Behcet's Disease

What Is Behcet's Disease?

Behcets disease or Behcets syndrome or silk road disease is rare but of a worldwide prevalence, especially along the silk road countries. It mainly affects the younger and middle-aged population between 20 to 40 years. It is a chronic syndrome and an inflammatory disorder involving the ocular, oral, and genital regions of the body. It is more prevalent between 20 to 40 years of age and with increased prevalence in men than in women. The other name for this syndrome is malignant apthosis. This mucocutaneous inflammatory disorder with uveitis and systemic vasculitis affects arteries and veins, leading to a multisystemic manifestation of a broadened disease spectrum.

The increased prevalence of this disease across the silk route gave it the name of the silk road disease (in people originating from the ancient trading routes between the china and Mediterranean basin). Though attributable genetic elements have been identified like HLA-B1 or B5 carriers who are at an increased predisposition to develop this disease, other factors like tumor necrosis factor or TNF, histocompatibility class 1 chain genes, and heat shock proteins have also been found to be carriers that may be inherited over a familial route. This autoimmune inflammatory condition is known to be due to the pathogenesis of the body's natural immune cells mistaking the healthy tissue cells to be foreign and attacking the blood vessels leading to systemic vasculitis.

How Does the Immune Response and Inflammation Happen?

Cell-mediated immunity in which the Type 1 helper T cell activation leads to increased circulating levels of T-lymphocytes accounts for the multisystemic presentation of Behcet disease. Pro-inflammatory cytokines (IL-1, IL-8, IL-12, IL-17, IL-37, and TNF) are involved in the pathophysiology behind the increased inflammatory response.

Mucocutaneous lesions, including oral aphthous ulcers and erythema nodosum, are attributed or researched due to this increased neutrophil activation (leading to a neutrophilic vascular reaction that causes tissue injury to the body's healthy tissue cells). Circulating immune complexes play a role in causing neutrophil stimulated vasculitis. Biopsy of the mucocutaneous lesions shows a predominance of neutrophil cells apart from endothelial swelling seen histopathologically.

Erythrocytes or the red blood cell extravasation is also a pathologic finding, along with fibrinoid necrosis of the blood vessels involved. The neutrophilic vascular reaction is the diagnostic criterion histopathologically in Behcet disease. The formation of aneurysms in the large arteries or vasculitis is a well-observed clinical feature. Exposure of certain infectious microorganisms like Streptococcus bacterium, Staphylococcus aureus, and Prevotella sp has remained elusive in linkage to Behcet's disease. However, research suggests that they may have an environmental role in causing systemic infection.

What Are the Patient's Signs and Symptoms?

The main clinical symptoms that should prompt the patient to consult the physician in this condition are usually the occurrence of these features:

• Reddening of the eyes

• Eye pain.

• Blurred vision.

• Mouth ulcerations.

• Genital ulcers.

• Skin acne.

• Headache.

• Joint pains.

• Joint stiffness or joint swellings.

What Are the Investigations Required?

Diagnosis of Behcet's disease can be made by-

• Complete blood count.

  Related Topics

  ---

  My daughter has swollen lymph nodes and positive ANA. Please provide opinion.

  Aortitis - Symptoms, Causes, Diagnosis and Treatment

  Budd-Chiari Syndrome - Causes, Symptoms and Treatment

• Routine blood examination.

• X rays.

• CT (computed tomography) or MRI (magnetic resonance imaging) scan.

• Skin Biopsy.

• Pathergy test (in which the skin tissue is needle pricked to observe and check if red spots begin to appear within 24 to 48 hours of the test time).

What Are the Systemic Manifestations?

Ocular Manifestations:

• Bilateral uveitis of the anterior and posterior uveal tracts.

• Erythema.

• Photophobia.

• Vision loss may be observed in progressive disease cases (untreated).

• Retinal vasculitis - leading to vision loss or blindness.

• Conjunctivitis (maybe rare but observed as well).

• Severe retinal disease. ·

Musculoskeletal manifestations:

• Oligoarthritis or polyarthritis or monoarthritis.

• Peripheral joint and spine involvement (arthritis).

Vascular and cardiac manifestations:

• Superficial or deep thrombophlebitis.

• Myocarditis.

• Endocarditis.

• Coronary artery vasculitis.

• Coronal aneurysms.

• Budd-Chiari syndrome or vena cava obstruction.

• Vasculitis of carotid, femoral and the popliteal arteries.

• Pulmonary aneurysms.

• Thromboembolism.

• Aortitis. ·

Neurologic Manifestations:

• Elevated protein in the cerebrospinal fluid.

• Cerebellar and pyramidal tract involvement.

• Cranial or peripheral nerve involvement.

• Headaches and papilledema.

• Sinus thrombi.

Gastrointestinal Manifestations:

• Mucosal ulcerations of ileum, cecum, colon, and esophagus.

• Ileocecal ulcerations or lesions.

Renal Manifestations:

• Amyloidosis.

• Glomerulonephritis. ·

Other systemic manifestations:

• Epididymitis.

• Anemia.

• Leukocytosis.

• Inflammatory marker elevation.

• Meningitis.

Orogenital Manifestations:

These are the most commonly reported and observed clinical features in a patient presenting with Behcet syndrome. Oral lesions are not only painful but may occur anywhere in the regions of the soft palate, hard palate, buccal mucosa, lips, gingival tissue, or the tonsils. These lesions are both painful and recurrent, and routine dental examination can also prompt the dental surgeon to suspect severe systemic illnesses associated with an inflammatory immune response presenting in the oral cavity. Oral ulcerations are not only indicative or confirmative in almost all patients presenting from Behcet disease but also are the first clinical features to present in the patient.

Genital ulcerations occur in up to 80% of affected patients and are recurrent. However, in sharp contrast to the nature of oral lesions or ulcers, genital ulcers are usually self-healing with a bit of scarring. These ulcers occur in the scrotum region of the males and the females upon the vulva or the vaginal parts.

What Is the Differential Diagnosis?

• Systemic lupus erythematosus ( SLE).

• Seronegative arthritis.

• Reactive arthritis.

• Multiple sclerosis.

• Inflammatory bowel disease (IBD).

• Sarcoidosis.

• Polychondritis.

• Herpetic infections.

• Sacroiliitis.

• Axial inflammatory arthritis.

• Systemic vasculitides.

How to Manage Behcet's Disease?

Behcet's disease is always associated with a significant physical and psychological impairment for life. Hence, multisystemic management should be provided which depends on medical intervention by the physician, gastroenterologist, dentist or maxillofacial surgeon, nephrologist, cardiologist, and neurologist as per the extent of organ damage and clinical symptoms exhibited by the individual. Younger individuals, especially the male population affected, need immediate treatment after detection of Behcet's disease. Otherwise, the long-term prognosis will be affected due to the high mortality rates. Hence early intervention is required. The treatment varies widely on the organ affected, ranging from corticosteroids to immunosuppressive agents. Surgery is mainly used to treat pulmonary or neural aneurysms (localized swelling of arteries are called aneurysms) and prevent their recurrence.

Conclusion:

To conclude, Behcet's disease is a lifelong systemic disease that needs multi-specialty intervention, psychological support, and, most importantly, diagnosis at the earliest to prevent the high rates of mortality risk that arise due to the complications developed by these individuals.