Background

In the fifth century BCE, Hippocrates might have provided a description of Behcet disease. The Greek ophthalmologist Benediktos Adamantiades described a patient in 1930 who had iritis, phlebitis, oral and genital ulcers, and inflammatory arthritis. This condition holds the name of Hulusi Behcet, a Turkish dermatologist who first noticed it in a patient in 1924 and wrote up a description of it in a 1937 publication. 

Several sets of diagnostic and classification criteria for Behcet disease have been proposed since 1946. The most commonly used criteria is still the International Criteria for Behcet’s Disease (ICBD), which was released in 2006. 

Epidemiology

Prevalence: 

According to reports, there are 0.12–0.33 cases of Behcet disease for every 100,000 people in the US. But over the course of 45 years, data from a study of Olmsted County, Minnesota residents revealed a prevalence of 5.2 cases per 100,000 people. 

From the Middle East to China, the ancient Silk Road had the highest incidence and prevalence of Behcet disease. 

Behcet disease is most prevalent in Turkey, where there are 420 cases for every 100,000 people. There are 13.5 to 22 cases per 100,000 people in Japan, Korea, China, Iran, and Saudi Arabia, depending on the country. In North America and Europe, the prevalence is much lower, ranging from one case per 15,000–500,000 people. 

The Middle East and Japanese populations have the highest rates of Behcet disease prevalence. 

Age of Onset: 

People in their 20s and 40s are most likely to be diagnosed with Behçet disease. 25 to 30 years old is the average age at onset. active clinical disease and eye disease are more common in cases that occur before the age of 25. 

Prevalence by gender: 

The prevalence of sex differs by nation. Behcet disease is more common in men in the Middle East, where male-to-female ratios are 3.8:1 in Israel, 5.3:1 in Egypt, and 3.4:1 in Turkey. Females are slightly more likely to contract the disease in Brazil, Germany, and Japan. The prevalence of Behcet disease is higher in women in the US (5:1 female-to-male ratio). 

Risk Factors: 

Severe heart disease presentations are more common in men. Males are more likely to experience thrombophlebitis, neurologic disease, pulmonary aneurysms, and eye involvement. Nonetheless, skin lesions resembling erythema nodosum are more common in women. 

Anatomy

Pathophysiology

Behcet disease is a type of auto-inflammatory vasculitis that affects veins and arteries of all shapes and sizes. Vasculitic lesions in Behcet disease do not form giant cells or necrotizing vasculitis, in contrast to other vasculitides. Behcet disease is distinct in that it causes venular involvement and the development of pulmonary and arterial aneurysms. In addition, as in the case of other autoimmune diseases like systemic lupus erythematosus, patients with Behcet disease do not have particular autoantibodies. 

Causes of infection 

There is a chance that an infectious agent exposure will cause a cross-reactive immune response. The following have been suggested as infectious agents: 

HSV, or herpes simplex virus 

species of Streptococcus 

Types of Staphylococcus 

Escherichia coli 

Research on heat shock proteins (HSPs) shed light on potential pathways that could aid in the onset of Behcet disease. The identification of a greater than 50% homology between human HSP-60 and HSP-65 and mycobacterial HSP has led to the elicitation of an enhanced T-cell response in Behcet disease patients when exposed to both bacterial and human homogenates, as compared to controls in Turkish, Japanese, and United Kingdom populations. 

Genetic Factors: 

Gene polymorphisms encoding cytokines, activator factors, and chemokines have been associated with an increased susceptibility to Behcet disease, according to genome-wide association (GWA) studies. The following genes have specific single-nucleotide polymorphisms identified: 

• Met694Val is a mutation in the family Mediterranean fever gene (MEFV). 
• TLR4 (involved in innate immunity activation and pathogen recognition) 
• The gene ERAP1 codes for a molecule that helps white blood cells digest microbial proteins. 
• CCR1–CCR3 (involved in attracting effector cells to infection sites) 
• STAT4 (associated with an elevated risk of autoimmune disorders) 
• KLRC1-4KLRK1 

Neutrophils and T cells 

Behcet disease is characterized by systemic involvement of multiple organs, primarily due to the development of vasculopathic or vasculitic lesions in the affected areas. These regions might show microscopic proof of T cells and neutrophils infiltrating inflammatory tissue. 

