Behçet's disease diagnostic study of choice: Difference between revisions

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__NOTOC__
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{{Behçet's disease}}
{{Behçet's disease}}
{{CMG}}; {{CZ}}
{{CMG}}; {{AE}} {{HQ}}


== Overview ==
== Overview ==
Currently, there is not a specific test to confirm the diagnosis of Behçet's disease. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks obtained by a thorough history of the patient's symptoms (outlined below). Behçet's disease is a diagnosis of exclusion, and other [[Chronic (medical)|chronic]] [[Inflammation|inflammatory]] diseases should be evaluated for. The various [[Inflammation|inflammatory]] symptoms do not necessarily occur together, and they will vary in severity.


== Diagnostic Study of Choice ==
== Diagnostic Study of Choice ==


=== Study of choice ===
=== Study of choice ===
* [Name of the investigation] is the gold standard test for the diagnosis of [disease name].
* The pathergy test should be performed when:<ref name="pmid7582698">{{cite journal| author=Ferraz MB, Walter SD, Heymann R, Atra E| title=Sensitivity and specificity of different diagnostic criteria for Behçet's disease according to the latent class approach. | journal=Br J Rheumatol | year= 1995 | volume= 34 | issue= 10 | pages= 932-5 | pmid=7582698 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7582698  }} </ref>
* The following result of [gold standard test] is confirmatory of [disease name]:
** The patient presented with symptoms of [[Visual impairment|vision problems]], mouth [[Sore|sores]], and [[Genital ulcer|genital ulcers]].
** Result 1
** A positive pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick) is detected in the patient.  
** Result 2
* Behçet disease is mainly diagnosed based on clinical presentation.
* The [name of the investigation] should be performed when:
** The patient presented with symptoms/signs 1. 2, 3.
** A positive [test] is detected in the patient.
* [Name of the investigation] is the gold standard test for the diagnosis of [disease name].
* The diagnostic study of choice for [disease name] is [name of the investigation].
* There is no single diagnostic study of choice for the diagnosis of [disease name].
* 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].
* [Disease name] is mainly diagnosed based on clinical presentation.
* Investigations:
** 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.
** Among patients who present with clinical signs of [disease name], the [investigation name] is the most efficient test for diagnosis.


==== The comparison of various diagnostic studies for [disease name] ====
==Diagnostic Criteria==
{|
There are three levels of certainty for diagnosis:  
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
*International Study Group diagnostic guidelines (very strict for research purposes)
! style="background: #4479BA; color: #FFFFFF; text-align: center;" | Test
*Practical clinical diagnosis (generally agreed pattern but not as strict)
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Sensitivity
*'Suspected' or 'Possible' diagnosis (incomplete pattern of symptoms)
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Specificity
*International Criteria for Behçet's disease (ICBD)
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Test 1
| style="background: #DCDCDC; padding: 5px; text-align: center;" |✔
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Test 2
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
| style="background: #DCDCDC; padding: 5px; text-align: center;" |✔
|}
<small> ✔= The best test based on the feature </small>


===== Diagnostic results =====
===International Study Group diagnostic guidelines (ISGD)===
The following result of [investigation name] is confirmatory of [disease name]:
The ISGD guidelines recommend the following:<ref name="pmid1970380">{{cite journal| author=| title=Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. | journal=Lancet | year= 1990 | volume= 335 | issue= 8697 | pages= 1078-80 | pmid=1970380 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1970380  }} </ref><ref name="pmid10568894">{{cite journal| author=Smith EL, Shmerling RH| title=The American College of Rheumatology criteria for the classification of systemic lupus erythematosus: strengths, weaknesses, and opportunities for improvement. | journal=Lupus | year= 1999 | volume= 8 | issue= 8 | pages= 586-95 | pmid=10568894 | doi=10.1191/096120399680411317 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10568894  }} </ref>
* Result 1
*Must have oral ([[aphthous]]) ulcers (any shape, size or number at least 3 times in any 12 months), along with 2 out of the next 4 "hallmark" symptoms:
* Result 2
**Genital ulcers (including [[anal]] ulcers and spots in the genital region and swollen [[testicle]]s or [[epididymitis]] in men)
**[[Skin]] [[lesion]]s (papulo-pustules, [[folliculitis]], [[erythema nodosum]], [[acne]] in post-adolescents not on corticosteroids)
**[[Eye]] inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous)
**Pathergy reaction (papule >2 mm diameter 24-48 hrs or more after needle-prick)


===== Sequence of Diagnostic Studies =====
===Practical clinical diagnosis===
The [name of investigation] should be performed when:
The practical clinical diagnosis for Behçet disease recommend the following:<ref name="pmid7582698">{{cite journal| author=Ferraz MB, Walter SD, Heymann R, Atra E| title=Sensitivity and specificity of different diagnostic criteria for Behçet's disease according to the latent class approach. | journal=Br J Rheumatol | year= 1995 | volume= 34 | issue= 10 | pages= 932-5 | pmid=7582698 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7582698  }} </ref>
* The patient presented with symptoms/signs 1, 2, and 3 as the first step of diagnosis.
*[[Mouth]] ulcers, along with 1 of the 4 hallmark symptoms above and with 2 of the symptoms below:
* A positive [test] is detected in the patient, to confirm the diagnosis.
**[[Arthritis]]/[[arthralgia]]
**[[Nervous system]] symptoms
**[[Stomach]] and/or [[bowel]] inflammation
**Deep [[vein]] [[thrombosis]]
**Superficial thrombophlebitis
**[[Cardio]]-[[vascular]] problems of [[Inflammation|inflammatory]] origin
**[[Inflammation|Inflammatory]] problems in [[chest]] and [[lungs]]
**Problems with [[Hearing (sense)|hearing]] and/or balance
**Extreme exhaustion
**Changes of personality, [[psychoses]]
**Any other members of the family with a diagnosis of Behçet disease


=== Diagnostic Criteria ===
==='Suspected' or 'Possible' diagnosis===
* Here you should describe the details of the diagnostic criteria.
Usually assigned when someone does not have mouth ulcers, or has [[Oral ulcer|mouth ulcers]] but does not have 1 of the 4 hallmark symptoms but has other [[Symptom|symptoms]] and [[Medical sign|signs]] of [[inflammation]], and other causes for these have been ruled out.
*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].
* 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:
** Criteria 1
** Criteria 2
** Criteria 3
 
IF there are clear, established diagnostic criteria:
*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].


