AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Behçet disease is a systemic disorder characterized by recurrent aphthous ulcers and intraocular inflammation. The clinical triad of uveitis with recurrent oral and genital ulcers bears the name of Hulusi Behçet, a Turkish dermatologist who described 3 patients who had this triad.

The Behçet's Disease Research Committee of the Ministry of Health and Welfare of Japan first proposed formal diagnostic criteria in 1972. This set of criteria, which has been used throughout the world, classifies disease findings into 4 major criteria and 5 minor criteria. When all 4 major criteria are met, the disease is said to be of the complete type, whereas the incomplete type consists of various combinations of major and minor criteria, with added weight given to ocular disease.

In 1990, The International Study Group for Behçet's Disease proposed a separate set of diagnostic criteria for Behçet disease. Based upon these criteria, a diagnosis of Behçet disease requires recurrent oral ulceration and at least 2 additional criteria, including recurrent genital ulcers, ocular lesions, skin lesions, and a positive pathergy test.

Pathophysiology

The cause and the pathogenesis of Behçet disease remain unknown, but the tumor necrosis factor alpha (TNF-alpha) pathway is likely involved in the pathophysiology of Behçet disease. The frequency of the R92Q TNFRSF1A mutation in patients with Behçet disease is significantly higher than that in control subjects (P=0.006 by the Fisher exact test) and is associated with an increased risk of extracranial venous thrombosis.

Frequency

United States

Prevalence of Behçet disease is estimated to be only 1 case per 300,000 per year.

In a university hospital setting, Behçet disease was responsible for 12-20% of cases of uveitis in Japan compared with only 0.2-0.4% in the United States.

International

Behçet disease occurs worldwide, with predominance among Asians, North Africans, and Europeans who live between the latitudes of 30-45°N.

It is one of the main causes of endogenous uveitis in Japan and Turkey, with prevalence rates of 1 case per 10,000 and 8-30 cases per 10,000, respectively.

Race

The prevalence of Behçet disease exhibits a strong geographic variation and has a unique geographic distribution, being most prevalent along the ancient Silk Road linking Rome with China, which was used for centuries as a trade passage.¹

The prevalence of Behçet disease is highest in Turkey, in the countries bordering the Mediterranean, in the Mideast, in the Far East, and in Japan, where it is 1 case per 1000.

Behçet disease occurs less frequently in northern Europe, the United States, and the United Kingdom.

The prevalence of Behçet disease in African Americans is 1 case per 100,000.

Sex

The male-to-female ratio varies with ethnic origin; androtropism is still observed in Arabian countries with a ratio of 2.3:1, whereas female predominance is seen in some European countries and in the United States.¹

Age

• Mean age of onset is in the third decade of life.
• When Behçet disease occurs in young men aged 15-25 years, it takes a more serious form.

CLINICAL

Section 3 of 11  

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• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
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History

• Ocular
• • Ocular involvement is seen in about 70% of patients who have Behçet disease.
  • In most cases, the ocular symptoms follow the oral and genital ulcers by 3-4 years, although ocular disease is the initial manifestation in about 20% of cases.
  • Behçet disease is characterized by severe recurrent attacks of intraocular inflammation. In one series, anterior uveitis was present in 59% of cases; posterior uveitis was present in 76% of cases; and panuveitis was present in 88.1% of cases.
  • Symptoms include periorbital pain, redness, photophobia, and blurred vision.
• Oral aphthous ulcers - Recurrent painful oral aphthous ulcers are the most common lesions associated with Behçet disease and occur in 99.3% of cases.
• Skin - Cutaneous hypersensitivity is relatively common, occurring in 81.8% of cases. Acnelike lesions or folliculitis occur frequently. Migratory thrombophlebitis also can develop.
• Genital ulcers - Recurrent painful genital ulcers occur in 62.8% of cases.

Physical

Initial ocular involvement may be unilateral but progresses to bilateral disease in at least two thirds of cases.