Research on T cells has indicated a predominance of T-helper type 1 (TH1) responses. In peripheral blood, there are higher concentrations of both CD4+ and CD8+ lymphocytes, along with corresponding elevations of cytokines (interleukin [IL]–2 and interferon-γ [IFN-γ]). Additionally, it has been demonstrated that Behcet disease patients have higher serum levels of IL-12, which may facilitate the response. Patients with Behcet disease who had pulmonary symptoms had bronchoalveolar lavage specimens that showed decreased levels and impaired activity of natural killer cells.

Etiology

Although the precise cause of Behcet disease is still unknown, genetic factors and infectious agent exposure may interact, as discussed in pathophysiology. Two forms of vascular damage arise from an environmentally induced hyperactive primed state of autoimmunity. Vasculitic lesions are the first, and they could be widespread. The various organ systems affected determine the sequelae. 

Certain pathologic modifications result from thrombosis and/or clot formation brought on by the emergence of a hypercoagulable state rather than vasculitis. Studies have shown increased thrombin formation and the possible involvement of compromised fibrinolytic kinetics in the development of the hypercoagulable/prothrombotic state, though the exact mechanism is still unknown. In patients with Behcet syndrome, pathologic activation of the procoagulant cascade through endothelial injury has also been shown. 

Genetics

Prognostic Factors

The prognosis is influenced by where it occurs and degree of involvement. Aneurysms are a very serious consequence. Ischemia may occur distal to vascular lesions as a result of thrombotic events and vasculitis. Loss of vision may result from uncontrolled ophthalmologic involvement, including anterior and posterior uveitis. Neurologic involvement can result in lifelong impairments or even death and is indicative of a progressive disease. 

The following are some data on mortality and morbidity: 

In a French cohort of 817 patients, the mortality rate was 5% after a median follow-up of 7.7 years. 

The rupture of a coronary or pulmonary arterial aneurysm associated with Behcet disease has a high fatality rate. 

In one Turkish study, neurologic involvement was linked to mortality rates as high as 20% at a seven-year follow-up. 

Thrombosis can be fatal. 

An involvement of the central nervous system may result in death or irreversible deficits. 

When the eyes are involved, blindness may occur. 

A study comparing 298 pregnancies in 94 patients with Behcet disease to 219 pregnancies in 95 healthy controls found that miscarriage rates were higher and smaller babies were delivered by Behcet disease women (3214 versus 3351 g, respectively; P= 0.028). According to the authors, placental vasculitis may be the possible cause 

Clinical History

The International Study Group (ISG) for Behcet’s Disease provided clarification on the diagnostic standards for the condition in 1990. The ISG contrasted 914 patients with a history of aphthous ulcers with controls in terms of their clinical findings. At least three episodes of oral aphthoous or herpetiform ulcerations must occur within a 12-month period, either directly observed by a doctor or reported by the patient, in order to meet the initial diagnostic criteria. At least two of the following must also be shown in order for the diagnosis to be verified: 

recurring, excruciating vaginal ulcers that leave scars Ocular lesions, such as retinal vasculitis, hypopyon, or anterior or posterior uveitis Skin involvement is present in about 70% of patients. A range of skin lesions can result from Behcet disease, but the most prevalent ones are acneiform lesions, erythema nodosum, cutaneous hypersensitivity, and thrombophlebitis.  

The development of a sterile, erythematous papule with a diameter of 2 mm or more that appears 48 hours after a skin prick sterile with a sterile sharp, needle (22–24 gauge; a dull needle may be used as a control) is considered a positive result from allergy skin testing. 

Taking into account the aforementioned diagnostic criteria, case presentation frequently consists of the following features: 

involvement of multiple organ systems, typically beginning with mucocutaneous involvement and avoiding the liver, kidneys, and heart onset age of 25–35 years old manifestations specific to a particular organ that are identified by outbreaks and a relapsing/remitting pattern 

manifestations of the mucous membrane and skin: Usually excluding the nonkeratinized surfaces of the gums, dorsal tongue, and hard palate, oral lesions are found in keratinized areas of the oropharynx. Although only three times a year is specified in the ISG criteria, the lesions frequently recur and appear as crops or multiple lesions, making them difficult to differentiate from those caused by other causes. 

In the genital area of both sexes, skin lesions are common. Lesions can also form on the penile shaft, although scrotal involvement is the most common in males. Lesions most often occur in the labial region in females, but they can also sporadically appear in the vagina and on the perineum. Male genital ulcers are more painful and usually heal with scarring. Women’s ulceration development and menstruation may be related. 

