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Adamantiades-Behcet disease

 
, medical expert
Last reviewed: 26.11.2021
 
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A multisystem inflammatory disease of a chronic nature, known as Behcet's disease (by the name of the Turkish dermatologist who described its three main symptoms), has another eponymous name - Adamantiades-Behcet syndrome or disease, which contains the name of a Greek ophthalmologist who made a great contribution to the study of this disease and its diagnosis.

But the name officially approved by WHO is Behcet's disease (according to ICD-10 code - M35.2). [1]

Epidemiology

According to world clinical statistics, the prevalence of Adamantiades-Behcet's disease per 100 thousand people is estimated at 0.6 cases in the UK, in Germany - 2.3 cases; in Sweden - 4.9; in the USA - 5.2; in France - 7; in Japan - 13.5; in Saudi Arabia -20; in Turkey - 421.[2], [3]

At the same time, among the patients, men aged 25-40 years predominate, but in the USA, China and northern European regions, the disease is more often detected in women. [4]

Younger patients have a more severe course of the disease. [5]

Causes Adamantiades-Behcet disease

Despite the fact that the causes of Adamantiades-Behcet's disease are not clearly defined, its etiological relationship with the autoimmune inflammation of the walls of blood vessels - systemic vasculitis - that affects the eyes, skin and other organs, is indisputable. [6]

According to the traditional international classification of systemic vasculitis (based on the size of predominantly affected blood vessels), Behcet's disease is a vasculitis that affects the endothelium - the inner lining of arterial and venous vessels of all sizes and types, causing their occlusion. [7]

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Risk factors

The immediate risk factors for the development of Behcet's disease (Adamantiades-Behcet) are genetic factors, that is, due to the predisposition of carriers of certain genes.

In particular, among residents of the Middle Eastern and Asian countries, the risk of developing this disease is increased in people with a mutation of the gene that controls the human leukocyte antigen HLA B51 and is associated with hyperreactivity of leukocyte neutrophils - mature blood phagocytes migrating through the blood vessels to the site of inflammation. [8], [9]

Also related to vasculitis are changes in genes encoding proteins that are involved in autoimmune and inflammatory reactions: tumor necrosis factor (TNF α), macrophage proteins (MIP-1β) and extracellular heat shock proteins (HSP and αβ-crystallin). [10], [11]

The effects of infections are not excluded as triggers: Staphylococcus aureus, Streptococcus sanguis, HSV-1 (herpes simplex virus type I), etc., causing an auto-inflammatory reaction in genetically predisposed people - with an increased level of IgG antibodies and general immune overexpression. [12]

See also -  What provokes systemic vasculitis?

Pathogenesis

The mechanism of vascular damage and systemic inflammatory manifestations in this disease continues to be the subject of study, but most researchers are confident that the pathogenesis of Behcet's disease is immune-mediated. [13]

Three options are considered:

  • deposition in the endothelium of immune complexes (molecules of associated antigens with antibodies);
  • humoral immune response, mediated by antibodies, which are secreted by plasma cells and circulate throughout the body (binding to exogenous and endogenous antigens);
  • cell-mediated response of T-lymphocytes - with an increase in the activity of tumor necrosis factor (TNF-α) and / or inflammatory protein of macrophages (MIP-1β). [14]

As a result of the action of one of these mechanisms, there is an increase in the level of pro-inflammatory cytokines, activation of macrophages and chemotaxis of neutrophils, which leads to dysfunction of endothelial cells (in the form of their swelling), autoimmune inflammation of the vessel walls with their subsequent obstruction and tissue ischemia. [15], [16]

Symptoms Adamantiades-Behcet disease

Adamantiades-Behcet's disease is paroxysmal, clinical symptoms are complex, and the first signs appear in patients, depending on the location of the lesion.

In almost all patients, at the initial stage, multiple painful ulcers in the oral cavity appear, which can spontaneously pass without leaving scars.

Eight patients out of ten also have recurrent genital ulcers (scrotum, vulva, vagina).