In a large retrospective study, the mean age at onset of uveitis was 28.5 years in male patients and 30 years in female patients. Ocular involvement was bilateral in 78.1% of patients and unilateral in 21.9% of patients. Panuveitis was the most common form in both sexes. Fundus lesions as well as sight-threatening complications were more common in male patients than in female patients. At the beginning of follow-up, potential visual acuity was 0.1 or less in 30.9% of eyes in male patients and 24.2% of eyes in female patients. The Kaplan-Meier survival analysis estimated the risks of losing useful vision (>0.1) at 5 and 10 years for male patients and female patients as 21% versus 10% and 30% versus 17%, respectively.

• Ocular
• • Described in 75% of patients, either anterior segment iridocyclitis or posterior segment involvement. Panuveitis and posterior uveitis/retinitis occurred more frequently in males than in females (28.9% vs 11.5% and 57.9% vs 36.1%, respectively; p <0.05).
  • The classic finding of this disease, iridocyclitis with hypopyon, is present in about one third of cases.
  • • Gonioscopy may reveal an occult hypopyon in many cases. One characteristic feature of the hypopyon in Behçet disease is that it may change position with head movement, and it may form and disappear rapidly without sequelae.
    • Recurrent attacks may result in posterior synechiae, peripheral anterior synechiae, iris atrophy, and secondary glaucoma.
  • Retinal disease is the most serious complication of Behçet disease. The classic fundus finding is retinal vasculitis, which affects both arteries and veins in the posterior pole.
  • Ophthalmoscopy shows venous engorgement, retinal hemorrhages, yellow-white exudates deep in the retina, white focal retinal infiltrates, retinal edema, and optic disc edema with hyperemia.
  • Severe vasculitis may lead to thrombosis of vessels and secondary ischemic retinal changes. Optic disc edema may be secondary to inflammation and may be seen during the acute phase in at least one fourth of cases.
  • Vitreous cellular infiltration almost always is present during the acute phase.
  • Retinal neovascularization, secondary to either retinal vein occlusion or chronic inflammation, may result in retinal or vitreous hemorrhage.
  • Neovascular glaucoma occurs in as many as 6% of patients and often results in phthisis bulbi.
  • Repeated episodes of posterior segment inflammation cause sheathing of retinal vessels, chorioretinal scars, and retinal and optic nerve atrophy.
• Oral aphthous ulcers
• • Recurrent aphthous ulceration of the oral mucosa is the most common nonocular manifestation of Behçet disease.
  • • They are the first symptoms in 50-70% of patients and develop in as many as 98% of patients.
    • The lesions are located on the gingiva, lips, tongue, buccal mucosa, hard palate, uvula, and posterior pharynx.
  • A nationwide study in Japan revealed that 97.7% of patients who have Behçet disease have recurrent aphthous ulcers. Ulcers tend to develop in crops, which recur at various frequencies.
  • White or yellowish pseudomembrane usually covers the surface of the ulcer.
  • They usually heal within 7-10 days with no scarring.
  • However, fusion of several small ulcers may produce a large ulcer that leads to scar formation.
• Skin involvement
• • Skin involvement appears in 70% of patients. Behçet disease may produce a variety of skin lesions in affected individuals.
  • Erythema nodosum, acneiform lesions, thrombophlebitis, and cutaneous hypersensitivity are most common.
  • • Erythema nodosum lesions, which occur in more than two thirds of patients with Behçet disease, usually are found on the anterior surface of the legs but also may be seen on the face, arms, and buttocks. These lesions appear as slightly raised, red nodules with subcutaneous induration and tenderness. They tend to involute in 10-14 days without ulceration.
    • Acneiform lesions occur in almost 60% of patients and may occur in patients who receive corticosteroid treatment; therefore, their presence is of questionable diagnostic usefulness.
    • A peculiar feature of Behçet disease is cutaneous hypersensitivity, which results in small pustules that form on skin after it has been scratched, shaved, or pricked with a needle.
• Genital ulcers
• • Genital ulcers occur in 80% of patients. They are painful punched-out lesions similar to those that occur in mouth and usually are located in the scrotum or vulva.
  • These genital ulcers may be deep and, in many cases, result in scarring. These scars are indicators of old disease and may help in diagnosis.
• Musculoskeletal
• • Painful swelling with redness of joints occurs in as many as 50% of patients.
  • Transient, recurrent, nondestructive, and nonmigratory arthritis commonly affects large joints such as the knee, ankle, elbow, and wrist. The knee joint is most commonly affected.
• Gastrointestinal
• • At least 50% of patients who have Behçet disease develop gastrointestinal symptoms.
  • Intestinal erosions and ulcers may cause abdominal pain, melena, and perforation.
  • This constellation of intestinal signs and symptoms may simulate Crohn disease and other inflammatory bowel disorders.
• Vascular
• • Thrombophlebitis is found in 15% of these patients.
  • Obliterating thrombophlebitis, arterial occlusion, and aneurysm may occur in vessels of all sizes.
• Neurologic
• • Multiple neurologic disorders that involve pyramidal and extrapyramidal tracts, the cerebellum, the cranial nerves, and rarely the peripheral nerves occur more commonly in male patients and in 5-30% of cases.
  • Both the central nervous system and the peripheral nervous system can be involved. Central nervous system manifestations can be divided into 2 main groups: (1) parenchymal involvement, which includes brainstem involvement, hemispheric manifestations, spinal cord lesions, and meningoencephalitic presentations; and (2) nonparenchymal involvement, including dural sinus thrombosis, arterial occlusion, and/or aneurysms. Peripheral neuropathy and myopathy are relatively rare.

