Behçet's disease overview: Difference between revisions
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==Historical Perspective== | ==Historical Perspective== | ||
Behcet disease was first discovered by Hippocrates in the 5th century. In 1937, Hulusi Behçet, Turkish dermatologist described behcet syndrome that genital ulcerations, uveitis and aphthous ulcers are it's major presentationsThe name (''Morbus Behçet'') was formally adopted at the International Congress of Dermatology in Geneva in September 1947. | Behcet disease was first discovered by Hippocrates in the 5th century. In 1937, Hulusi Behçet, Turkish dermatologist described behcet syndrome that genital ulcerations, uveitis and aphthous ulcers are it's major presentationsThe name (''Morbus Behçet'') was formally adopted at the International Congress of Dermatology in Geneva in September 1947. | ||
==Classification== | |||
Neurologic disease of Behcet disease is classified into parenchymal or non-parenchymal. Parenchymal disease is due to lesions in the corticospinal tract, brainstem, periventricular white matter, spinal cord, and basal ganglia. Focal parenchymal lesions and complications of vascular thrombosis are the most common abnormalities. Progressive personality change, psychiatric disorders, and dementia may develop. Parenchymal disease may be divided into acute and chronic progressive neuro-Behçet syndrome. Central nervous system lesions are detectable with MRI. In the chronic phase, lesions may be smaller or resolve, atrophy may be present, nonspecific white matter lesions may be present, and lesions usually do not enhance. Cerebrospinal fluid (CSF) may show increased protein and increased cells, and neutrophils may predominate. Non-parenchymal disease of Behcet disease include cerebral venous thrombosis, intracranial hypertension syndrome (pseudotumor cerebri), acute meningeal syndrome, and uncommonly stroke due to arterial thrombosis, dissection, or aneurysm. On average, a period of approximately five to six years elapsed between the onset of the earliest non-neurologic symptoms of Behçet syndrome and the appearance of neurologic symptoms or findings. | |||
==Pathophysiology== | ==Pathophysiology== | ||
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==Causes== | ==Causes== | ||
The reason for the overactive immune system in Behçet's disease is not known. | The reason for the overactive immune system in Behçet's disease is not known. | ||
==Differentiating Behçet's disease from other Diseases== | ==Differentiating Behçet's disease from other Diseases== | ||
Behçet's disease needs to be differentiated from other diseases which present with similar symptoms like [[erythema nodosum]], [[inflammatory bowel disease]] among others. | Behçet's disease needs to be differentiated from other diseases which present with similar symptoms like [[erythema nodosum]], [[inflammatory bowel disease]] among others. | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
The prevalence of behcet disease is approximately 0.12 to 7.5 per 100,000 individuals in the United States. Behcet disease commonly affects young adults 20 to 40 years of age. Males are more commonly affected by Behcet disease than females. Behcet disease usually affects individuals of the Turkish, Asian, and Middle Eastern populations. Middle Eastern and Asian individuals are more likely to develop Behcet disease due to the increased incidence of skin pathergy and HLA-B51 antigen. The male to female ratio ranges from approximately 11 to 1 to 2 to 1. The majority of behcet disease cases are reported along the ancient silk road (from eastern Asia to the Mediterranean). | |||
==Risk Factors== | ==Risk Factors== | ||
Common risk factors in the development of Behcet disease may be occupational, environmental, genetic, and viral. Behçet's disease can affect people in any age, but the most common age is 20~30 years old. Behçet's disease is more common in Middle East and Japan then in other race. It is rare in America. Researches demonstrate that the presence of the gene HLA–B51 is a risk factor for Behçet's disease. | |||
==Natural History, Complications and Prognosis== | ==Natural History, Complications and Prognosis== | ||
Behçet's disease can have flares or with effective treatment, enter into remission. Serious symptoms may appear months or years after the first signs. Male gender is associated with a poorer prognosis. | Behçet's disease can have flares or with effective treatment, enter into remission. Serious symptoms may appear months or years after the first signs. Male gender is associated with a poorer prognosis. | ||
==Diagnosis== | ==Diagnosis== | ||
===Diagnostic Criteria=== | ===Diagnostic Criteria=== | ||
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. | |||
===History and Symptoms=== | ===History and Symptoms=== | ||
The hallmark of Behcet disease is recurrent aphthous ulcerations, genital ulcers, and uveitis. A positive history of joint pains and skin lesions are suggestive of Behcet disease. The most common symptoms of Behcet disease include [[mouth sores]], [[uveitis]], [[joint pain]], and genital sores. Less common symptoms of Behcet disease include diarrhea, abdominal pain, headache, skin lesions-red nodules, acne, joint pains, and poor balance. | |||
===Laboratory Findings=== | ===Laboratory Findings=== | ||
The laboratory findings to diagnose Behçet's disease are a [[pathergy test]], [[skin biopsy]] and a [[lumbar puncture]]. | The laboratory findings to diagnose Behçet's disease are a [[pathergy test]], [[skin biopsy]] and a [[lumbar puncture]]. | ||
===Other Diagnostic Studies=== | ===Other Diagnostic Studies=== | ||
A [[colonoscopy]] may be needed to exclude intestinal cancer if patient presents with gastrointestinal complaints. | A [[colonoscopy]] may be needed to exclude intestinal cancer if patient presents with gastrointestinal complaints. | ||
==Treatment== | ==Treatment== | ||
Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. Anti-TNF therapy such as [[infliximab]] has shown promise in treating the uveitis associated with the disease. Another Anti-TNF agent, [[etanercept]], may be useful in patients with mainly skin and mucosal symptoms. Interferon alfa-2a, [[azathioprine]], [[colchicine]], [[thalidomide]], [[dapsone]] and [[rebamipide]] are among other agents that are used as alternative. Surgery in Behçet's disease is performed to treat severe complications like [[gastrointestinal perforation]] or ocular inflammatory diseases. | Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. Anti-TNF therapy such as [[infliximab]] has shown promise in treating the uveitis associated with the disease. Another Anti-TNF agent, [[etanercept]], may be useful in patients with mainly skin and mucosal symptoms. Interferon alfa-2a, [[azathioprine]], [[colchicine]], [[thalidomide]], [[dapsone]] and [[rebamipide]] are among other agents that are used as alternative. Surgery in Behçet's disease is performed to treat severe complications like [[gastrointestinal perforation]] or ocular inflammatory diseases. | ||
Revision as of 22:43, 1 May 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
Behçet's disease is a chronic inflammatory condition caused by disturbances in the immune system. The immune system normally protects the body against infections through a controlled inflammatory reaction against the pathogen. In Behçet's disease, the immune response becomes overactive and produces unpredictable outbreaks of exaggerated inflammation. This extra inflammation affects blood vessels, in particular the smaller vessels. As a result, symptoms appear wherever the exaggerated inflammation response is produced. The inflammatory response can occur anywhere on the body where there is a blood supply.