In females, erythema nodosum-like nodules are more common in the lower extremities. They usually go away in two to three weeks but frequently come back. They are nodules, erythematous, and tender. Erythema nodosum could be a sign of mild Behcet syndrome. 

Ocular lesions: In 10–20% of Behcet disease patients, ocular presentations (retinalanterior or posterior uveitis, hypopyon, retinal vasculitis, and cystoid macular degeneration) are the initial signs of the illness. However, ocular involvement typically occurs 3–4 years after oral and genital ulcers. 

Common signs and symptoms include scleral injection, photophobia, periorbital pain, blurred vision, and excessive lacrimation. Men are more likely to present with more severe eye involvement, especially those of Iranian and Japanese descent. 

Recurrent posterior uveitis with high risk of blindness 

Blindness caused by ocular involvement typically appears within the first seven years. Those who experience symptoms later in the disease’s progression have a better prognosis. 

Neurological manifestations: Overall, neurologic symptoms and signs typically appear 1–8 years after the start of the disease, which is an uncommonly late manifestation. Among them are the following: 

Most of the time, memory is impacted, especially when it comes to recall and learning. Language, math, and orientation are frequently unaffected. The majority of the time, parenchymal symptoms involve the brainstem.  In 54% of cases, there are behavioral changes, mostly apathy or disinhibition. It is less common for ophthalmoplegia to cause seizures and bulbar signs. 

Some less typical results are as follows: 

thrombosis or vasculitis-related infarctions 

demyelination 

Infectious meningitis 

Meningeal lymphocytic infiltration 

Vasculopathy 

Aneurysms in the pulmonary arterial tree, which are frequently fatal, can be caused by Behcet disease. Right-sided cardiac thromboses are linked to pulmonary artery aneurysmal involvement, which can present as hemoptysis, coughing, dyspnea, or chest pain.  

Depending on where the lesions are located, vasculitis of the small and large vessels can cause a wide range of symptoms. 

Males are primarily affected by arterial disease; females are rarely affected.  

Venous involvement is more frequent than arterial involvement, typically manifesting as superficial thrombophlebitis. It is common to confuse superficial thrombophlebitis with erythema nodosum because it manifests itself in a linear fashion with erythema on top. When these linear areas of vasculopathy form in males, the affected areas thicken and swell like strings. 

Arthritis: 

Up to 60% of patients experience arthritis and arthralgias, which mainly affect the lower extremities, particularly the knee. Elbows, wrists, and ankles may also be the main areas affected. 

The type of arthritis is asymmetric, nondeforming, and can involve one or more joints in a monoarticular, oligoarticular, or polyarticular pattern. 

Rarely do symptoms become chronic; they often recur and abate. 

Moreover, diffuse arthralgias are typical. 

Genitourinary (GU) and Gastrointestinal (GI) symptoms: 

Behcet disease patients have GI involvement in 3–16% of cases. The ileocecal region and the esophagus are frequently impacted. Bloating, GI bleeding, and stomach pain are among the symptoms. Deep ulceration of intestinal sections frequently leads to complications. 

The GU can be involved in sterile urethritis, neurogenic bladder, and epididymitis. The classic signs of urinary retention can also be present in neurogenic bladder patients. 

Renal manifestations: 

There might be a lack of indicating of renal symptoms. According to one study, 1-29% of Behcet disease patients experienced these symptoms. 

Amyloidosis associated with it could occur. 

Nephritic-range proteinuria that is unintentionally identified frequently manifests first. There have also been some reports of IgA nephritis and proliferative and crescentic glomerulonephritis. 

Cardiac manifestations: 

The following are cardiac manifestations, which occur in 5–17% of cases: 

Thrombotic events and coronary vasculitis 

Pericarditis 

Heart Myopathy 

Endocarditis exhibiting fibrosis or granulomatous variations 

Regurgitation 

Diastolic failure 

Pulmonary manifestations: 

Up to 18% of patients with Behcet disease have lung involvement. There have been reports of pleural effusions, hypertension, and pulmonary vasculitis. One of the most dangerous side effects of Behcet disease is pulmonary aneurysms, which can cause severe hemoptysis. When a pulmonary aneurysm is found alongside a vasculitic illness, it strongly suggests Behcet disease. 

