Behcet Syndrome 

Updated: Jan 05, 2018
Author: C Egla Rabinovich, MD, MPH; Chief Editor: Lawrence K Jung, MD 

Overview

Background

Behçet syndrome is a multisystem disease of unknown etiology probably first described by Hippocrates in the 5th century. The syndrome carries the name of the Turkish dermatologist Hulusi Behçet, who, in 1937, described a syndrome of recurrent aphthous ulcers, genital ulcerations, and uveitis leading to blindness. Although the cause of the disease is still unknown, it has become recognized as a multisystemic inflammatory disease with a heterogeneity of clinical manifestations. Complicating matters, clinical presentations vary among geographical regions and manifestations tend to cluster together. See the images below.

MRI, T2-weighted images of brainstem involvement w MRI, T2-weighted images of brainstem involvement with meningoencephalitis in an 11-year-old girl with neurologic Behçet syndrome.
Histology of ulcers revealing neutrophilic infiltr Histology of ulcers revealing neutrophilic infiltrate and vasculitis.

Pathophysiology

Behçet syndrome is characterized by recurrent aphthous ulcers, genital ulcers, and uveitis or retinal vasculitis. Other manifestations of the disease include skin lesions, arthritis, GI lesions, CNS involvement, and vascular lesions, including aneurysms and thrombosis. In Behçet syndrome, the basic lesion is vasculitis. Biopsies have shown vasculitis near affected lesions, including the oral and genital ulcers and lesions of the CNS and the eyes; large vessels are affected by a vasculitis of the vasa vasorum. Vascular injuries may be superimposed on the hypercoagulability observed in some patients.

Neutrophilic hyperfunction is observed in patients with Behçet syndrome with neutrophilic infiltration of skin at the site of a prick with a sterile needle (the pathergy test). Lymphocyte function has also been reported as abnormal, with a clonal expansion of autoreactive T cells.

Results of recent genome-wide association studies (GWASs) confirm the association with HLA-B51, although there is currently not a diagnostic or prognostic role for the presence of HLA-B51. These GWASs demonstrate that the most common non–HLA association is with the interleukin (IL)–10 and IL23R loci and underline the essential role of the IL-10 and IL23/17 pathways in the pathogenesis of Behçet syndrome.[1] IL-6 has been shown to be elevated and IL-10 decreased in patients with Behçet syndrome compared with controls, and IL-6 is elevated in those with active disease.[2]

Epidemiology

Frequency

United States

Frequency data for Behçet syndrome should be considered suspect because of problems with case ascertainment.[3] This problem is inherent in any disease where no specific diagnostic test exists and only a set of clinical criteria is used for diagnosis. Figures available from Olmstead County, Minnesota reveal prevalence in this community to be 5 cases per 100,000 persons. Other estimates of prevalence vary from 0.12-0.33 cases per 100,000 persons.

International

Behçet syndrome is thought to be more common along the ancient Silk Road, extending from Asia to the Mediterranean. Estimates from Turkey vary from 80-370 cases per 100,000 population, whereas prevalence estimates from Japan, Korea, China, and the Middle East vary from 13-20 cases per 100,000 population. In northern Spain, prevalence has been reported as 0.66 cases per 100,000 population, whereas estimates from Germany are 2.26 cases per 100,000 population. 

A review by Leccese et al reported similarities in Behcet’s prevalence between regions with immigrant populations and originating countries, however, symptoms are less severe.[4]

Mortality/Morbidity

See the list below:

  • There is a wide range of phenotypic variations in Behçet syndrome, which may be due to genetic and ethnic differences.

  • Ocular: Uveitis occurs in 60-80% of patients. Retinal arterial and venous lesions are prognostic indicators for blindness, which is a major complication of Behçet syndrome. In Middle Eastern populations, the mean time from onset of disease to blindness is 3-4 years in untreated patients or in those treated only with corticosteroids.

