August
12
19:53
Rheumatology

Behcet's disease: symptoms, treatment

Behcet's disease is characterized by three main symptoms - aphthous ulcers, genital ulcers, and uveitis.Hippocrates described Behcet's disease is still in the V century BC, but the first definition and references to diseases related to the Turkish dermatologist Behçet in 1924.All theories about the origin of Behcet's disease tend to the leading role of autoimmune managers.But in recent years, numerous studies suggest that the disease can be triggered by infectious agents (herpes simplex virus, a number of streptococci, staphylococci and Escherichia coli), which are commonly found in the mouth.

Content

  • 1 frequency
  • 2 morbidity and mortality in Behcet's disease
  • 3 Symptoms of Behcet's disease
    • 3.1 Specific changes:
      • 3.1.1 Defeatsskin and mucous
      • 3.1.2 eye disease
      • 3.1.3 Neurological manifestations
      • 3.1.4 vasculopathy
      • 3.1.5 Arthritis
      • 3.1.6 Gastrointestinal manifestations
      • 3.1.7 Defeats genitourinary system
      • 3.1.8 Renal
      • 3.1.9 Other symptoms
  • 4 diagnosis of Behcet's disease
    • 4.1 To confirm the diagnosis requires at least 2 main criteria:
    • 4.2 to confirm the diagnosis as additional criteria:
  • 5 Laboratory diagnosis
    • 5.1 clinical and biochemical blood
    • 5.2 specific analysis
    • 5.3 imaging studies
    • 5.4 Other tests
    • 5.5 Histological findings
  • 6 Differential diagnosis of Behcet's disease
  • 7 Treatment
    • 7.1 Therapy
    • 7.2 Surgical treatments
  • 8 Power
  • 9 Activity
  • 10 Medications
    • 10.1 Corticosteroids
      • 10.1.1 Methylprednisolone
      • 10.1.2 Prednisone
      • 10.1.3 Dexamethasone
    • 10.2 Immunosuppressive drugs
      • 10.2.1 Azathioprine (Imuran)
      • 10.2.2 cyclophosphamide
    • 10.3 Immunomoduljatory
      • 10.3.1 Colchicine
      • 10.3.2 sulfasalazine
      • 10.3.3 Levamisole
      • 10.3.4 Cyclosporine
      • 10.3.5 infliximab (Remicade)
      • 10.3.6 etanercept
  • 11 Observation
    • 11.1 Further observation at the hospital
    • 11.2 further outpatient
  • 12 The prognosis for patients with Behcet's disease

systems and involvement of Behcet's disease is limited to vascular lesions of these organs.Microscopic examination revealed signs of inflammation in the tissue with a large number of neutrophils.

Behcet's disease most commonly occurs in the territory of the Silk Road, from Central Asia to China.Turkey has the highest incidence - 420 cases per 100 000 population.In other countries - 1 in 15 000-500 000 people.In the Middle East, men are affected more often than women (5: 1), in Europe the opposite.The average age of manifestations of the disease - 25-30 years.In an earlier age in the development of Behcet's disease most commonly affects the eyes.

Men often develop serious complications: the gap artery thrombosis, CNS, and women more likely to develop skin lesions.

symptoms: blurred vision, periorbital pain, photophobia, injected sclera, lacrimation.

the precursors of blindness, including uveitis, said the program "Live healthy!»:

Behcet's disease is quite difficult to diagnose.This is due not only to the variability of the symptoms, but also the fact that the disease occurs in our country are rare.Due to the diverse manifestations of the disease the patient appointed following consultation of experts:

skin test: minimal damage to the skin leads to the formation of inflammatory papules 24-48 hours.

Although there is no specific histological changes for Behcet's disease, biopsy samples are signs of vasculitis and perivascular infiltration.CNS may be accompanied by meningeal and cerebral inflammation, brain atrophy and encephalomalacia (softening of the brain tissue).Thrombosis razvivayutsyadostatochno often, but they have to be differentiated from other diseases of vasculitis.

activity is not restricted except in cases where the patient struck hard joints.

Typically, Behcet's disease used immunosuppressive drugs.Since the true cause of the disease is unknown, treatment aims to reduce symptoms by suppressing the immune system.Unfortunately, these drugs increase the risk of infection.Symptomatic therapy directed at specific symptoms: ulcers, arthritis and so on. D.

can be administered orally or parenterally for systemic lesions, topical ulcers and lesions of the eye or intra - arthritis.

reduce inflammation by inhibiting leukocyte migration, and reversibly reduces the permeability of capillaries.Given intravenously in severe cases

dose

Adults: 1 mg / kg, depending on the clinical manifestations.

