Lupus nephritis diagnostic study of choice: Difference between revisions

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{{Lupus nephritis}}
{{Lupus nephritis}}
{{CMG}}; {{AE}}
{{CMG}}; {{AE}}{{OK}}
== Overview ==
==Overview==
* The page name should be '''"[Disease name] diagnostic study of choice"''', with only the first letter of the title capitalized. Note that the page is called "Diagnostic study of choice."
In SLE, nephritis we suspect renal involvment by an abnormal urinalysis and/or increased serum creatinine. Histopathologic findings on renal biopsy confirm the diagnosis.  
* '''Goal:'''
**To describe the most efficient/sensitive/specific test that is utilized for diagnosis of [disease name].
**To describe the gold standard test for the diagnosis of [disease name].
**To describe the diagnostic criteria, which may be based on clinical findings, physical exam signs, pathological findings, lab findings, findings on imaging, or even findings that exclude other diseases.
* As with all microchapter pages linking to the main page, at the top of the edit box put <nowiki>{{CMG}}</nowiki>, your name template, and the microchapter navigation template you created at the beginning.
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* Remember to follow the same format and capitalization of letters as outlined in the template below.
* You should include the name of the disease in the first sentence of every subsection.


== Diagnostic Study of Choice ==
==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.<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>


===== Template statements =====
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'''
* 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"
! align="center" style="background: #4479BA; color: #FFFFFF; " |Category
! align="center" style="background: #4479BA; color: #FFFFFF; " |Criterion
! align="center" style="background: #4479BA; color: #FFFFFF; " |Definition
|-
| rowspan="8" |<small>Clinical</small>
! style="background: #DCDCDC; " | 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 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  


=== Gold standard/Study of choice: ===
* Subacute cutaneous lupus
* [Name of the investigation] is the gold standard test for the diagnosis of [disease name].
** Nonindurated psoriaform and/or annular polycyclic [[lesions]] that resolve without [[scarring]], although occasionally with [[Postinflammatory hypopigmentation|postinflammatory dyspigmentation]] or [[Telangiectasia|telangiectasias]]
* The following result of [gold standard test] is confirmatory of [disease name]:
|-
** Result 1
! style="background: #DCDCDC; " |Chronic cutaneous lupus
** Result 2
|
* The [name of the investigation] should be performed when:
* Classic [[Discoid lupus|discoid rash]]
** The patient presented with symptoms/signs 1. 2, 3.
** Localized (above the neck)
** A positive [test] is detected in the patient.
** Generalized (above and below the neck)
* [Name of the investigation] is the gold standard test for the diagnosis of [disease name].
** [[Hypertrophic]] (verrucous) lupus
* The diagnostic study of choice for [disease name] is [name of the investigation].
** Lupus [[panniculitis]] (profundus)
* There is no single diagnostic study of choice for the diagnosis of [disease name].
** [[Mucosal]] lupus
* There is no single diagnostic study of choice for the diagnosis of [disease name], but [disease name] can be diagnosed based on [name of the investigation 1] and [name of the investigation 2].
** [[Lupus erythematosus]] tumidus
* [Disease name] is mainly diagnosed based on clinical presentation.
** Chilblains lupus OR
* Investigations:
** Discoid lupus[[Lichen planus|/lichen planus]] overlap
** Among patients who present with clinical signs of [disease name], the [investigation name] is the most specific test for the diagnosis.
|-
** Among patients who present with clinical signs of [disease name], the [investigation name] is the most sensitive test for diagnosis.
! style="background: #DCDCDC; " |Nonscarring [[alopecia]]
** Among patients who present with clinical signs of [disease name], the [investigation name] is the most efficient test for diagnosis.
|
* 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
|
* [[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]]
|
* [[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
|-
! style="background: #DCDCDC; " |[[Serositis]]
|
* 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]]
|-
! style="background: #DCDCDC; " |[[Renal]]
|
* Urine protein-to-[[creatinine]] ratio (or 24-hour urine protein) representing 500 mg protein/24 hours
* [[Red blood cell]] casts
|-
! style="background: #DCDCDC; " |[[Neurological|Neurologic]]
|
* [[Seizure|Seizures]]
* [[Psychosis]]
* [[Mononeuritis multiplex]] (in the absence of other known causes, such as primary [[vasculitis]])
* [[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]]
|
* [[Hemoglobin]] less than 12 g/dL in females and 13 g/dL in males
|-
! 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]])