Historical Perspective
Behcet disease was first discovered by Hippocrates in the 5th century. In 1937, Hulusi Behçet, Turkish dermatologist described behcet syndrome that genital ulcerations, uveitis and aphthous ulcers are it's major presentationsThe name (Morbus Behçet) was formally adopted at the International Congress of Dermatology in Geneva in September 1947.
Classification
Neurologic disease of Behcet disease is classified into parenchymal or non-parenchymal. Parenchymal disease is due to lesions in the corticospinal tract, brainstem, periventricular white matter, spinal cord, and basal ganglia. Focal parenchymal lesions and complications of vascular thrombosis are the most common abnormalities. Progressive personality change, psychiatric disorders, and dementia may develop. Parenchymal disease may be divided into acute and chronic progressive neuro-Behçet syndrome. Central nervous system lesions are detectable with MRI. In the chronic phase, lesions may be smaller or resolve, atrophy may be present, nonspecific white matter lesions may be present, and lesions usually do not enhance. Cerebrospinal fluid (CSF) may show increased protein and increased cells, and neutrophils may predominate. Non-parenchymal disease of Behcet disease include cerebral venous thrombosis, intracranial hypertension syndrome (pseudotumor cerebri), acute meningeal syndrome, and uncommonly stroke due to arterial thrombosis, dissection, or aneurysm. On average, a period of approximately five to six years elapsed between the onset of the earliest non-neurologic symptoms of Behçet syndrome and the appearance of neurologic symptoms or findings.
Pathophysiology
The underlying pathophysiological mechanism of Behçet's disease is over-active immune system which produces recurrent outbreaks of inflammation in small blood vessels. Common symptoms include mouth ulcers, genital ulcers, eye inflammation, and arthritis in older patients.
Causes
The reason for the overactive immune system in Behçet's disease is not known.
Differentiating Behçet's disease from other Diseases
Behçet's disease needs to be differentiated from other diseases which present with similar symptoms like erythema nodosum, inflammatory bowel disease among others.
Epidemiology and Demographics
The prevalence of behcet disease is approximately 0.12 to 7.5 per 100,000 individuals in the United States. Behcet disease commonly affects young adults 20 to 40 years of age. Males are more commonly affected by Behcet disease than females. Behcet disease usually affects individuals of the Turkish, Asian, and Middle Eastern populations. Middle Eastern and Asian individuals are more likely to develop Behcet disease due to the increased incidence of skin pathergy and HLA-B51 antigen. The male to female ratio ranges from approximately 11 to 1 to 2 to 1. The majority of behcet disease cases are reported along the ancient silk road (from eastern Asia to the Mediterranean).
Risk Factors
Common risk factors in the development of Behcet disease may be occupational, environmental, genetic, and viral. Behçet's disease can affect people in any age, but the most common age is 20~30 years old. Behçet's disease is more common in Middle East and Japan then in other race. It is rare in America. Researches demonstrate that the presence of the gene HLA–B51 is a risk factor for Behçet's disease.
Natural History, Complications and Prognosis
Behçet's disease can have flares or with effective treatment, enter into remission. Serious symptoms may appear months or years after the first signs. Male gender is associated with a poorer prognosis.
Diagnosis
Diagnostic Criteria
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.
History and Symptoms
The hallmark of Behcet disease is recurrent aphthous ulcerations, genital ulcers, and uveitis. A positive history of joint pains and skin lesions are suggestive of Behcet disease. The most common symptoms of Behcet disease include mouth sores, uveitis, joint pain, and genital sores. Less common symptoms of Behcet disease include diarrhea, abdominal pain, headache, skin lesions-red nodules, acne, joint pains, and poor balance.
Laboratory Findings
The laboratory findings to diagnose Behçet's disease are a pathergy test, skin biopsy and a lumbar puncture.
Other Diagnostic Studies
A colonoscopy may be needed to exclude intestinal cancer if patient presents with gastrointestinal complaints.
Treatment
Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. Anti-TNF therapy such as infliximab has shown promise in treating the uveitis associated with the disease. Another Anti-TNF agent, etanercept, may be useful in patients with mainly skin and mucosal symptoms. Interferon alfa-2a, azathioprine, colchicine, thalidomide, dapsone and rebamipide are among other agents that are used as alternative. Surgery in Behçet's disease is performed to treat severe complications like gastrointestinal perforation or ocular inflammatory diseases.