Causes

• The etiology and the pathogenesis of Behçet disease are not clear but are presumed to be multifactorial, involving genetic, infectious, and immunological factors. There is increasing evidence that antigens derived from infectious agents (eg, Streptococcus sanguis, herpes simplex virus, heat shock proteins) are implicated in the pathogenesis of the disease, and it has also become increasingly apparent that these events, once triggered, may be influenced by numerous interdependent and independent genetic regions.
• Despite 20 years of intense efforts to identify other associated genetic regions in chromosome 6 and elsewhere, human leukocyte antigen B51 (HLA-B51) remains foremost among candidate risk factors for the disease. MICA alleles in the major histocompatibility complex (MHC) have also been implicated, but their close linkage with HLA-B51 has made their independent contribution to the disease less easy to define.¹
• Since the disease is characterized by a vasculitis, the underlying mechanism is believed to be an autoimmune process.
• Although familial occurrences have been reported, no consistent inheritance pattern has been established; however, human leukocyte antigen B5 (HLA-B5) is associated with Behçet disease and poor visual prognosis.
• • Considerable evidence now points toward a more specific association with HLA-B51, a split antigen of HLA-B5.
  • The frequency of HLA-B51 is 57% in Japanese patients who have Behçet disease compared to only 14% in the control group, which equates to a relative risk of 7.9 for this marker.
  • Restriction fragment link polymorphism studies showed linkage disequilibrium between the HLA-B51 locus and the tumor necrosis factor beta (TNF-beta) gene. This finding may imply that lower levels of TNF-beta may contribute to activation of inflammatory cascade in Behçet disease.
  • In Japan, an association has been found between human leukocyte antigen A26 (HLA-A26) and Behçet disease.
• The disease occurs more frequently in temperate northern parts of Japan than in subtropical southern parts, a distribution that suggests that environmental factor influences the prevalence of disease.
• With regard to the immunology of Behçet disease, several data suggest a direct role of Th1 lymphocytes in the pathogenesis of the disease lesions. Th1 cytokines, including interleukin 2 (IL-2), TNF-alpha, interferon gamma (IFN-gamma), interleukin 12 (IL-12), and interleukin 18 (IL-18), are elevated and probably contribute to neutrophil and endothelial cell activation.¹
• Other diagnostic considerations
• • It is important to consider other forms of uveitis in the differential diagnosis, especially in those patients who have a mild or atypical presentation of Behçet disease.
  • Human leukocyte antigen B27 (HLA-B27)-related anterior uveitis also may give recurrent iridocyclitis with hypopyon, but it is typically unilateral.
  • Since Behçet disease is a bilateral panuveitis, other inflammatory processes that affect both eyes must be considered. Syphilis causes a retinitis with vitreitis rather than a strict vasculitis. The diagnosis for syphilis is confirmed by serology.
  • Sarcoidosis, another bilateral inflammatory process, may have posterior pole findings similar to those in Behçet disease but is generally more indolent, in contrast to the explosive recurrent attacks of Behçet disease. Furthermore, the vasculitis seen in sarcoidosis usually is not occlusive in nature and typically involves only veins compared with the involvement of both arteries and veins in Behçet disease.
  • Other conditions that may mimic the ocular changes of Behçet disease include collagen vascular diseases and viral retinitis.

