Behçet's disease diagnostic study of choice: Difference between revisions
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== Overview == | == Overview == | ||
== Diagnostic Study of Choice == | == Diagnostic Study of Choice == | ||
=== Study of choice === | === Study of choice === | ||
* The pathergy test should be performed when: | * The pathergy test should be performed when: | ||
** The patient presented with symptoms of vision problems, mouth sores, and genital ulcers | ** The patient presented with symptoms of vision problems, mouth sores, and genital ulcers | ||
** A positive [test] is detected in the patient. | ** A positive [test] is detected in the patient. | ||
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=== Diagnostic Criteria === | === Diagnostic Criteria === | ||
* Here you should describe the details of the diagnostic criteria. | |||
*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]. | |||
Because oral aphthous ulcers are so common in the general population and Behçet syndrome is so rare, Behçet syndrome is best diagnosed in the context of recurrent aphthous ulcerations along with characteristic systemic manifestations. Systemic manifestations which should raise suspicion for Behçet syndrome include ocular disease, especially hypopyon, panuveitis, or retinal vasculitis; neurologic disease including characteristic central nervous system parenchymal findings; vascular disease, particularly pulmonary artery aneurysms, Budd-Chiari syndrome, and cerebral venous thrombosis; and patients with pathergy manifestations. Oral ulcerations also tend to be more frequent and severe in patients with Behçet syndrome. Genital ulcerations are more specific and less sensitive for the detection of Behçet syndrome. Behçet syndrome should also be considered more strongly in patients with the aforementioned symptoms who live along the Silk Road from eastern Asia to the Mediterranean Sea [104,105]. | |||
There are no pathognomonic laboratory tests in Behçet syndrome; as a result, the diagnosis is made on the basis of the clinical findings. In the absence of other systemic diseases, we diagnose Behçet syndrome in patients with recurrent oral aphthae (at least three times in one year) plus two of the following clinical features: | There are no pathognomonic laboratory tests in Behçet syndrome; as a result, the diagnosis is made on the basis of the clinical findings. In the absence of other systemic diseases, we diagnose Behçet syndrome in patients with recurrent oral aphthae (at least three times in one year) plus two of the following clinical features: | ||
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There are patients who do not meet these criteria in whom the diagnosis of Behçet syndrome is still made, and establishing the diagnosis in such patients is much more difficult. Thus, it is often appropriate to refer a patient in whom the diagnosis of Behçet syndrome is suspected to a rheumatologist with experience in this disease. | There are patients who do not meet these criteria in whom the diagnosis of Behçet syndrome is still made, and establishing the diagnosis in such patients is much more difficult. Thus, it is often appropriate to refer a patient in whom the diagnosis of Behçet syndrome is suspected to a rheumatologist with experience in this disease. | ||
==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). Behcet'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 Criteria== | ==Diagnostic Criteria== | ||
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===International Study Group diagnostic guidelines=== | ===International Study Group diagnostic guidelines=== | ||
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 [[testicle]]s or [[epididymitis]] in men), | *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), | *[[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), | *[[eye]] inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous), | ||
*pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick). | *pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick). | ||
===Practical clinical diagnosis=== | ===Practical clinical diagnosis=== | ||
Revision as of 13:48, 23 April 2018
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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
Diagnostic Study of Choice
Study of choice
- The pathergy test should be performed when:
- The patient presented with symptoms of vision problems, mouth sores, and genital ulcers
- A positive [test] is detected in the patient.
- Behcet disease is mainly diagnosed based on clinical presentation.
Diagnostic Criteria
- Here you should describe the details of the diagnostic criteria.
- 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 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].
Because oral aphthous ulcers are so common in the general population and Behçet syndrome is so rare, Behçet syndrome is best diagnosed in the context of recurrent aphthous ulcerations along with characteristic systemic manifestations. Systemic manifestations which should raise suspicion for Behçet syndrome include ocular disease, especially hypopyon, panuveitis, or retinal vasculitis; neurologic disease including characteristic central nervous system parenchymal findings; vascular disease, particularly pulmonary artery aneurysms, Budd-Chiari syndrome, and cerebral venous thrombosis; and patients with pathergy manifestations. Oral ulcerations also tend to be more frequent and severe in patients with Behçet syndrome. Genital ulcerations are more specific and less sensitive for the detection of Behçet syndrome. Behçet syndrome should also be considered more strongly in patients with the aforementioned symptoms who live along the Silk Road from eastern Asia to the Mediterranean Sea [104,105].
There are no pathognomonic laboratory tests in Behçet syndrome; as a result, the diagnosis is made on the basis of the clinical findings. In the absence of other systemic diseases, we diagnose Behçet syndrome in patients with recurrent oral aphthae (at least three times in one year) plus two of the following clinical features:
●Recurrent genital aphthae (aphthous ulceration or scarring).
●Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit lamp examination, or retinal vasculitis observed by an ophthalmologist).
●Skin lesions (including erythema nodosum, pseudo-vasculitis, papulopustular lesions, or acneiform nodules consistent with Behçet syndrome).
●A positive pathergy test. Pathergy is defined by a papule 2 mm or more in size developing 24 to 48 hours after oblique insertion of a 20-gauge needle 5 mm into the skin, generally performed on the forearm.
This approach is consistent with the International Study Group (ISG) diagnostic criteria published in 1990 (table 2) [25]. These remain the most widely used and well-accepted criteria among experts in Behçet syndrome. (See 'Classification criteria' below.)
Pathergy is less common in Northern European and North American patients. Thus, it has been suggested that other features might be substituted for pathergy in these populations, including aseptic meningoencephalitis, cerebral vasculitis, recurrent phlebitis, arteritis, synovitis, or focal bowel ulceration [106].
There are patients who do not meet these criteria in whom the diagnosis of Behçet syndrome is still made, and establishing the diagnosis in such patients is much more difficult. Thus, it is often appropriate to refer a patient in whom the diagnosis of Behçet syndrome is suspected to a rheumatologist with experience in this disease.
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). Behcet'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 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 Study Group diagnostic guidelines
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 dia. 24-48 hrs or more after needle-prick).
Practical clinical diagnosis
Must have
- mouth ulcers,
along with 1 of the 4 hallmark symptoms above and with 2 of the symptoms below:
- arthritis/arthralgia,
- nervous system symptoms,
- stomach and/or bowel inflammation,
- deep vein thrombosis,
- superficial thrombophlebitis,
- cardio-vascular problems of inflammatory origin,
- inflammatory problems in chest and lungs,
- problems with hearing and/or balance,
- extreme exhaustion,
- changes of personality, psychoses,
- any other members of the family with a diagnosis of Behcet disease.
'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.