Children: As in adults.

Contraindications Hypersensitivity.Viral, fungal or tuberculosis skin lesions.

caution when: hyperglycemia, edema, osteonecrosis, peptic ulcers, osteoporosis, euphoria, psychosis, growth suppression, myopathy, infections.

reduces the release of inflammatory mediators, migration of neutrophils, monocytes and T cell function.

dose

Adults: 60 mg / kg, depending on the clinical manifestations.

Children: As in adults.

Contraindications No absolute contraindications;care in diabetes, hypertension, aseptic necrosis, cataracts and active infection.

Much as the advantages and disadvantages.Cells and stabilizes lysosomal membranes, enhances the synthesis of the surfactant increases the concentration of vitamin A in the serum.Side effects: hyperglycemia, hypertension, bleeding and ulcers, lesions of the central nervous system.Most of these effects depend on the dose.

dose

Adults: 0.5 mg / kg, depending on the clinical manifestations.

Children: 0.03-0.15 mg / kg

Contraindications Hypersensitivity.Fungal or viral infection.

Reduces immune response that causes the symptoms of Behcet's disease.

Appointed to suppress delayed hypersensitivity reaction.

dose Adults: 2-3 mg / kg, single or multiple doses, depending on the clinical manifestations.

Children: adult dose.

Contraindications Hypersensitivity.Acute cytopenia, liver dysfunction, acute liver disease.

alkylating agent which inhibits a variety of cellular functions.

dose Adults: 1-3 mg / kg, 500-1000 mg / m², depending on the clinical symptoms, blood reactions and toxicity.

Children: adult dose.

Contraindications Hypersensitivity.Infection.Acute dysfunction of the bone marrow cytopenia.

These drugs act on the immune system in different ways, which reduces symptoms of Behcet's disease.However, these drugs do not suppress the immune system.

applied in autoimmune diseases, but the mechanism of action is not yet clear enough.

dose

Adults: 0.6 mg / kg.

Children: 0.02 mg / kg.

Contraindications Hypersensitivity.Acute liver dysfunction and / or kidney disease, disorders of the blood system.

consists of the two drugs: sulfapyridine and 5-aminosalicylic acid, which are usually applied in rheumatoid arthritis.Also effective in inflammatory bowel disease, spondyloarthropathies.

dose Adults: 2-4 g / kg.

Children: 40-60 mg / kg.

Contraindications Hypersensitivity.

is used in patients with Behcet's disease with aphthous ulcers and sores in the genital area.

dose

Adults: 150 mg / kg twice a week.

Children: Not applicable.

Contraindications Hypersensitivity.

Used for uveitis.Originally used in transplant patients.Inhibits cell activation, especially in T-lymphocytes.

dose

Adults: 2.5-5 mg / kg.

Children: adult dose.

Contraindications Hypersensitivity.Uncontrolled hypertension, cancer.

Infliximab has established itself in the treatment of vasculitis localized in the central nervous system, ulceration of the colon and esophagus, panuveitah, ulceration of the skin and mucous membranes, polyarthritis.The dose of 3, 5 or 10 mg / kg.Infusion performed 1-4 times for 2 months, with / without a gradual increase in the dose.Remission in these patients increased from 2 months to 2 years.No particular side effects during use of the drug is not marked.The effect is noticeable at 24 hours after infusion.

dose Adults: 3-5 mg / kg in 4 infusions.

Children: Not applicable.

Contraindications Hypersensitivity.

soluble p75 anti-TNF protein.It inhibits the binding of TNF receptors, which in turn reduces the inflammatory and immune response.

Good results are observed within a week after initiation of therapy.

dose

Adults: 25 mg twice weekly or 50 mg once a week.

Children:

Up to 4 years old - to be determined.

4-7 years - 0.4 mg / kg twice a week.

Older than 17 years - is assigned as an adult.

Contraindications Hypersensitivity.Sepsis, vaccination with live vaccines.

Hospitalization in specialized department and the treatment caused the defeat of certain organs.

goal of treatment at a polyclinic stage is the formation of stable remission and reducing the doses of drugs that control the disease.

depends on the extent of damage and organ involvement.

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