Behcet’s disease is a disease of a group of systemic vasculitis with lesions of small and medium–sized arteries and veins, recurrent erosive ulceration of the mucous membranes of the mouth, genitals, eyes, skin, involvement of internal organs and joints. Manifestations are diverse and may include aphthous stomatitis, genital ulcers, cutaneous and subcutaneous nodular erythema, uveitis, arthritis, vasculitis, lung, kidney, stomach dysfunction, meningoencephalitis. The diagnosis is based on clinical and laboratory data; treatment includes local and systemic immunosuppressive therapy. Disease proceeds with periods of relapses and temporary remission, complications from the concerned organs.
ICD 10
M35.2 Behcet’s disease
Meaning
The etiology of Behcet’s disease is unclear. Genetic conditioning plays a certain role in its development. It is known that the prevalence of the disease is higher among men 20-40 years old, inhabiting the Middle Eastern and Asian regions. The importance of infection and toxic factors in the development is taken into account. The influence of autoimmune mechanisms is not excluded: in patients with Behcet’s disease, CEC, antibodies to the cells of the oral mucosa are determined, suppression of specific and nonspecific immune protection is noted. The most likely is a simultaneous combination of several factors, when viral, bacterial, toxic, etc. agents provoke the onset of the disease, followed by the addition of autoimmune mechanisms.
Behcet’s disease symptoms
Behcet’s disease is polysymptomatic; the defining signs are aphthous ulceration of the mucous membranes of the mouth and genitals, inflammation of the eyes. On the lips and in the oral cavity (on the palate, tongue, gums, cheek mucosa), small vesicles filled with opaque contents appear, which open with the formation of bright pink ulcers (aft) with a diameter of 2 to 10-20 mm. The healing of ulcerative stomatitis in Behcet’s disease takes up to 1 month, but its relapses occur 3-4 times a year.
Genital ulcers are localized in men in the penis and scrotum, in women – on the mucous membrane of the vulva and vagina. Ulcers on the mucous membranes of the oral cavity and genitals are painful, merging with each other, forming continuous erosive surfaces. After the ulcers heal, scars may remain on the mucous membrane.
Symptoms from the organs of vision appear a few weeks after ulcerative stomatitis. Pathology is characterized by the development of recurrent iritis and iridocyclitis (inflammation of the iris and ciliary body of the eyeball), uveitis (inflammation of the vascular membrane of the eye), conjunctivitis (inflammatory changes in the mucous membrane), keratitis (inflammation of the cornea). Patients note lacrimation and pain in the eyes, blurred vision, increased photosensitivity.
Skin symptoms include erythema nodosum, rash, papules, ulcers, folliculitis, subarticular panaritia. From the musculoskeletal system, 50-60% of patients develop oligoarthritis or monoarthritis with lesions of the ankle, knee, elbow, wrist joints. Arthritis in Behcet’s disease proceeds without destruction of the joints. Neurological symptoms in Behcet’s disease are the development of meningoencephalitis, spinal and stem disorders, tetraplegia, hemiparesis, edema of the optic nerve disc due to increased ICP.
Disease is characterized by the development of intravascular inflammatory changes that cause narrowing of the lumen of veins and arteries, and then necrosis of their walls. Vascular disorders are manifested by vasculitis, thrombophlebitis of the veins of the lower extremities, thrombosis of the hepatic veins and pulmonary embolism, aneurysms and thrombosis of the aorta, ulnar, popliteal femoral arteries. Patients with Behcet’s disease are affected by the gastrointestinal tract (nausea, diarrhea, enteritis, colitis), heart (pericarditis, myocarditis, endocarditis), lungs (hemoptysis, pleurisy, diffuse infiltration of lung tissue).
Diagnostics
Clinical and diagnostic criteria of Behcet’s disease are the recurrent course of stomatitis, aphthous lesions of the genitals, arthritis, uveitis, vasculitis, meningoencephalitis. For suspicion of Behcet’s disease, the presence of any 3 of the listed symptoms is sufficient with the obligatory presence of aphthous stomatitis. To clarify the diagnosis, a consultation with a rheumatologist is necessary.
Of the diagnostic tests, the most important is the patergic test: patients with Behcet’s disease develop reactive inflammation 24-48 hours after subcutaneous injection at the injection site. A clinical blood test for Behcet’s disease shows moderate anemia, neutrophilic leukocytosis, increased ESR. In the biochemical analysis of blood, there is an increase in the values of fibrinogen, haptoglobin, α-2-globulins, seromucoid. The study of the coagulogram shows an increase in the activity of blood clotting factors.
Immunological tests reveal a decrease in the number of T-helper cells and T-lymphocytes, an increase in CEC, the level of complement components, immunoglobulins (IgA), etc. To diagnose joint changes in Behcet’s disease, radiography, ultrasound of the joint and arthroscopy are performed. According to the indications, a diagnostic puncture of the joint is performed. Neutrophils predominate in the synovial fluid, indicating the inflammatory nature of the process.
Behcet’s disease treatment
Behcet’s disease requires complex and long-term systemic therapy. The drug course includes the appointment of antiviral and antibacterial agents, multivitamins, immunosuppressants (chloroquine), anti-inflammatory and anti-inflammatory drugs (colchicine).
After 1-2 months of therapy, prednisone is added in the absence of dynamics; after another month, cytostatics (chlorambucil orally or cyclophosphane intravenously). Cytostatic therapy is carried out under the control of the dynamics of the general blood test, prescribing, if necessary, transfusion of platelet or leukocyte mass.
In Behcet’s disease, extracorporeal therapy is indicated – plasmapheresis, hemosobrtion, which have an immunomodulatory effect. Local symptomatic therapy for Behcet’s disease includes mouthwash, the use of hepatrombin and indomethacin ointments, etc. During an exacerbation of uveitis, subconjunctival administration of mezaton with dexamethasone, parabulbar injections of dexamethasone and heparin, methylprednisolone, triamcinolone are prescribed.
Complications and prognosis
The danger of eye damage in Behcet’s disease lies in the risk of secondary glaucoma, optic nerve atrophy, partial or complete loss of vision. A sharp decrease or complete blindness develops within 2-5 years from the onset of Behcet’s disease. Meningoencephalitis can lead to paralysis, hearing loss, blindness, mental retardation. Peripheral arterial thrombosis in Behcet’s disease often ends with the development of gangrene of the extremities.
In the isolated form, affecting only the lesion of the external mucous membranes, the prognosis is satisfactory. In cases involving the eyes, central nervous system, blood vessels, internal organs, the course of the disease is prognostically unfavorable. The prevention has not been developed due to the ambiguity of the causes and mechanisms of its development.