==== The comparison table for diagnostic studies of choice for [disease name] ====
* [[Lymphopenia]] (<1000/mm3 at least once) (in the absence of other known causes, such as [[glucocorticoids]], [[drugs]], and [[infection]])
{|
|-
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! style="background: #DCDCDC; " |[[Thrombocytopenia]]
! style="background: #FFFFFF; color: #FFFFFF; text-align: center;" |
|
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Sensitivity
* [[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: #4479BA; color: #FFFFFF; text-align: center;" |Specificity
|-
| rowspan="6" |<small>[[Immunological|Immunologic]]</small>
! style="background: #DCDCDC; " |[[ANA]]
|
* [[ANA]] level above laboratory reference range
|-
! style="background: #DCDCDC; " |[[Anti-dsDNA antibody|Anti-dsDNA]]
|
* [[Anti-dsDNA antibody]] level above laboratory reference range (or >twofold the reference range if tested by [[Enzyme linked immunosorbent assay (ELISA)|ELISA]])
|-
! style="background: #DCDCDC; " |Anit-SM
|
* Presence of [[antibody]] to [[Smith antigen|Sm nuclear antigen]]
|-
! style="background: #DCDCDC; " |[[Antiphospholipid antibody|Antiphospholipid]]
|
* [[Antiphospholipid antibodies|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]])
|-
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Test 1
! style="background: #DCDCDC; " |Low [[complement]]
| style="background: #DCDCDC; padding: 5px; text-align: center;" |✔
|
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
* Low [[C3 glomerular disease|C3]]
* Low [[C4 disease|C4]]
* Low CH50
|-
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Test 2
! style="background: #DCDCDC; " |[[Coombs' Test|Direct Coombs' test]]
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
|
| style="background: #DCDCDC; padding: 5px; text-align: center;" |✔
* [[Coombs' Test|Direct Coombs' test]] in the absence of [[hemolytic anemia]]
|}
|}
<small> ✔= The best test based on the feature </small>
===== Diagnostic results =====
The following result of [investigation name] is confirmatory of [disease name]:
* Result 1
* Result 2


===== Sequence of Diagnostic Studies =====
== Renal Involvement ==
The [name of investigation] should be performed when:
* The patient presented with symptoms/signs 1, 2, and 3 as the first step of diagnosis.
* A positive [test] is detected in the patient, to confirm the diagnosis.


=== Diagnostic Criteria ===
=== Urinanalysis: ===
* Here you should describe the details of the diagnostic criteria.
Hematuria and cellular cast<ref name="pmid18046678">{{cite journal |vauthors=Lee LC, Lam KK, Lee CT, Chen JB, Tsai TH, Huang SC |title="Full house" proliferative glomerulonephritis: an unreported presentation of subacute infective endocarditis |journal=J. Nephrol. |volume=20 |issue=6 |pages=745–9 |date=2007 |pmid=18046678 |doi= |url=}}</ref>
*Always mention the name of the criteria/definition you are about to list (e.g. modified Duke criteria for the diagnosis of endocarditis / 3rd universal definition of MI) and cite the primary source of where this criteria/definition is found.
*Although not necessary, it is recommended that you include the criteria in a table. Make sure you always cite the source of the content and whether the table has been adapted from another source.
*Be very clear as to the number of criteria (or threshold) that needs to be met out of the total number of criteria.
*Distinguish criteria based on their nature (e.g. clinical criteria / pathological criteria/ imaging criteria) before discussing them in details.
*To view an example (endocarditis diagnostic criteria), click [[Endocarditis diagnosis|here]]
*If relevant, add additional information that might help the reader distinguish various criteria or the evolution of criteria (e.g. original criteria vs. modified criteria).
*You may also add information about the sensitivity and specificity of the criteria, the pre-test probability, and other figures that may help the reader understand how valuable the criteria are clinically.
* [Disease name] is mainly diagnosed based on clinical presentation. There are no established criteria for the diagnosis of [disease name].
* There is no single diagnostic study of choice for [disease name], though [disease name] may be diagnosed based on [name of criteria] established by [...].


* The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
Protein excretion and protein to creatinine ratio
* The diagnosis of [disease name] is based on the [criteria name] criteria, which includes [criterion 1], [criterion 2], and [criterion 3].


* [Disease name] may be diagnosed at any time if one or more of the following criteria are met:
Increased anti-DNA titers <ref name="pmid15780090">{{cite journal |vauthors=Haas M, Kaul S, Eustace JA |title=HIV-associated immune complex glomerulonephritis with "lupus-like" features: a clinicopathologic study of 14 cases |journal=Kidney Int. |volume=67 |issue=4 |pages=1381–90 |date=April 2005 |pmid=15780090 |doi=10.1111/j.1523-1755.2005.00215.x |url=}}</ref>
** Criteria 1
** Criteria 2
** Criteria 3


IF there are clear, established diagnostic criteria:
Low complement (C3 and C4) levels<ref name="pmid11145039">{{cite journal |vauthors=Ramos-Casals M, Font J, García-Carrasco M, Cervera R, Jiménez S, Trejo O, de la Red G, Sánchez-Tapias JM, Ingelmo M |title=Hepatitis C virus infection mimicking systemic lupus erythematosus: study of hepatitis C virus infection in a series of 134 Spanish patients with systemic lupus erythematosus |journal=Arthritis Rheum. |volume=43 |issue=12 |pages=2801–6 |date=December 2000 |pmid=11145039 |doi=10.1002/1529-0131(200012)43:12<2801::AID-ANR21>3.0.CO;2-V |url=}}</ref>
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
*The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
*The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
IF there are no established diagnostic criteria: 
*There are no established criteria for the diagnosis of [disease name].