DIFFERENTIALS

Section 4 of 11  

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• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Collagen vascular diseases
Viral retinitis

WORKUP

Section 5 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• Diagnosis of Behçet disease is based on clinical findings rather than on specific laboratory test results. Some tests are useful adjuncts in the evaluation of patients who have Behçet disease.
• The pathergy test (or Behçetine test) may be useful in patient evaluation; the test result is a nonspecific inflammatory reaction to a needle prick or an intradermal injection of saline. The reaction, which varies from an indurated erythema to pustule formation, occurs in 40-60% of patients who have Behçet disease.
• Several other laboratory tests may help in the evaluation of a patient who has Behçet disease. During episodes of acute inflammation, patients may have high erythrocyte sedimentation rates, high C-reactive protein levels, or increased number of peripheral leukocytes.
• In neuro-Behçet disease, an analysis of cerebrospinal fluid reveals pleocytosis and elevated protein levels.

Imaging Studies

• Fundus fluorescein angiography shows diffuse retinal vascular leakage and occlusion of retinal vessels.
• • Fluorescein leakage from retinal vessels may be seen before any clinical signs of vasculitis.
  • During acute inflammation, retinal vascular leakage is prominent, especially in the radial peripapillary area.
  • Affected retinal and optic nerve vessels leak fluorescein profusely during early transit and their walls stain in late transit.
  • Fluorescein angiography also may reveal macular ischemia and cystoid macular edema.
• In neuro-Behçet disease, magnetic resonance imaging (MRI) is the imaging study of choice and often reveals iso-hypointense lesions in T1-weighted images and hyperintense lesions in T2-weighted images, mostly in the mesodiencephalic junction, cerebellar peduncles, and other parts of the brainstem.

Histologic Findings

Histopathologic changes include necrotizing, leukocytoclastic, and obliterative vasculitis, which affect arteries and veins of all sizes and are probably immune-complex mediated. Only a few eyes that have had active disease have been examined histologically. Vasculitis is the key feature of Behçet disease. Underlying changes seen in the eye are similar to those that occur in other organs of the body.

During acute inflammation, the iris, the ciliary body, and the choroid show diffuse infiltration with neutrophils. In late stages, there is proliferation of collagen fibers, thickening of the choroid, formation of cyclitic membrane, and sometimes hypotonia and phthisis bulbi. Lymphocytic and plasma cell infiltration occurs during remission. Of all ocular tissues, the retina suffers the most damage. In the phase of acute inflammation, there is severe vasculitis with marked infiltration of leukocytes in and around blood vessels. Recent and old hemorrhages are present. Retinal vessels have thickened basement membranes with swollen endothelial cells, which can lead to thrombus formation and vascular obliteration.

TREATMENT

Section 6 of 11  

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• Follow-up
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Medical Care

