Questions and Answers
What is a required criterion for the diagnosis of Behcet's disease?
Which of the following is a common gastrointestinal symptom associated with Behcet's disease?
What does the pathergy test assess in patients suspected of Behcet's disease?
Which treatment option is specifically mentioned for musculoskeletal manifestations of Behcet's disease?
Signup and view all the answers
What is a potential side effect of using thalidomide in the treatment of Behcet's disease?
Signup and view all the answers
Which laboratory feature can be abnormal in Behcet's disease?
Signup and view all the answers
What combination therapy may be used for severe Behcet's disease?
Signup and view all the answers
Which skin lesion is associated with Behcet's disease?
Signup and view all the answers
What is a key characteristic of Behçet's disease?
Signup and view all the answers
What is the male-to-female ratio commonly observed in Behçet's disease?
Signup and view all the answers
Which factor is associated with the genetic component of Behçet's disease?
Signup and view all the answers
What condition can resemble Behçet's disease due to similar nervous system symptoms?
Signup and view all the answers
What is typically associated with ocular inflammation in Behçet's disease?
Signup and view all the answers
Which type of arthritis is commonly observed in Behçet's disease?
Signup and view all the answers
What is one of the cardiovascular manifestations associated with Behçet's disease?
Signup and view all the answers
Which of the following statements is false regarding the epidemiology of Behçet's disease?
Signup and view all the answers
Study Notes
Introduction
- Behçet's disease is a rare vasculitic disorder marked by oral aphthous ulcers, genital ulcers, and uveitis.
Epidemiology
- Prevalence in Europe ranges from 0.3 to 6.4 per 100,000 individuals.
- Significantly more prevalent in regions along the Ancient Silk Road, particularly in Turkey (400 per 100,000).
- Familial clustering occurs rarely in Europe but is noted in up to 18% of Turkish cases.
- Typically presents in the 3rd decade of life.
- Affected ratio is 5 males to 1 female.
Etiology
- The specific cause remains unknown.
- Possible genetic involvement linked to HLA antigen B51.
- Environmental factors such as infections and smoking may contribute.
Clinical Manifestations
Mucocutaneous Involvement
- Oral Aphthae: Painful lesions that vary in size.
- Genital Aphthae: Last longer than oral lesions, deeper lesions with post-healing scars.
- Erythema Nodosum: More common in females.
- Other Lesions: Papulo-pustular lesions and superficial thrombophlebitis.
- Pathergy Test: Highly specific indicator for Behçet's disease.
Ocular Inflammation
- Characterized by anterior uveitis, potentially with:
- Hypopyon
- Panuveitis involving the posterior chamber
- Retinal vasculitis with complications.
Central Nervous System Involvement
- May include aseptic meningitis or parenchymal lesions causing focal or diffuse dysfunction.
- Predilection for diencephalon, midbrain, and brainstem, mimicking multiple sclerosis.
Musculoskeletal Manifestations
- Intermittent, symmetric oligoarthritis typically affecting knees, ankles, hands, and wrists.
- Commonly presents with arthralgia.
Cardiovascular Manifestations
- Risk of cerebral and deep venous thrombosis.
- Possible arterial complications such as stenoses, occlusions, or aneurysms.
Gastrointestinal Manifestations
- Symptoms include melena and abdominal pain.
- Inflammation can resemble inflammatory bowel disease (IBD).
Classification Criteria
- Recurrent Oral Ulcers: At least 3 episodes within 12 months.
- Requires 2 of the following:
- Eye Lesion: Anterior/posterior uveitis or retinal vasculitis.
- Skin Lesion: Erythema nodosum or pseudofolliculitis.
- Pathergy Test: Positive if interpreted within 24-48 hours.
Laboratory Features
- Acute-phase reactants may be normal or elevated.
- HLA B51 is associated with Behçet's disease in high-prevalence areas, indicating a connection to ocular disease.
Treatment
- Glucocorticoids: Topical or intralesional for managing aphthous lesions.
- Colchicine: Primarily for musculoskeletal symptoms; higher doses may cause GI intolerance.
- Thalidomide: Used for mucocutaneous lesions, but potential toxicity is a concern.
- Combination Therapy: Glucocorticoids combined with immunosuppressants for severe manifestations.
-
Specific Treatments:
- Pulmonary arterial aneurysms may respond to prednisone and cyclophosphamide.
- Cerebral venous thrombosis treated with anticoagulants and glucocorticoids.
- Budd-Chiari syndrome management includes anticoagulants and immunosuppressive medications.
- Cyclosporine may be used for uveitis, either alone or in combination.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
Description
This quiz covers the essential aspects of Behçet's disease, a rare vasculitic disorder characterized by significant symptoms such as oral and genital ulcers. You'll learn about its epidemiology, prevalence rates across different regions, and demographic trends. Test your knowledge on this unique medical condition prevalent along the Ancient Silk Road.