==== Renal Biospy: ====
Histopathology will guide the treatment.<ref name="pmid11682680">{{cite journal |vauthors=Smet AD, Kuypers D, Evenepoel P, Maes B, Messiaen T, Van Damme B, Vanrenterghem Y |title='Full house' positive immunohistochemical membranoproliferative glomerulonephritis in a patient with portosystemic shunt |journal=Nephrol. Dial. Transplant. |volume=16 |issue=11 |pages=2258–62 |date=November 2001 |pmid=11682680 |doi= |url=}}</ref>
* Glomerular deposits: "Full house" immunofluorescence pattern containing IgG, immunoglobulin A (IgA), immunoglobulin M (IgM), C3, and C1q.
* Mesangial, subendothelial, and subepithelial glomerular deposits.
* Tubular basement membrane, the interstitial, and blood vessels extraglomerular immune-type deposits.<ref name="pmid7814619">{{cite journal |vauthors=Rich SA |title=De novo synthesis and secretion of a 36-kD protein by cells that form lupus inclusions in response to alpha-interferon |journal=J. Clin. Invest. |volume=95 |issue=1 |pages=219–26 |date=January 1995 |pmid=7814619 |pmc=295410 |doi=10.1172/JCI117643 |url=}}</ref>
* Glomerular endothelial cells containing tubuloreticular inclusions composed of ribonucleoprotein and membrane, usually stimulated by alpha-interferon.


==References==
==References==

Latest revision as of 15:20, 20 July 2018

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

Overview

In SLE, nephritis we suspect renal involvment by an abnormal urinalysis and/or increased serum creatinine. Histopathologic findings on renal biopsy confirm the diagnosis.

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

Renal Involvement

Urinanalysis:

Hematuria and cellular cast[4]

Protein excretion and protein to creatinine ratio

Increased anti-DNA titers [5]

Low complement (C3 and C4) levels[6]

Renal Biospy:

Histopathology will guide the treatment.[7]

  • Glomerular deposits: "Full house" immunofluorescence pattern containing IgG, immunoglobulin A (IgA), immunoglobulin M (IgM), C3, and C1q.
  • Mesangial, subendothelial, and subepithelial glomerular deposits.
  • Tubular basement membrane, the interstitial, and blood vessels extraglomerular immune-type deposits.[8]
  • Glomerular endothelial cells containing tubuloreticular inclusions composed of ribonucleoprotein and membrane, usually stimulated by alpha-interferon.

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.
  4. ↑ Lee LC, Lam KK, Lee CT, Chen JB, Tsai TH, Huang SC (2007). ""Full house" proliferative glomerulonephritis: an unreported presentation of subacute infective endocarditis". J. Nephrol. 20 (6): 745–9. PMID 18046678.
  5. ↑ Haas M, Kaul S, Eustace JA (April 2005). "HIV-associated immune complex glomerulonephritis with "lupus-like" features: a clinicopathologic study of 14 cases". Kidney Int. 67 (4): 1381–90. doi:10.1111/j.1523-1755.2005.00215.x. PMID 15780090.
  6. ↑ Ramos-Casals M, Font J, García-Carrasco M, Cervera R, Jiménez S, Trejo O, de la Red G, Sánchez-Tapias JM, Ingelmo M (December 2000). "Hepatitis C virus infection mimicking systemic lupus erythematosus: study of hepatitis C virus infection in a series of 134 Spanish patients with systemic lupus erythematosus". Arthritis Rheum. 43 (12): 2801–6. doi:10.1002/1529-0131(200012)43:12<2801::AID-ANR21>3.0.CO;2-V. PMID 11145039.
  7. ↑ Smet AD, Kuypers D, Evenepoel P, Maes B, Messiaen T, Van Damme B, Vanrenterghem Y (November 2001). "'Full house' positive immunohistochemical membranoproliferative glomerulonephritis in a patient with portosystemic shunt". Nephrol. Dial. Transplant. 16 (11): 2258–62. PMID 11682680.
  8. ↑ Rich SA (January 1995). "De novo synthesis and secretion of a 36-kD protein by cells that form lupus inclusions in response to alpha-interferon". J. Clin. Invest. 95 (1): 219–26. doi:10.1172/JCI117643. PMC 295410. PMID 7814619.

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