• Treatment of the various manifestations of Behçet disease remains controversial because of the paucity of randomized, controlled trials and the absence of standardized outcome measures for this disease. For example, colchicine, which has historically been the drug of choice for the treatment of the various manifestations of Behçet disease, has been evaluated for its efficacy and safety in only one randomized, controlled trial. In this 2-year trial, colchicine was found to only decrease arthritis in male and female patients and to decrease genital ulcers and erythema nodosum in female patients.²
• The goals of therapy in Behçet disease are to suppress inflammation, to reduce the frequency and severity of recurrences, and to minimize involvement of the retina.
• To be effective, treatment must be started early. Extent of involvement and severity of disease determine the choice of medication. Treatment options include corticosteroids, cytotoxic agents, cyclosporine, and colchicine.
• Corticosteroids
• • Systemic corticosteroids effectively suppress all phases of ocular involvement in Behçet disease.
  • Although these drugs do not prevent visual deterioration, systemic corticosteroid therapy may still be helpful, especially when used in concurrence with other immunosuppressive agents.
  • Prednisone may be used in a pulse mode to treat Behçet disease, with a 1-g bolus given intravenously over 1 hour and repeated once a day for 3 days. The pulse treatment is repeated as needed.
  • In Japan, systemic corticosteroids are not used for ocular disease. This decision is based upon the findings of several retrospective studies that show long-term visual outcomes to be worse in patients who receive systemic corticosteroids compared to those who did not.
• Cytotoxic agents
• • Chlorambucil, cyclophosphamide, and azathioprine are the cytotoxic agents used most commonly for the treatment of Behçet disease.
  • Chlorambucil was the first cytotoxic drug to be used in the treatment of ocular Behçet disease. This slow-acting alkylating agent may be administered on an outpatient basis.
  • Cyclophosphamide is an alkylating agent that is superior to corticosteroids in the control of inflammation in Behçet disease, but profound bone marrow toxicity limits its use. Since cyclophosphamide acts faster and is more toxic than chlorambucil, its use is reserved for very refractory cases.
  • Azathioprine is a mercaptopurine derivative that is effective in the treatment of Behçet disease.
  • All of these cytotoxic agents may produce variable degrees of bone marrow suppression and may affect reproductive organs resulting in azoospermia and amenorrhea.
• Cyclosporine
• • Cyclosporine inhibits T-lymphocyte activation and consequently is safer than cytotoxic agents; however, renal complications may occur.
  • Usual starting dose is 5 mg/kg per day. Cyclosporine does not appear to induce permanent immunosuppression; therefore, patients need continuous treatment for many years.
  • A rebound phenomenon has been noted after withdrawal of cyclosporine therapy.
  • These factors have limited the use of cyclosporine for the treatment of Behçet disease, but a study conducted in Japan found that a starting dose of 5 mg/kg per day effectively limited the frequency of ocular inflammatory attacks in 70% of patients with Behçet disease who previously had refractory disease.
• Colchicine
• • Colchicine is a plant alkaloid that interferes with microtubule function, which results in dysfunction of neutrophils.
  • Colchicine may be used with other drugs and may enable the disease to be controlled using lower doses of immunosuppressants. The optimum dosage of colchicine is 0.5-1 mg/d.
  • In Japan, colchicine is considered the drug of choice because of its few adverse effects and because of the presumed poor long-term prognosis of systemic corticosteroids.
  • When colchicine fails to limit recurrences, treatment is switched to or combined with cyclosporine.
• It appears that the best results may be obtained using a combination of drugs, such as corticosteroids with either cytotoxic agents or cyclosporine.
• Overall, no safe and conclusive treatment exists for patients with Behçet disease. The treatment regimen needs to be tailored to the severity and extent of disease in each individual case.
• The goal of therapy is to suppress inflammation, to prevent sequelae, and to minimize the systemic adverse effects of treatment.
• Newer therapies have been advocated; see Media file 1 for a complete review.¹
• Biological therapies
• • Interferons