  • CNS: Neurologic involvement is one of the most serious manifestations of Behçet syndrome, occurring in 10-30% of patients and carrying a poor prognosis. Manifestations include meningitis or meningoencephalitis; psychiatric symptoms, including personality changes; neurological deficits, including hemiparesis; and brainstem symptoms. Neurological deficits may be progressive, with 30% of those patients with neurologic manifestations eventually developing dementia. Compared with adults, children tend to have dural sinus thrombosis more often than parenchymal disease.[5]

  • Vascular: Vascular involvement in Behçet syndrome is unique in that it involves both the arterial and venous systems. Vascular complications, which occur in 7-40% of patients, include venous and arterial thromboses, vessel occlusions and stenoses, and aneurysm formation. Venous involvement typically includes superficial thrombophlebitis or deep venous thrombosis, usually of the lower extremities. Vena cava thrombosis can also occur, with extension to the hepatic vein, leading to Budd-Chiari syndrome and its associated morbidity and mortality. Patients with arterial manifestations may present with thrombosis or aneurysm formation with possible fatal rupture, especially if pulmonary arteries are involved.

  • GI: GI disease is associated with pyoderma gangrenosum and with papulopustular lesions, especially in children.[6] GI involvement consists of small vessel disease that involves mucosa and results in ulcers or large vessel disease that results in ischemia and infarction. The ileocecal region is the area most commonly involved with ulcers; the ulcers may be superficial or more typically deep with increased risk of perforation.

Race

Behçet syndrome is thought to be more common in Turkish, Asian, and Middle Eastern populations. However, the severity of disease may be increased in these populations, with better case ascertainment as a result. An increased incidence of skin pathergy and HLA-B51 antigen is observed in Middle Eastern and Asian patients, compared with North American or northern European patients.

Sex

In Japan and Korea, Behçet syndrome is more common in females, with a male-to-female ratio of 1:2, but it is more common in males in the Middle East, with a male-to-female ratio of 2:1. In the literature, estimates of male-to-female ratios range from 11:1 to 0.2:1. Despite the variability of the reported sex ratios, the disease tends to run a more severe course in males.

Age

Onset typically occurs in patients in the late third and early fourth decades of life. Onset during the childhood years is well recognized, but Behçet syndrome rarely occurs before school age. Mean age of onset for pediatric patients in a large Turkish series was 11.7 years, and the mean age of onset for neurological Behçet syndrome was 13 years.

 

Presentation

History

Two sets of criteria are commonly used for diagnosis of Behçet syndrome: an international criteria for Behçet syndrome, derived in 1990, and the O'Duffy criteria. Both sets of criteria may be too stringent for application in children who have lower risk for oral or genital ulcerations from other causes. In addition, these criteria have not been validated in children.

  • The international criteria include recurrent oral ulcerations, plus 2 of the following:

    • Recurrent genital ulcerations

    • Eye lesions

      • Anterior uveitis

      • Posterior uveitis

    • Cells in the vitreous

    • Retinal vasculitis

    • Skin lesions

      • Erythema nodosum

      • Pseudofolliculitis

      • Papulopustular lesions

      • Acneiform nodules (in a postadolescent patient not taking corticosteroids)

    • Positive pathergy test

  • The O'Duffy criteria require the presence of recurrent aphthous ulcerations, plus any 2 of following:

    • Genital ulcers

    • Uveitis

    • Cutaneous pustular vasculitis

    • Synovitis

    • Meningoencephalitis

    • Exclusion of inflammatory bowel disease, systemic lupus erythematosus (SLE), Reiter syndrome, and herpetic infections

  • Oral ulceration, the hallmark of this disease, is usually the initial clinical symptom and can precede other manifestations by years. Ulcers are typically painful, appear in crops, and are nonscarring. For diagnostic purposes, at least 3 episodes in a 12-month period are required. In one study of pediatric Behçet syndrome, the average time interval between the initial oral ulceration and the second manifestation was 8.8 years.

  • Genital ulcers appear in the vulva and vagina in females and scrotum and penis in men. Ulcers are painful, recurring, and scarring.

  • Ocular manifestations may be asymptomatic initially, or may present quite dramatically with hypopyon uveitis. Patients may report blurred vision, eye pain, photophobia, increased lacrimation, and erythematous conjunctiva.[7, 8]

  • Skin manifestations are nonspecific and include erythema nodosum, folliculitis, and pustular rash.

  • Arthralgias and arthritis can occur in small or large joints. Sacroiliitis has been described in HLA-B27–positive patients.

  • Gastrointestinal symptoms are common and include abdominal pain, diarrhea, and melena. Perforation may occur.