Physical Examination

Oral ulcers: 

In 50–70% of cases, recurrent mouth ulcers are the first clinical sign of Behcet disease; in up to 98% of cases, these ulcers eventually develop. Aphthous or herpetiform in nature, ulcers can develop in the gingiva, lips, tongue, buccal mucosa, hard palate, uvula, and posterior pharynx, among other keratinized areas of the oral cavity. The surface of the ulcers is typically covered in a white or yellowish pseudomembrane.  

Smaller ulcers typically heal in 7–10 days without leaving any scars. But can differ in size and have a duration of three to five weeks. A large ulcer (> 10 mm in diameter) may result from the fusion of multiple small ulcers.  Both large ulcers and their genital counterparts heal with scarring.  

Skin: 

Skin involvement is present in about 70% of patients. A range of skin lesions can result from Behcet disease, but the most prevalent ones are erythema nodosum, acneiform lesions, thrombophlebitis, and cutaneous hypersensitivity. 

More than two-thirds of Behcet disease patients have erythema nodosum, which is more common in women. Although the face, arms, and buttocks are occasionally affected, lesions typically affect the anterior surface of the legs. These are red, slightly elevated nodules that are tender and indurated beneath the skin. They usually involute without ulceration in 10–14 days. In patients with erythema nodosum, the concurrent presence of ulcers—which are more indicative of Behcet disease—may aid in narrowing the differential diagnosis. 

Observe the hair for signs of thinning or brittleness, which can occur in malnourished individuals. 

Examine the nails for evidence of pitting, ridges, or brittle nails, which can be related to nutritional deficiencies. 

Eyes: 

Of those with Behcet disease, 75% have ocular involvement. Results could reveal the following: 

Both forms of anterior uveitis and hypopyon formation 

blindness-causing posterior uveitis 

Insomnia 

Compartilhae 

vitreous vasculitis 

Ischemias 

Disturbance 

The disc appears edematous, and the retina appears detached. 

Fluorescein angiography reveals leaky retinal vessels, which can occasionally result in atrophy and fibrosis. 

Roughly one-third of cases have iridocyclitis with hypopyon, the classic ocular finding of Behcet disease. In many cases, a gonioscopy can reveal an occult hypopyon. A distinctive quality of the hypopyon in Behcet’s illness is its ability to shift in orientation in response to head movements and to form and disappear quickly without leaving any aftereffects. Iris atrophy, posterior synechiae, peripheral anterior synechiae, and other conditions may arise from repeated attacks. 

Nervous System: 

Certain patients with Behcet disease have been shown to have dementia and spastic paralysis due to pyramidal tract lesions. Symptoms of neurological disorders could be any of the following: 

Alterations in mental state 

Epilepsy 

Clonus 

An ideal Babinski sign 

difficulty swallowing and speaking 

psychological instabilities 

Sharp deafness 

Resistance or apathy are frequent. Learning and memory difficulties have been shown. Involvement of peripheral nerves is uncommon. Vascular System: 

The surface thrombophlebitis of lower-extremity frequently exhibits erythema and tenderness overlying it in a linear fashion. Sclerosed thrombophlebitis distinctly presents as a subcutaneous string-like quality. 

In certain instances, deep vein thrombosis (DVT) may occur, which usually presents as either localized discomfort or an uneven limb girth. 

Saadoun et al. discovered that in a sizable cohort of Behcet disease patients, 7.8% had cerebral venous thrombosis (CVT). The primary side effect of CVT was optic atrophy, which resulted in severe vision loss. Peripheral venous thrombosis and concurrent prothrombotic risk factors were linked to relapse of thrombosis, while papilledema and prothrombotic risk factors were independently associated with the occurrence of sequelae. In as many as 90% of patients, anticoagulant therapy proved to be both safe and successful. 

The signs of claudication may be caused by arterial vasculitis. 

Musculoskeletal system:  

Though it can manifest in any pattern, the arthritis primarily affects the lower extremities, particularly the knees. Joint involvement is often intermittent but persistent. Usually, arthritis does not cause deformity or destruction. Joint fluid content frequently only shows signs of inflammation. Rarely, aseptic necrosis develops. 

Gastrointestinal system: 

Intestinal perforations, bloody diarrhea, and stomach discomfort are all possible outcomes of ulcerative lesions. GI lesions typically occur in the ileocecal region and are indistinguishable from those linked to inflammatory bowel disease. 

