background: #DCDCDC; " |Anit-SM | ||
| | | | ||
* Presence of antibody to Sm nuclear antigen | * Presence of [[antibody]] to [[Smith antigen|Sm nuclear antigen]] | ||
|- | |- | ||
! style="background: #DCDCDC; " |[[Antiphospholipid antibody|Antiphospholipid]] | |||
|Antiphospholipid | |||
| | | | ||
* Antiphospholipid antibody positivity as determined by any of the following: | * [[Antiphospholipid antibodies|Antiphospholipid antibody]] positivity as determined by any of the following: | ||
** Positive test result for lupus anticoagulant | ** Positive test result for [[lupus anticoagulant]] | ||
** False-positive test result for rapid plasma reagin | ** False-positive test result for [[rapid plasma reagin]] | ||
** Medium- or high-titer anticardiolipin antibody level (IgA, IgG, or IgM) | ** Medium- or high-titer [[anticardiolipin antibody]] level ([[IgA]], [[IgG]], or [[IgM]]) | ||
** Positive test result for anti-beta 2-glycoprotein I (IgA, IgG, or IgM) | ** Positive test result for anti-beta 2-glycoprotein I ([[IgA]], [[IgG]], or [[IgM]]) | ||
|- | |- | ||
|Low complement | ! style="background: #DCDCDC; " |Low [[complement]] | ||
| | | | ||
* Low C3 | * Low [[C3 glomerular disease|C3]] | ||
* Low C4 | * Low [[C4 disease|C4]] | ||
* Low CH50 | * Low CH50 | ||
|- | |- | ||
|Direct Coombs' test | ! style="background: #DCDCDC; " |[[Coombs' Test|Direct Coombs' test]] | ||
| | | | ||
* Direct Coombs' test in the absence of hemolytic anemia | * [[Coombs' Test|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
- Biopsy-proven nephritis compatible with SLE in the presence of antinuclear antibodies (ANA) or 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.
| Category | Criterion | Definition |
|---|---|---|
| Clinical | Acute cutaneous lupus |
|
| Chronic cutaneous lupus |
| |
| Nonscarring alopecia |
| |
| Oral or nasal ulcers |
| |
| Joint disease |
| |
| Serositis |
| |
| Renal |
| |
| Neurologic |
| |
| Hematologic | Hemolytic anemia |
|
| Leukopenia or lymphopenia |
| |
| Thrombocytopenia |
| |
| Immunologic | ANA |
|
| Anti-dsDNA |
| |
| Anit-SM |
| |
| Antiphospholipid |
| |
| Low complement | ||
| Direct Coombs' test |
|
References
- ↑ 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.
- ↑ 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<1725::AID-ART29>3.0.CO;2-Y. PMID 9324032.
- ↑ 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.