  • CNS involvement may occur in up to 25% of children and is the most severe manifestation of the disease. Patients with meningoencephalitis present with headache and stiff neck; focal neurological abnormalities can also be observed. Neuropsychiatric symptoms include hallucinations and personality changes. Other features that have been described include brainstem lesions; pseudotumor cerebri; cranial nerve palsies; and pyramidal, extrapyramidal, and cerebellar symptoms.

  • Vascular manifestations are varied and depend on the type and location of the vessel involved. The most common vascular complaints are secondary to venous thrombosis, often of the superficial veins.[9, 10] This can occur after venipuncture. Patients who develop superficial thrombophlebitis are more at risk than other patients with Behçet syndrome for the development of deep vein thrombosis and arterial disease. Well-known syndromes of large venous occlusions, such as Budd-Chiari syndrome or superior vena-caval syndrome, may occur. Patients with cerebral venous thrombosis develop signs and symptoms of increased intracranial pressure, such as headache and visual blurring. Arterial occlusions may present with symptoms related to ischemia. Cigarette smoking may be a risk factor for arterial disease in Behçet syndrome.

  • Pulmonary manifestations include pulmonary vasculitis and pulmonary arterial aneurysm formation; patients may present with hemoptysis, dyspnea, chest pain, or cough.

  • Studies have found that fatigue is common in clinically active Behcet's syndrome patients compared with healthy controls and inactive BS patients. The study also noted that depression, anxiety and physical dysfunction were significantly associated with fatigue.[11, 12]

Physical

See the list below:

  • Physical findings vary, reflecting the disease manifestations in a particular patient.

    • Oral ulcerations: Ulcers are typically 2-15 mm in diameter, with a necrotic center and surrounding red rim. A white or yellow pseudomembrane covers the surface of the ulcer. The ulcers are typically painful, nonscarring, and found on the lips, buccal mucosa, tongue, tonsils, and larynx. Most last 7-14 days and occur in crops.

    • Genital ulcerations: These typically occur less often than the oral ulcerations. The ulcers occur on the scrotum and vulva, are painful and heal with scarring, especially on the scrotum. Genital ulcerations tend to be deeper and larger than the oral lesions. Females can have asymptomatic ulcers, especially in the vagina.

    • Ocular manifestations: Uveitis can occur in both the anterior and posterior chambers of the eye.[13] Frank pus (hypopyon) may be observed in the anterior chamber. Retinal vasculitis is the most serious ocular finding. Vaso-occlusive lesions of the retinal vessels may cause a progressive decreased visual acuity. A slit lamp examination is necessary for diagnosis of uveitis, and fluorescein angiography is useful to identify retinal lesions.

    • Skin manifestations: Erythema nodosum lesions typically occur on the extremities, especially the lower legs, but they can also be observed on the face, neck, and buttocks. The lesions are painful and resolve spontaneously, although some may ulcerate or leave hyperpigmentation. A folliculitislike rash, resembling acne vulgaris, appears on the face, neck, chest, back, and hairline of patients. Some lesions become more pustular; 24-48 hours after a sterile needle prick, some patients develop erythema with a nodule or pustule at the prick site. This pathergy response is commonly observed in patients from Asia and the Middle East and is uncommon in northern European and North American patients.

    • Skeletal involvement: Monoarthritis or polyarthritis occurs in at least 50% of patients. Knees are the most commonly affected joints, followed by wrists, ankles, and elbows. The arthritis is typically nonerosive.

    • GI manifestations: In addition to the oral mucosa, ulcerative lesions may occur anywhere in the GI tract. Most commonly, ulcers occur in the ileocecal region. Other involved areas include the transverse and ascending colon and the esophagus. Anticoagulation is controversial in patients with Behçet syndrome because of the risk of bleeding from one of these ulcers.

    • CNS involvement: CNS involvement occurs in as many as 25% of patients and may be the most serious manifestation of disease. The immune-mediated meningoencephalitis that is most commonly seen predominantly involves the brainstem. Dural venous sinus thrombosis is less common. Findings may include meningitis, encephalitis, focal neurological deficits, and psychiatric symptoms. The CNS lesions may have exacerbations and remissions. In some patients, irreversible dementia ultimately results.