Genitourinary system: 

Eighty percent of patients develop genital ulcers. They are severe lesions that resemble punched-out oral lesions. Genital lesions in females can occur in the vulva and vagina as well as the labial folds. They typically appear on the scrotum in men, but they can also form in the penile shaft and perianal area. The scrotal tenderness is a symptom of epididymitis. Compared to oral lesions, genital ulcers are deeper, last longer, and usually leave scars after healing. 

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

The differential diagnosis of a number of neurologic syndromes, such as the following, requires the consideration of neurologic Behcet disease: 

Multiple sclerosis 

disorders of movement 

stroke in young adults 

intracranial hypertension 

diseases causing sinovenous occlusive brain damage  

ophthalmologic considerations: 

When making a differential diagnosis, it is crucial to take into account alternative types of uveitis, particularly in patients exhibiting a mild or unusual presentation of Behcet disease. 

Recurrent iridocyclitis with hypopyon can also be brought on by human leukocyte antigen B27 (HLA-B27)-related anterior uveitis, although this condition is usually unilateral. 

It is necessary to take into account additional inflammatory processes that impact both eyes because Behcet disease is a bilateral panuveitis. Rather than causing strict vasculitis, syphilis causes retinitis along with vitreitis. Serology is utilized to confirm the syphilis diagnosis. 

Differential diagnoses: 

Inflammatory Bowel Disease(IBD) 

Polyarteritis nodosa 

Antiphospholipid syndrome 

SLE-systemic lupus erythematosus 

GPA, formerly Wegener Granulomatosis (Granulomatosis with Polyangiitis) 

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

Assessment and Diagnosis: 

Suppressing inflammation and lessening the frequency and severity of recurrences are the two main objectives of therapy for Behcet disease. In order for treatment to be effective, it must begin early. The selection of medication is contingent upon the locations, degree, and severity of the ailment. 

The course of treatment is determined by the specific patient, the extent of the illness, and the involvement of the major organs. The management of various aspects of Behcet disease is aided by the 2008-developed and 2018-updated European League Against Rheumatism (EULAR) recommendations for the disease. 

Medication: 

Topical steroids or sucralfate solution are the first-line treatments for mild, isolated oral and genital ulcerations. Mucocutaneous relapse has also been avoided with the use of colchicine. Systemic corticosteroids, azathioprine, pentoxifylline, dapsone, interferon-alfa, colchicine, and thalidomide have all shown promise for treating severe mucocutaneous lesions. The second-line treatment for mouth ulcers brought on by Behcet disease is apremilast. 

The first line of treatment for ocular diseases is generally agreed to be azathioprine. In cases of severe eye disease, such as retinal vasculitis, macular involvement, or a significant decline in visual acuity, azathioprine and corticosteroids may be combined with either infliximab or cyclosporine. When treating ocular manifestations, an expert panel has suggested using infliximab and adalimumab as first-line immunomodulatory drugs.  

Hospitalization or Intensive Inpatient Programs: 

Reduce medication to the lowest dose that manages disease activity as it starts to decline. Individual organ-system involvement is the foundation for inpatient care. Consider the particulars of each patient’s organ-system involvement when customizing the transfer scenario. 

Non-Pharmacological therapy: 

Surgical care: 

The following GI presentations call for surgical intervention: 

Stomach stenosis 

lesions that don’t respond to medication 

Fistula development 

A hole made in 

excessive bleeding 

Under the following conditions, surgery may also be considered: 

• Resection may be necessary for pulmonary aneurysms and regions that experience ischemia damage as a result of vasculitis or thrombosis. 
• Surgery is occasionally an option for treating coronary thrombosis, endocardial fibrosis, and ventricular aneurysms. 
• Surgical intervention may be necessary in certain cases for glaucoma, cataracts, and retinal detachment. 
• Certain cerebral aneurysms and clots may require neurosurgery to be corrected. 

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

lifestyle-modifications

Activity and Diet: For Behcet disease, there are no general dietary guidelines available. Individuals with inflammatory bowel disease are advised to adhere to the same GI guidelines as those with severe bowel involvement. A lot of the time, these patients must have complete parenteral nutrition. 

It is advised to only engage in activity as tolerated; limitations may apply due to arthritis or systemic symptoms. 