Skin symptoms are manifested in the form of erythema nodosum on the lower extremities, pyoderma, acne, inflammation of the hair follicles.

In half of the cases, ophthalmic manifestations occur in the form of recurrent bilateral  uveitis  (more often anterior) - with conjunctival and iris hyperemia, increased intraocular pressure, and accumulation of pus in the lower anterior chamber of the eye (hypopyon).[17]

Joint disorders (knees, ankles, elbows, wrists) include inflammatory arthritis (no deformity), polyarthritis, or  oligoarthritis .

The most common manifestation of the vascular system is deep and  superficial thrombophlebitis of the lower extremities ; a pulmonary or coronary artery aneurysm may form, and hepatic veins may be blocked. Inflammation of the pericardium becomes a fairly frequent cardiac manifestation of the disease.[18]

Among the lesions of the gastrointestinal tract, ulcers of the intestinal mucosa are noted. If the lungs are affected, patients have a cough and hemoptysis, and pleurisy may develop.[19], [20]

From the side of the central nervous system, lesions are noted in 5-10% of cases of Behcet's disease, and most often the brain stem suffers, causing impaired gait and coordination of movements, problems with hearing and speech, headaches, [21]decreased tactile sensitivity, etc.[22]

Complications and consequences

In addition to increased mortality (the risk of death increases the rupture of the resulting aneurysm of the pulmonary or coronary artery), this disease can give serious complications and have lifelong negative consequences.

Damage to the eyes in the form of uveitis can be complicated by persistent opacity of the vitreous body, resulting in blindness. [23]

Clinical manifestations of cardiac complications may include shortness of breath, chest pain, and fever.

Bowel ulcers can lead to perforation, lung damage can lead to pulmonary thrombosis, and central nervous system damage can lead to severe neurologic deficits.

Diagnostics Adamantiades-Behcet disease

Diagnosis of Adamantiades-Behcet Behcet's disease is clinical and includes examination of the oral cavity, skin, ophthalmological examination.

Diagnosis is based on three features, including mouth ulcers, eye inflammation, genital ulcers, and skin lesions.

Blood tests are taken: general, for markers of inflammation, for IgM autoantibodies (rheumatoid factor) and IgG antibodies, for CIC (circulating immunocomplexes). A biopsy of the affected skin is performed to confirm the dermatological diagnosis.[24]

Instrumental diagnostics is aimed at examining the affected organs and may include angiography, X-rays of the joints or lungs, computed and magnetic resonance imaging. [25]

Differential diagnosis

Differential diagnosis is of particular difficulty, which should exclude aphthous stomatitis, herpes vulgaris, reactive arthritis, ulcerative colitis and all inflammatory bowel diseases, systemic lupus, urethritis, and other systemic vasculitis. [26], [27]

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Treatment Adamantiades-Behcet disease

Treatment is prescribed taking into account the nature of the lesion and the clinical picture. [28]

To date, a fairly wide range of pharmacological preparations is used, in particular:

  • local or systemic glucocorticoids (Prednisolone, Methylprednisone, Triamcinolone);
  • NSAIDs (non-steroidal anti-inflammatory drugs);
  • interferon alpha (2a and 2b); [29]
  • Sulfasalazine (Azulfidine),
  • Azathioprine  (Imuran),
  • Colchicine;
  • Infliximab  and Rituximab;[30]
  • Adalimumab;
  • Cyclosporine ;
  • Cyclophosphamide and  Methotrexate ;
  • Etanercept.

Clinical practice confirms the effectiveness for achieving long-term remission of immunoglobulin therapy: subcutaneously administered synthetic leukocyte interferon. [31]

Prevention

To date, recommendations for the prevention of this disease have not been developed.

Forecast

With Adamantiades-Behcet's disease, the prognosis is disappointing: the disease is incurable. But in more than 60% of cases, after the active stage of the disease, a long remission occurs, and periods of exacerbation are easier.

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