  • Vascular involvement

    • Venous involvement includes migratory superficial thrombophlebitis of the skin and deep venous thrombosis. Patients with lower extremity deep vein thrombosis may have distal edema. With chronic venous occlusion, collateral circulation may develop.

    • Arteritis may involve the aorta or its branches and lead to aneurysm formation. Rupture of aneurysms may be fatal. Pulmonary artery involvement may result in aneurysm formation with pulmonary artery–to–bronchus fistula formation and resultant hemoptysis. Aneurysm formation carries a worse prognosis than occlusive disease. Patients with pulmonary aneurysms often have extrapulmonary vascular complications, such as superficial or deep vein thrombosis.

    • Cardiac valves may develop vegetations with subsequent emboli. Clinically, these lesions are similar to bacterial endocarditis, but cultures are negative, and round cell infiltration is most typically observed on histology. Right ventricular thrombi may also develop, and are frequently found in patients with pulmonary aneurysms.

    • Nephrotic syndrome and kidney amyloidosis have rarely been described in patients with Behçet syndrome.

  • Muscular involvement: Myositis has been described in pediatric Behçet syndrome.

Causes

See the list below:

  • The etiology of Behçet syndrome is unknown. Behçet syndrome is thought to be caused by a combination of hereditary and environmental factors. The HLA-B51 allele (one of the split antigens of B5) is commonly found in patients from Asia and the Middle East, yet it is rarely found in northern European and North American patients. Infections may play a pathogenic role, as patients who have Behçet syndrome have a higher incidence of antibodies to herpes simplex virus, hepatitis C virus, and parvovirus B19. Streptococcal antigens have also been implicated; a trial of prophylactic penicillin treatment decreased the number of acute arthritis episodes in patients with Behçet syndrome.

  • Patients who have a parent with Behçet syndrome have disease onset at a younger age (genetic anticipation). In addition, pediatric patients are more likely to have a family history of Behçet syndrome, compared to patients with disease onset as an adult.

 

DDx

 

Workup

Laboratory Studies

See the list below:

  • No specific laboratory test result is diagnostic of Behçet syndrome.

    • Serum complement levels are within the reference range, except for just prior to eye or mucous membrane involvement, at which time they may be decreased.

    • Sedimentation rate or C-reactive protein may be elevated. Chronic anemia common, and a neutrophil leukocytosis is seen in about 15% of patients.

    • Human leukocyte antigen (HLA)-B51 may be present in patients of Asian, Mexican, or Middle Eastern descent.

    • Anticardiolipin antibodies are present in as many as 30% of patients.

  • Systemic lupus erythematosus and other vasculitic syndromes must be ruled out. Patients with Behcet syndrome have negative antinuclear and antineutrophilic cytoplasmic antibodies.

  • In patients with CNS findings, cerebral spinal fluid pleocytosis may be present.

  • In addition to thrombosis associated with antiphospholipid antibodies, thrombosis has been reported in Behçet syndrome associated with factor V Leiden mutations and with prothrombin G20210A mutations.

Imaging Studies

See the list below:

  • Brain MRI and/or CT scanning for visualization of the neurological lesions is often helpful in patients with CNS involvement. Focal lesions may be observed anywhere in the CNS on the MRI, appearing as high signal on the T2-weighted images and low signal on the T1-weighted images. Flare images may be especially helpful. Enlargement of ventricles or subarachnoid spaces may be observed. However, the MRI findings of the brain may be normal even in the presence of neurologic involvement. Neuropsychologic testing results may be abnormal prior to any detectable lesions on neuro-imaging.

  • Angiography shows areas of aneurysm formation and thrombosis.

  • Echocardiography is useful in patients with murmurs because it is useful for diagnosing the valve vegetations and ventricular thrombi, which can occur in Behçet syndrome.

    MRI, T2-weighted images of brainstem involvement w MRI, T2-weighted images of brainstem involvement with meningoencephalitis in an 11-year-old girl with neurologic Behçet syndrome.

Other Tests

See the list below:

  • Endoscopy of the GI tract is useful for detecting gastrointestinal ulcerations.

  • A thorough eye examination by an experienced ophthalmologist is essential. Consider fluorescein angiography for evaluation of retinal vessels. Follow-up visits with an ophthalmologist should be scheduled at least every 6-12 months.