Take a break during the flares: When symptoms start to show up, give yourself some time. To ensure that you get the rest you require, be adaptable and modify your schedule when needed. Aim to reduce your stress. 

When you have the energy, exert yourself: Walking or swimming are examples of moderate exercise that can help you feel better in between flare-ups of Behcet’s disease. Exercise can lift your spirits, maintain the flexibility of your joints, and strengthen your body. 

Establish connections: It could be challenging to locate other people who have Behcet’s because it is an uncommon disorder. Find out from your physician about local support groups. The American Behcet’s Disease Association provides message boards and chat rooms where you can interact with others if you are unable to contact a close friend or family member. 

Use of corticosteroids

These medications can be applied topically for ulcers or ocular involvement, intra-articularly for arthritis, orally or parenterally for systemic symptoms. 

dexamethasone: increases the synthesis of surfactants, raises the concentration of vitamin A in the blood, stabilizes cell and lysosomal membranes, and suppresses the production of prostaglandins and proinflammatory cytokines (such as TNF-alpha, IL-6, IL-2, and IFN-gamma).

The recruitment of inflammatory cells into affected areas is inhibited by the inhibition of chemotactic factors and factors that increase capillary permeability. direct cytolysis is used to suppress the growth of lymphocytes and prevents mitosis. enhances pulmonary microcirculation and disintegrates granulocyte aggregates. 

methylprednisolone: reduces inflammation through reversing increased capillary permeability and inhibiting polymorphonuclear leukocyte migration. given intravenously in cases of extreme severity. 

prednisone reduces T-cell, monocyte, and neutrophil migration as well as the release of inflammatory mediators. 

All glucocorticoids have side effects, such as death, adrenal suppression, GI bleeding or perforation, weight loss, hyperglycemia, and hypertension. The majority of corticosteroid side effects are either duration- or dose-dependent. 

Immunosuppressive agents

These medications lessen the immunological reaction that results in Behcet disease signs and symptoms. 

cyclophosphamide: Strong alkylating agent that prevents a number of biological processes. DNA cross-linking, reduced DNA synthesis, and cellular death are caused by alkylation. 

azathioprine: An analog of purines that prevents DNA synthesis. The liver and red blood cells metabolize the 50 mg tablets into 6-mercaptopurine. 

chlorambucil: Strong alkylating agent that prevents a number of biological processes. DNA cross-linking, reduced DNA synthesis, and cellular death are caused by alkylation. Compared to cyclophosphamide, the onset of action is slower

Immunomodulators

By influencing the immune system in different ways, these medications lessen the autoimmune symptoms that are typical of Behcet disease. However, the widespread immunosuppression that is associated with immunosuppressive medications is not caused by immunomodulators. 

colchicine: suppresses the production of cellular microtubules and may result in leukopenia that is followed by leukocytosis. Its primary method of use in autoimmune diseases is empirical, and it is not a true immunomodulator with a clear mechanism of action in reducing inflammation. 

infliximab: infliximab is a monoclonal antibody that targets TNF.  stops the cytokine TNF-alpha from binding to the TNF-alpha receptor by neutralizing it. CNS vasculitis, colonic ulcers, esophageal ulcers, panuveitis, mucocutaneous ulcers, and polyarthritis have all been effectively treated with infliximab.  

etanercept: TNF receptor fusion protein soluble at position p75 (sTNFR-Ig). It prevents TNF from attaching to cell surface receptors, reducing immunological and inflammatory reactions. 

The use of etanercept in patients with Behcet disease was studied for four weeks, with a four-week washout period for systemic immunosuppressants. The study was double-blind and placebo-controlled. Etanercept 25 mg SC was administered twice a week to patients with arthritis and mucocutaneous lesions. 

tacrolimus: Streptomyces tsukubaensis is the bacteria that produces this immunomodulator. action mechanisms akin to those of cyclosporine. used to treat uveitis in Behcet disease, but mostly in transplants. 

cyclosporine: applied to uveitis. initially applied to patients undergoing transplants; now used to treat a range of immune-mediated diseases. without being cytotoxic, calcineurin inhibition prevents early-stage cellular activation, most notably T lymphocytes. 

levamisole: used to treat aphthous and genital ulcers in patients with Behcet disease. an immune modulator that has been authorized for use in colon cancer therapy. immune system is restored, T-cell activation and proliferation are stimulated, and monocyte function is increased. increases the adhesion, motility, and chemotaxis of neutrophils. 

thalidomide: used to treat aphthous ulcerations and possibly treat lesions of erythema nodosum. an immunomodulatory drug that has an unclear mechanism of action. possibly inhibit TNF-alpha. certain adhesion molecules are downregulated. 

