Behçet Disease (Behçet Syndrome / Malignant Aphthosis)
Description
- Introduction / History
Hulusi Behçet of Istanbul originally described it in 1937.
The classic trio consists of erythema nodosum, uveitis, vaginal ulcers, and mouth ulcers.
All diameters of veins and arteries are affected by systemic auto-inflammatory vasculitis.
- Causes
The reason is unknown.
Factors that contribute:
Genetic: highly related with HLA-B51/B5 (especially in Turkey, Japan, and the Middle East).
Staphylococcus aureus, HSV-1, Streptococcus sanguinis, and other infectious triggers.
Pathogenesis: An aberrant immunological response in a susceptible host due to an environmental stimulus.
- The study of epidemiology
Age: 20–40 years old (sometimes kids).
Gender: East Asia, the United States, and North Europe are dominated by women, whereas the Middle East is dominated by men.
Location: Along the Silk Road (with the highest in Turkey at 420/100,000).
Presence in the US: around 5.2/100,000.
Younger onset and more severe in males.
- Pathophysiology
Vascular and vein vasculitis (no large cells or necrosis).
Important immunological characteristics:
Activation of Th1 and Th17 - ↑ TNF, IL-1, IL-8, IL-12, and IL-17. Activation of neutrophils leads to a neutrophilic vascular response.
Deposition of the immune complex with endothelial dysfunction.
Venular involvement and pulmonary artery aneurysms are distinctive characteristics.
- Clinical Characteristics
- Mucocutaneous (distinctive)
Oral ulcers are painful (97–99%), recurring, and often heal without leaving scars.
80% of genital sores scar.
Skin lesions include superficial thrombophlebitis, acneiform eruptions, erythema nodosum, and pyoderma gangrenosum-like symptoms.
Pathergy test: 60–70% positive (especially in Turkey and Japan).
- Ocular
bilateral, recurrent, anterior + posterior ocular uveitis.
Hypopyon conceivable.
Blindness risk due to retinal inflammation.
- -Skeletal
arthritis of the knees, ankles, wrists, and elbows that is non-erosive and non-deforming.
- Vascular veins:
Budd-Chiari syndrome, superficial and deep vein thrombophlebitis.
Aneurysms in the arteries, particularly the pulmonary artery, are a leading cause of mortality.
- neurological (5–10%)
Parenchymal (80%): brainstem, pyramidal/cerebellar symptoms.
Non-parenchymal (20%): papilledema, headache, and dural sinus thrombosis.
- gastrointestinal
Aphthous-like ulcers, most frequently ileocecal.
May resemble IBD.
- Alternative systems
Heart conditions include coronary aneurysms, endocarditis, myocarditis, and pericarditis.
Amyloidosis and glomerulonephritis (rare) are renal conditions.
Reproductive: miscarriages, epididymitis.
Assessment and Diagnosis:
medical diagnosis (no diagnostic test).
Labs: nonspecific (↑ leukocytosis, chronic illness anaemia, and ESR/CRP).
imaging (CT, angiography, MRI) according to the involvement of organs.
The 2008 ICBD Criteria:
One point for oral aphthosis
Lesions on the skin (1 point)
Lesions in the arteries (1 point)
Test of Pathergy (1 point)
Two points for genital aphthosis
Lesions in the eyes (2 points)
Three or more points—diagnosis
Differentials:
IBD, Reactive arthritis, SLE, Herpes simplex, other vasculitides.
Protocol
Treatment
Depending on which organs are involved:
Mucocutaneous: anaesthetics, sucralfate, and topical steroids.
First-line: Colchicine (1–2 mg/day).
For oral ulcers, Apremilast.
TNF inhibitors (refractory), interferon, and azathioprine.
Topical steroids (anterior uveitis) are an eye condition.
Azathioprine with prednisone.
TNF inhibitors (adalimumab, infliximab) are considered severe.
NSAIDs and colchicine are first-line treatments for arthritis.
TNF inhibitors, azathioprine, and prednisone for refractory patients.
GI disorders: azathioprine with glucocorticoids.
if refractory, TNF inhibitors.
Vascular disease: cyclophosphamide with glucocorticoids for large arteries.
Immunosuppression with anticoagulation for venous thrombosis (case by case).
Neuro-Behçet: immunosuppressants combined with high doses of glucocorticoids.
In order to minimise problems and enhance quality of life, nursing treatments for Behçet illness are focused on symptom management, patient education, and facilitating interdisciplinary care.
Symptom Tracking and Treatment
Watch for any new or deteriorating lesions in your mouth, genitalia, skin, or eyes, and notify the medical staff right once if anything changes.
Use mild antiseptics or sterile saline to treat skin ulcers, and avoid doing thorough debridement unless absolutely required.
To ease the discomfort caused by mucosal ulcers, apply topical medications as directed and help with dental hygiene.
During flares, keep track of and document any symptoms, such as pain, fever, joint swelling, or changes in eyesight.
Administration of Medication and Instruction
Colchicine, corticosteroids, and immunosuppressive drugs should be used as directed while being closely watched for toxicity and adverse effects.
Advocacy for Patients and Supportive Care
To address emotional effects, offer psychosocial assistance and promote involvement in counselling or support groups.
Give advice on maintaining good oral hygiene and avoiding situations that might lead to mouth ulcers.
Encourage proper rest during acute flare-ups and moderate exercise during times of remission.
To guarantee thorough care, work together with the interdisciplinary team.
Education of Patients and Families
Inform patients and their families about Behçet illness, its possible side effects, and the need of getting help right away if you see any warning signs, such as excruciating eye pain, changes in vision, or neurological problems.
Reiterate the need of continuous follow-up and self-monitoring techniques for early problem detection.
For people with Behçet illness, these therapies are essential for avoiding organ damage, lowering morbidity, and promoting the best possible outcomes.
Notes
for more details visit https://www.ncbi.nlm.nih.gov/books/NBK470257/