  • Neuropsychologic testing may be useful with CNS involvement, revealing memory impairment or personality changes, and can be useful in monitoring neuropsychologic status.

Histologic Findings

See the list below:

  • Behçet syndrome is diagnosed clinically, not by means of tissue evaluation. However, round cell infiltration may be found in cardiac valve lesions.

  • Biopsy of the buccal and genital ulcers reveals lymphocytic and plasma cell invasion in the prickle cell layer of the epidermis.

  • Dermal vessels are infiltrated with lymphocytes and plasma cells with immune deposits of immunoglobulin M (IgM) and C3. Occasionally, necrotizing vasculitis is observed.

    Histology of ulcers revealing neutrophilic infiltr Histology of ulcers revealing neutrophilic infiltrate and vasculitis.
 

Treatment

Medical Care

See the list below:

  • Treatment of Behçet syndrome must be tailored to each patient's clinical manifestations. Corticosteroids are considered palliative; they are useful in controlling acute manifestations, but progression of CNS and ocular disease may occur in patients treated with corticosteroids alone.

  • Cytotoxic medications are usually indicated in patients with ocular, CNS, and vascular disease. Biologic medications are also being used in patients with these complications. Decreasing morbidity and mortality is the goal of treatment for children with Behçet syndrome.

  • The European League Against Rheumatism (EULAR) has recently released guidelines for the management of Behçet disease.[14]

  • There are not drugs targeted or indicated for Behçet syndrome by US regulatory agencies; therefore, the use of medications is considered off-label.

Surgical Care

See the list below:

  • Surgical resection of aneurysms with graft placement should be considered if feasible because of the high risk of aneurysmal rupture. However, complications of arterial surgery, such as aneurysms at the surgical site (similar to a pathergylike effect) and local thrombus formation, commonly occur.

Consultations

A rheumatologist should be consulted for all patients with Behçet syndrome. For children, a pediatric rheumatologist is preferable. Consider other consultations depending on patient signs and symptoms.

  • All patients should have regular eye examinations by an ophthalmologist experienced with vasculitis.

  • Consultation with a neurologist should be considered for patients with CNS symptoms.

  • A consultation with a gastroenterologist is appropriate for evaluation and management of abdominal symptoms.

  • Consultation with a vascular surgeon is important for patients with aneurysm formation.

Diet

See the list below:

  • No specific dietary recommendations are needed for patients with Behçet syndrome. However, patients on long-term corticosteroid treatment should avoid excessive weight gain and follow a low-salt, low-fat diet.

Activity

See the list below:

  • Restriction of activity should be tailored to a patient's clinical manifestations.

 

Medication

Medication Summary

Choice of medications depends on a patient's clinical manifestations of Behçet syndrome.

  • Ocular, CNS, and large vessel involvement requires corticosteroids and a second-line agent such as a cytotoxic or biologic medication. Corticosteroids have a suppressive effect on most manifestations of Behçet syndrome but do not prevent dementia or blindness and are associated with side effects that increase with duration of therapy.

  • Azathioprine is widely accepted as initial therapy for significant ocular involvement, especially posterior segment disease. Cyclophosphamide, cyclosporine, and azathioprine may be used for large vessel involvement, with cyclophosphamide preferred for arterial aneurysms. Corticosteroids, azathioprine, interferon-alfa, cyclophosphamide, methotrexate, and tumor necrosis factor-alpha (TNF-alpha) blockers have been reported to be efficacious in CNS disease; cyclosporin should be avoided in CNS involvement. Increasing reports and case series suggest the efficacy of biologics in major organ system involvement, especially TNF-alpha blockade.

  • Colchicine is used for treatment and prevention of ulcerations and treatment of arthritis. Corticosteroids, sulfasalazine, and azathioprine have also been shown to be useful in ulcer disease, and TNF-alpha antagonists and thalidomide have been reported to be efficacious in resistant disease.

  • Close communication between subspecialists, such as ophthalmologists and neurologists, is important for patient care.

Antigout medications

Class Summary

These agents are useful in decreasing frequency of mucosal ulcerations, the skin findings of pseudofolliculitis and erythema nodosum, and can be useful in the management of uveitis and retinal vasculitis.