  • • Interferons (INFs) are a large family of glycoproteins that possess antiviral, antitumor, and immunomodulatory properties. The rationale for their use in Behçet disease is founded, firstly, on the putative association between the disease and viral infections and, secondly, on their biologic effects, including the ability to improve the activity of natural killer cells and to inhibit gamma delta T cells.¹
    • The most impressive results have been achieved for severe and/or refractory ocular manifestations. Patients with mucocutaneous and articular manifestations also benefit from interferon alpha (IFN-alpha), but there are fewer complete remissions, and relapses often occur after discontinuation. Intermediate-to-high IFN-alpha doses are more effective than low-dose regimens, and long-term remissions are associated with higher IFN-alpha doses but not with longer treatment duration. Adverse effects are frequent but dose dependent and not severe. They are the same as those reported for IFN-alpha in the treatment of other disorders, such as chronic hepatitis B or C, or hematological diseases, such as chronic myelogenous leukemia or non-Hodgkin lymphoma.¹
  • TNF-alpha inhibitors
  • • The rationale for anti-TNF use in Behçet disease is founded on the strong implication of TNF-alpha in the pathogenesis of this disease. It is well known that Behçet disease is mediated by a variety of cytokines, including TNF-alpha released from Th1 lymphocytes. Patients with active disease demonstrate increased numbers of monocytes and T lymphocytes expressing gamma/delta receptors that overproduce TNF-alpha. Patients also show increased levels of circulating TNF-alpha and soluble receptors, and high TNF-alpha levels are observed in the aqueous humor from patients with uveitis associated with Behçet disease.¹
    • Both infliximab (a chimeric monoclonal antibody to TNF-alpha) and etanercept (a dimeric, soluble p75 TNF receptor) have been shown to be effective in the treatment of various inflammatory disorders.
    • Results of therapeutic studies suggest that this drug is highly effective in inducing short-term remission of virtually all manifestations of the disease, including sight-threatening panuveitis. In cases of ocular inflammation, remission was observed in some patients within 24 hours, and marked improvement of visual acuity was observed in other patients by 7 days. Infliximab was usually administered in doses of 3–5 mg/kg with continuous treatment every 2 months for as long as 2 years in some patients. Complete remission has been maintained with a reduction in concomitant immunosuppressive therapy. Continuous therapy has been reported in most studies, but a few patients have remained in remission for up to 12 months after treatment. Data that continue to accumulate strongly suggest that infliximab and etanercept are effective and safe in the treatment of Behçet disease. Whether infliximab is more effective than etanercept in the treatment of Behçet disease remains unclear.
  • Both IFN and TNF blockers seem to be effective in the management of ocular and extraocular manifestations of Behçet disease. However, the data need to be further verified by randomized, controlled trials in larger numbers of patients with longer follow-up periods.¹
• Other alternative therapeutic strategies
• • Tolerizing agents
  • • Tolerance induction has primarily been used for the treatment of autoimmune uveitis, as it is known to be caused by lymphocytes that recognize and attack self-protein antigens within the eye. S-antigen (S-Ag) and interphotoreceptor retinoid binding protein (IRBP) are the best characterized self-antigens. Conventional therapeutic approaches suppress the activity of the leukocytes (anti-inflammatory) and lymphocytes (immunosuppressive) in an unspecific manner, leading to suppression of the entire immune system. New immunoregulatory approaches that suppress only the aberrant immune response to ocular antigens are under development. One of these approaches is mucosal tolerance, a mechanism of specific immune tolerance to soluble antigens applied via mucosal surfaces.¹
    • To date, various oral antigens have been applied to patients with uveitis. They have included IRBP, retinal S-Ag, retinal extracts, and a peptide from the sequence of associated HLA-B-antigens mimicking retinal S-Ag peptide.¹
    • Oral tolerance seems to be safe and effective in Behçet disease. Yet, clinical trials have been performed on a limited number of patients; therefore, these results have to be interpreted cautiously.¹
  • Immunoablation: Several case series have demonstrated the efficacy of autologous hematopoietic stem cell transplantation (HSCT) in inducing remission in patients with Behçet disease.¹