PDE4 inhibitors and DMARDs: 

apremilast: Increased intracellular cAMP levels are the outcome of an oral small molecule inhibitor of phosphodiesterase-4 that is specific for cyclic adenosine monophosphate (cAMP). Recommended for mouth ulcers brought on by Behcet disease

Behcet’s syndrome is a complicated and long-lasting autoimmune condition that requires several stages of care to manage and treat its different symptoms. 

Evaluation and Diagnosis: 

Clinical Assessment: Behcet’s syndrome diagnosis starts with a comprehensive clinical evaluation. Doctors search for the typical trio of symptoms, which consists of genital ulcers, eye inflammation (uveitis), and recurrent oral ulcers. 

Differential Diagnosis: It is necessary to rule out other illnesses that have comparable symptoms, such as infections or autoimmune diseases. 

The International Study Group’s diagnostic criteria, which include recurrent oral ulcers along with two or more of the following: genital ulcers, skin lesions, eye involvement, or a positive pathergy test, can be used to diagnose Behcet’s syndrome. 

Management of acute flares:  

Anti-Inflammatory Drugs: Prednisone and other corticosteroids are frequently used to reduce inflammation and ease symptoms during acute flares. 

Colchicine: For certain patients, colchicine can be used as a long-term maintenance therapy because it works well in treating mucocutaneous symptoms. 

Topical Treatments: For genital and oral ulcers, topical treatments such as corticosteroid mouthwashes or gels can be helpful. 

Pain Relief: Pain can be controlled with over-the-counter or prescription painkillers. 

Maintenance Counseling: 

Immunomodulatory Drugs: To lessen the frequency and intensity of flare-ups, patients frequently need to take immunosuppressive drugs for an extended period of time. Methotrexate, mycophenolate mofetil, and azathioprine are typical choices. 

Biologic Agents: To target particular immune pathways in refractory cases, biologic agents like infliximab or adalimumab may be taken into consideration. 

Gastrointestinal and Mucocutaneous Symptoms: For the management of mucocutaneous and gastrointestinal symptoms, colchicine or other specialized treatments may be continued. 

Patients with vascular complications may be prescribed anticoagulants or antiplatelet agents to lower their risk of thrombosis and prevent blood clots. 

Corticosteroid Eye Drops: To manage inflammation, patients experiencing ocular involvement may need to use corticosteroid eye drops. 

Systemic Immunosuppressants: Drugs that suppress the immune system throughout the body may be necessary in cases of severe ocular symptoms. 

Ophthalmologic Evaluation: To identify and stop vision-threatening complications, ophthalmologists must perform routine eye exams. 

Vascular Involvement: Anticoagulation or Antiplatelet Therapy: Patients may require anticoagulants or antiplatelet agents to treat vascular complications. 

Interventional Procedures: To treat vascular issues, angioplasty or stent implantation may be required in some circumstances. 

Topical Treatments for Skin and Mucosal Lesions: Corticosteroid creams or ointments can be applied to treat skin and mucosal lesions. 

Systemic Therapy: Systemic immunosuppressive drugs may be necessary in cases of severe skin involvement. 

medication involvement in the gastrointestinal tract. Medication for pain relief, inflammation control, and nutritional deficiencies can all be used to treat digestive problems. 

Dietary adjustments, such as avoiding trigger foods, may be required to manage gastrointestinal symptoms. 

High-Dose Corticosteroids: When neurological involvement occurs, such as in central nervous system vasculitis, high-dose corticosteroids are frequently used as a first line of treatment. 

Immunosuppressive drugs: To treat severe neurological symptoms, additional immunosuppressive drugs might be needed. 

Psychological Support: In order to manage stress, keep a positive outlook, and deal with the chronic nature of the illness, patients may find it helpful to receive psychological support. 

Frequent Follow-Up: To evaluate the state of the disease, modify medication, and handle any new symptoms or complications, ongoing monitoring is necessary. 

For comprehensive care, coordination and communication between medical professionals from various specialties are essential. 

Medication

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References

https://www.ncbi.nlm.nih.gov/books/NBK470257/