Colchicine

Mechanism of action is unknown, but may have to do with decreased motility and lactic acid production of leukocytes. First-line therapy for PO ulcerations, ocular manifestations, and skin lesions.

Corticosteroids

Class Summary

This agent decreases acute inflammatory manifestations of Behçet syndrome. Depending on patient needs, this agent may be administered topically, orally, parenterally, or by intraocular injection.

Triamcinolone topical (Aristocort, Kenalog)

Topical treatment is useful to decrease the pain and inflammation of aphthous ulcers.

Betamethasone ointment (Alphatrex, Diprolene, Maxivate)

Useful to decrease the pain and inflammation of genital ulcers.

Dexamethasone injectable (Decadron)

Administered subtenon intraocular injection for retinal vasculitis.

Prednisone (Deltasone, Orasone)

Low-dose: Second-line therapy for erythema nodosum, anterior uveitis, and arthritis. High-dose: First-line therapy for GI lesions, acute meningoencephalitis, chronic progressive CNS lesions, and arthritis. Second-line treatment for retinal vasculitis and venous thrombosis.

Methylprednisolone (Medrol, Solu-Medrol)

Used as first-line therapy for acute meningoencephalitis, chronic progressive CNS lesions, and arteritis. Alternate therapy for GI lesions and venous thrombosis.

Immunosuppressant agents

Class Summary

These agents are used for the more serious long-term effects of Behçet syndrome (ie, ocular and CNS involvement, severe vasculitis).

Azathioprine (Imuran)

Used as alternate therapy for retinal vasculitis, arthritis, chronic progressive CNS lesions, arteritis, and venous thrombosis.

Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.

Chlorambucil (Leukeran)

Used as alternate therapy for retinal vasculitis, chronic progressive CNS lesions, arteritis, and venous thrombosis.

Alkylates and cross-links strands of DNA, inhibiting DNA replication and RNA transcription. Many are discarding this therapy because of significant cumulative toxicity and increased risk of malignancy.

Cyclophosphamide (Cytoxan, Neosar)

Used as alternate therapy for retinal vasculitis, arthritis, chronic progressive CNS lesions, arteritis, and venous thrombosis.

A cell cycle phase–nonspecific antineoplastic agent and immunosuppressant. A prodrug that requires activation by the cytochrome P-450 system in order to be cytotoxic.

Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

Because of toxicities, cyclophosphamide is being replaced by calcineurin inhibitors and antitumor necrosis factor agents where available.

Methotrexate (Folex PFS, Trexall)

An antimetabolite that interferes with the enzyme dihydrofolate reductase, leading to depletion of DNA precursors and inhibition of DNA and purine synthesis, particularly adenosine.

Unknown mechanism of action in treatment of inflammatory reactions (although may involve adenosine receptors and cell-cell adhesion); may affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). Adjust dose gradually to attain satisfactory response.

Cyclosporine (Sandimmune, Neoral)

First-line therapy for retinal vasculitis.

Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft versus host disease for a variety of organs. For children and adults, base dosing on ideal body weight.

Immunomodulatory agent

Class Summary

Thalidomide has a broad range of immunomodulatory properties. Use of this drug is limited by well-documented teratogenicity and potentially irreversible peripheral neuropathy.

Thalidomide (Thalomid)

Used for aphthous ulcerations and may also be effective in erythema nodosum lesions. An immunomodulatory agent whose mode of action is not fully known. May suppress TNF-alpha. Downregulates some adhesion molecules.

Tumor necrosis factor antagonists

Class Summary

Blockade of TNF-alpha by biologics have been shown in uncontrolled reports to be beneficial in uveitis, severe GI disease, severe ulcerations, and CNS vasculitis. Long-term efficacy is unknown.

Etanercept (Enbrel)

Soluble p75 TNF receptor fusion protein (sTNFR-Ig). Inhibits TNF binding to cell surface receptors, which, in turn, decreases inflammatory and immune responses.

Infliximab (Remicade)

Neutralizes cytokine TNF-alpha and inhibits it from binding to TNF-alpha receptor.

Adalimumab (Humira)

Recombinant human IgG1 monoclonal antibody specific for human tumor necrosis factor (TNF).

Rheostatic agents

Class Summary

These agents are used to improve peripheral blood flow and to improve delivery of oxygen to tissue suffering from vascular disease.