Consultations

• Uveitis service if available
• Gastroenterology
• Rheumatology
• Neurology: Neuro-Behçet disease must be considered in the differential diagnosis of stroke in young adults, multiple sclerosis, movement disorders, intracranial hypertension, intracranial sinovenous occlusive diseases, and other neurologic syndromes.

MEDICATION

Section 7 of 11  

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Treatment is aimed toward individual symptoms as they occur. Medication is given to reduce the inflammatory response.

Drug Category: Anti-inflammatory agents

Systemically interfere with events leading to inflammation.

Drug Category: Corticosteroids

Systemic corticosteroids are not useful in the long-term management of Behçet disease. Some authors use corticosteroids as initial therapy especially in mild cases. However, other authorities state that there is no role for corticosteroids in the treatment of this disease. Systemic corticosteroids should be used only for short periods of time and probably in combination with immunosuppressive therapy. Topical or sub-Tenon corticosteroids have proven effective.

Drug Category: Immunosuppressive agents

Used to treat acute attacks and reduce frequency of recurrences.

FOLLOW-UP

Section 8 of 11  

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• Follow-up
• Miscellaneous
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Further Outpatient Care

• Patients should receive follow-up care as needed.

Prognosis

• Spontaneous remissions and relapses characterize the natural history of ocular Behçet disease.
• Visual prognosis in countries (ie, Japan, Turkey) that have the highest prevalence of disease is poor.
• • In Japan and Turkey, more than 50% of cases result in legal blindness within 4 years of onset.
  • The prevalence of blindness in North America is 25%.
  • Possible explanation for this discrepancy includes genetic heterogeneity, with the occurrence of more severe disease in the Mideast and the Far East.

MISCELLANEOUS

Section 9 of 11  

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• Medication
• Follow-up
• Miscellaneous
• Multimedia
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Medical/Legal Pitfalls

• Early diagnosis leads to more prompt treatment and fewer sequelae.

MULTIMEDIA

Section 10 of 11  

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• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Treatment modalities currently used in Behçet disease according to clinical symptoms.
	View Full Size Image
Media type:  Chart

REFERENCES

Section 11 of 11

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• Differentials
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• Follow-up
• Miscellaneous
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• References

1. Houman MH, Hamzaoui K. Promising new therapies for Behcet's disease. Eur J Intern Med. May 2006;17(3):163-9. [Medline].
2. Yurdakul S, Mat C, Tuzun Y, Ozyazgan Y, Hamuryudan V, Uysal O, et al. A double-blind trial of colchicine in Behcet's syndrome. Arthritis Rheum. 2001;44(11):2686-92. [Medline].
3. Baldassano VF Jr. Ocular manifestations of rheumatic diseases. Curr Opin Ophthalmol. Dec 1998;9(6):85-8. [Medline].
4. Borhani Haghighi A, Pourmand R, Nikseresht AR. Neuro-Behcet Disease. A Review. Neurologist. Mar 2005;11(2):80-89. [Medline].
5. Borruat FX. Neuro-ophthalmologic manifestations of rheumatologic and associated disorders. Curr Opin Ophthalmol. Dec 1996;7(6):10-8. [Medline].
6. Bredvik BK, Trocme SD. Ocular manifestations of immunological and rheumatological inflammatory disorders. Curr Opin Ophthalmol. Dec 1996;7(6):91-5. [Medline].
7. Dinowitz K, Aldave AJ, Lisse JR, Trocme SD. Ocular manifestations of immunologic and rheumatologic inflammatory disorders. Curr Opin Ophthalmol. Dec 1994;5(6):91-8. [Medline].
8. Eldem B, Onur C, Ozen S. Clinical features of pediatric Behcet's disease. J Pediatr Ophthalmol Strabismus. May-Jun 1998;35(3):159-61. [Medline].
9. George RK, Chan CC, Whitcup SM, Nussenblatt RB. Ocular immunopathology of Behcet's disease. Surv Ophthalmol. Sep-Oct 1997;42(2):157-62. [Medline].
10. Pipitone N, Boiardi L, Olivieri I, Cantini F, Salvi F, Malatesta R, et al. Clinical manifestations of Behcet's disease in 137 Italian patients: results of a multicenter study. Clin Exp Rheumatol. 2004;22(6 Suppl 36):S46-51. [Medline].
11. Sakane T, Takeno M, Suzuki N, Inaba G. Behcet's disease. N Engl J Med. Oct 21 1999;341(17):1284-91. [Medline].
12. Torres RM, Yanez B, Herreras JM, Calonge M. Ocular Behcet disease. Retrospective study. Arch Soc Esp Oftalmol. Dec 2004;79(12):599-603. [Medline].
13. Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, Huseyin Altunbas H, Urgancioglu M. Uveitis in Behcet disease: an analysis of 880 patients. Am J Ophthalmol. Sep 2004;138(3):373-80. [Medline].
14. Yazici H, Barnes CG. Practical treatment recommendations for pharmacotherapy of Behcet's syndrome. Drugs. Nov 1991;42(5):796-804. [Medline].

Article Last Updated: Oct 12, 2007