===International Criteria for Behçet's disease (ICBD)===
*The International Criteria for Behçet's disease (ICBD) were developed in 2006 and require a total of at least three points for diagnosis of Behçet syndrome:<ref name="pmid21961081">{{cite journal| author=Davatchi F| title=Diagnosis/Classification Criteria for Behcet's Disease. | journal=Patholog Res Int | year= 2012 | volume= 2012 | issue=  | pages= 607921 | pmid=21961081 | doi=10.1155/2012/607921 | pmc=3180812 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21961081  }} </ref>
**[[Genital skin lesions|Genital]] aphthosis – 2 points
**[[Ocular]] lesions ([[Uveitis|anterior uveitis]], posterior [[uveitis]], or [[retinal]] [[vasculitis]]) – 2 points
**[[Mouth|Oral]] aphthosis – 1 point
**[[Skin lesion|Skin lesions]] ([[Pseudofolliculitis barbae|pseudofolliculitis]] or [[erythema nodosum]]) – 1 point
**[[Vascular]] lesions (superficial [[Thrombophlebitis|phlebitis]], [[deep vein thrombosis]], large [[Venous thromboembolism|vein thrombosis]], [[arterial thrombosis]], or [[aneurysm]]) – 1 point
**Pathergy – 1 point
*[[Sensitivity (tests)|Sensitivity]] of 87 to 96.5 percent and [[Specificity (tests)|specificity]] of 88.9 to 97.3 percent<ref name="pmid21961081">{{cite journal| author=Davatchi F| title=Diagnosis/Classification Criteria for Behcet's Disease. | journal=Patholog Res Int | year= 2012 | volume= 2012 | issue=  | pages= 607921 | pmid=21961081 | doi=10.1155/2012/607921 | pmc=3180812 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21961081  }} </ref>


==References==
==References==
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[[Category:Autoimmune diseases]]
 
[[Category:Medicine]]
[[Category:Up-To-date]]
[[Category:Dermatology]]
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[[Category:Rheumatology]]
[[Category:Rheumatology]]

Latest revision as of 20:35, 29 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

Currently, there is not a specific test to confirm the diagnosis of Behçet's disease. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks obtained by a thorough history of the patient's symptoms (outlined below). Behçet's disease is a diagnosis of exclusion, and other chronic inflammatory diseases should be evaluated for. The various inflammatory symptoms do not necessarily occur together, and they will vary in severity.

Diagnostic Study of Choice

Study of choice

  • The pathergy test should be performed when:[1]
    • The patient presented with symptoms of vision problems, mouth sores, and genital ulcers.
    • A positive pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick) is detected in the patient.
  • Behçet disease is mainly diagnosed based on clinical presentation.

Diagnostic Criteria

There are three levels of certainty for diagnosis:

  • International Study Group diagnostic guidelines (very strict for research purposes)
  • Practical clinical diagnosis (generally agreed pattern but not as strict)
  • 'Suspected' or 'Possible' diagnosis (incomplete pattern of symptoms)
  • International Criteria for Behçet's disease (ICBD)

International Study Group diagnostic guidelines (ISGD)

The ISGD guidelines recommend the following:[2][3]

  • Must have oral (aphthous) ulcers (any shape, size or number at least 3 times in any 12 months), along with 2 out of the next 4 "hallmark" symptoms:
    • Genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men)
    • Skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in post-adolescents not on corticosteroids)
    • Eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous)
    • Pathergy reaction (papule >2 mm diameter 24-48 hrs or more after needle-prick)

Practical clinical diagnosis

The practical clinical diagnosis for Behçet disease recommend the following:[1]

'Suspected' or 'Possible' diagnosis

Usually assigned when someone does not have mouth ulcers, or has mouth ulcers but does not have 1 of the 4 hallmark symptoms but has other symptoms and signs of inflammation, and other causes for these have been ruled out.

International Criteria for Behçet's disease (ICBD)

References

  1. ↑ 1.0 1.1 Ferraz MB, Walter SD, Heymann R, Atra E (1995). "Sensitivity and specificity of different diagnostic criteria for Behçet's disease according to the latent class approach". Br J Rheumatol. 34 (10): 932–5. PMID 7582698.
  2. ↑ "Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease". Lancet. 335 (8697): 1078–80. 1990. PMID 1970380.
  3. ↑ Smith EL, Shmerling RH (1999). "The American College of Rheumatology criteria for the classification of systemic lupus erythematosus: strengths, weaknesses, and opportunities for improvement". Lupus. 8 (8): 586–95. doi:10.1191/096120399680411317. PMID 10568894.
  4. ↑ 4.0 4.1 Davatchi F (2012). "Diagnosis/Classification Criteria for Behcet's Disease". Patholog Res Int. 2012: 607921. doi:10.1155/2012/607921. PMC 3180812. PMID 21961081.

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