Pentoxyfylline (Trental)

Inhibits production of various proinflammatory cytokines, particularly TNF. FDA-approved for use in peripheral vascular disease. May alter rheology of red blood cells, which in turn reduces blood viscosity. Has been reported to be helpful in orogenital ulcerations.

 

Follow-up

Further Outpatient Care

See the list below:

  • Close follow-up care is warranted to monitor clinical status, including routine eye examinations.

Further Inpatient Care

See the list below:

  • Patients with Behçet syndrome may need to be admitted during acute flares for intravenous therapy or for diagnostic testing or surgical intervention.

Inpatient & Outpatient Medications

See the list below:

  • Medications (see Medication) vary depending on the clinical manifestations. Monitor drug side effects and efficacy closely.

Transfer

See the list below:

  • Transfer to a tertiary care center for diagnosis and intervention may be warranted, depending on the clinical symptomatology.

Deterrence/Prevention

See the list below:

  • No preventative measures are known for Behçet syndrome.

Complications

See the list below:

  • Complications include those caused by medications and the primary disease, and they vary depending on clinical presentation and disease manifestations.

Prognosis

See the list below:

  • Prognosis depends on clinical manifestations. The worst prognoses are associated with retinal vasculitis, leading to blindness; vascular aneurysm formation, with possible rupture; and neuro–Behçet syndrome, which may lead to dementia despite appropriate aggressive treatment.

Patient Education

See the list below:

  • Patients must be educated in disease manifestations, long-term prognosis, and medication side effects.

 

Questions & Answers

Overview

What is Behcet syndrome?

What is the pathophysiology of Behcet syndrome?

What is the global prevalence of Behcet syndrome?

What is the racial predilection of Behcet syndrome?

What is the prevalence of Behcet syndrome in the US?

What is the morbidity associated with Behcet syndrome?

What is the sexual predilection of Behcet syndrome?

Which age groups have the highest incidence of Behcet syndrome?

Presentation

What are the O'Duffy diagnostic criteria for Behcet syndrome?

What are the limitations of diagnostic criteria for Behcet syndrome?

What are the international diagnostic criteria for Behcet syndrome?

What are the signs and symptoms of Behcet syndrome?

Which physical findings are characteristic of Behcet syndrome?

Which vascular findings are characteristic of Behcet syndrome?

Which muscular findings suggest Behcet syndrome?

What causes Behcet syndrome?

DDX

What are the differential diagnoses for Behcet Syndrome?

Workup

What is the role of lab testing in the workup of Behcet syndrome?

What is the role of imaging studies in the workup of Behcet syndrome?

What is the role of endoscopy in the workup of Behcet syndrome?

What is included in an eye exam for the evaluation of Behcet syndrome?

What is the role of neuropsychologic testing in the workup of Behcet syndrome?

Which histologic findings are characteristic of Behcet syndrome?

Treatment

How is Behcet syndrome treated?

What is the role of surgery in the treatment of Behcet syndrome?

Which specialist consultations are beneficial to patients with Behcet syndrome?

Which dietary modifications are used in the treatment of Behcet syndrome?

Which activity modifications are used in the treatment of Behcet syndrome?

Medications

What is the role of drug treatment for Behcet syndrome?

Which medications in the drug class Rheostatic agents are used in the treatment of Behcet Syndrome?

Which medications in the drug class Tumor necrosis factor antagonists are used in the treatment of Behcet Syndrome?

Which medications in the drug class Immunomodulatory agent are used in the treatment of Behcet Syndrome?

Which medications in the drug class Immunosuppressant agents are used in the treatment of Behcet Syndrome?

Which medications in the drug class Corticosteroids are used in the treatment of Behcet Syndrome?

Which medications in the drug class Antigout medications are used in the treatment of Behcet Syndrome?

Follow-up

What is included in the long-term monitoring of Behcet syndrome?

When is inpatient care indicated for Behcet syndrome?

Which medications are used in the treatment of Behcet syndrome?

When should patients be transferred to a tertiary care center for the treatment of Behcet syndrome?

How is Behcet syndrome prevented?

What are the possible complications of Behcet syndrome?

What is the prognosis of Behcet syndrome?

What is included in patient education about Behcet syndrome?