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Behcets Disease

Andrea Honeycutt

AM Report

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Behcets: Epidemiology

Disease prevalence and expression varygeographically and it affects people of

Middle Eastern or Far Eastern ancestrymore often than those from other regions.

Turkey has the highest prevalence: 80 to

370 cases per 100,000 population.

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Behcets: Epidemiology

Prevalence in Japan, Korea, China, Iran,and Saudi Arabia ranges from 13.5 to 20

cases per 100,000 The prevalence is much lower in Western

countries.

Prevalence of 0.33 per 100,000 in theUnited States.

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Behcets: Pathophysiology

The underlying cause of Behcet's diseaseis unknown.

As with other autoimmune diseases, thedisorder may represent aberrant immuneactivity triggered by exposure to an agent,

perhaps infectious, in patients with agenetic predisposition to develop thedisease.

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The prevalence of the HLA-B51 allele is highamong patients with Behet's disease who are of

Middle Eastern or Far Eastern ancestry (up to 81percent of Asian patients have the allele) but notamong white patients who live in Westerncountries (13 percent).

Studies suggest a possible pathogenic role ofcertain bacterial/viral antigens that have cross-reactivity with human peptides.

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Endothelial activation in affected bloodvessels has been proposed as a mediator

of vascular inflammation as well asthrombosis in Behcet's.

Hyperfunction of neutrophils noted within

active lesions

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Clinical Manifestations: Oral

Lesions Oral ulceration is usually an initial symptom and

is seen in all patients at some time in the clinical

course, sometimes precedes othermanifestations by a number of years.

Oral aphthae which are grossly and histologicallysimilar to common oral ulcers, but tend to be

more extensive and often multiple

Lesions heal within about 10 days withoutscarring.

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Clinical Manifestations: Uro-genital

Lesions Genital ulceration occur in 75 percent or more of

patients with Behcet's disease.

The ulcers are similar in appearance to the oralaphthae.

Genital ulcers are most commonly found on thescrotum in men and the vulva in women

Recurrence is typically less frequent than withoral ulcerations.

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Clinical Manifestations: Cutaneous

Lesions Cutaneous lesions also occur in over 75

percent of patients with Behcet's disease.

May include acneiform lesions, erythemanodosum, pyoderma gangrenosum-typelesions, and palpable purpura.

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Clinical Manifestations: Cutaneous

Lesions Pathergy refers to an erythematous papular or

pustular response to local skin injury. Defined as a greater than 5 mm lesion that

appears 24 to 48 hours after skin prick by aneedle.

Less common in European decent patients withBehcet's disease (10 to 20 percent), than in

Eastern patients (50 to 75 percent). The pathergy test can also be positive in Sweet's

syndrome and pyoderma gangrenosum.

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Clinical Manifestations: Arthritis

Nonerosive, asymmetric, usuallynondeforming arthritis occurs in about

one-half of patients with Behcet's disease,particularly during exacerbations.

Most commonly affects the medium and

large joints, including the knee, ankle, andwrist.

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Clinical Manifestations: Ocular

Ocular disease occurs in 25 to 75 percentof patients with Behcet's disease, and may

progress to blindness. Symptoms, including blurred vision, eye

pain, photophobia, lacrimation, floaters

Uveitis is often the dominant feature ofBehcet's disease. It is typically bilateraland episodic.

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Clinical Manifestations: Ocular

Hypopyon, a visible layer of pus in theanterior ocular chamber, is characteristic

of Behet's disease The most serious ocular problem in

patients with Behet's disease is retinal

disease, as a result of vaso-occlusivelesions.

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Clinical Manifestations:

Gastrointestinal Symptoms include abdominal pain, diarrhea,

melena, and sometimes perforation. Gastrointestinal ulcerations occur most often in

the terminal ileum, cecum, and ascending colon. Histologically, the intestinal ulcers of Behet's

disease are indistinguishable from those ofulcerative colitis.

It is often difficult to distinguish betweenBehet's disease and inflammatory boweldiseases, because of the similarity inextraintestinal symptoms

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Clinical Manifestations:

Gastrointestinal The other parts of the gastrointestinal

system and liver (except in Budd-Chiari

syndrome), pancreas, and spleen arerarely involved.

A large Japanese autopsy registry of

subjects with Behcets, reported 5 cases ofhistologic pancreatitis in a total 170 cases.

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Clinical Manifestations: Neurologic

Neurologic disease occurs in fewer than one-fifthof patients with Behcet's disease, more

frequently in men than women. Classically, meningitis or meningoencephalitis,

neurologic deficits such as motor disturbancesand brain-stem symptoms, and psychiatric

symptoms including personality changes developmore than five years after Behet's disease isdiagnosed.

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Clinical Manifestations: Neurologic

Symptoms have exacerbations andremissions and gradually cause irreversible

disability. In the terminal stage, dementia becomes

evident in about 30 percent of affected

patients

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Clinical Manifestations: Vascular

Small-vessel vasculitis is common andaccounts for much of the pathologic

process in Behet's disease. Large vessel vascular involvement occurs

in approximately one-third of patients withBehcet's disease.

Superficial and deep venous thrombosisare common.

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Clinical Manifestations: Vascular

This may lead to stenosis or aneurysmformation. Rupture of such aneurysms

may be fatal.Vascular lesions in the lung, including

thrombosis, aneurysm, and

arteriobronchial fistula, cause recurrentepisodes of dyspnea, cough, chest pain,and hemoptysis.

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Vascular disease in 728 patients with Behcet's disease

Number of patients

Venous diseaseDeep venous thrombosis 221

Subcutaneous thrombophlebitis 205

SVC occlusion 122

IVC occlusion 93

Cerebral sinus thrombosis 30

Budd-Chiari syndrome 17

Other venous occlusion* 24

Arterial disease

Pulmonary artery occlusion or aneurysm 36

Aortic aneurysm 17

Extremity arterial occlusion or aneurysm 45

Other arterial occlusion or aneurysm 42

Right ventricular thrombus 2

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Behcets Disease: Diagnosis

There are no pathognomonic symptoms orlaboratory findings, the diagnosis is made

on the basis of clinical findings New international criteria were published

in 1990

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Behcets Disease: Treatment

The choice of the treatment depends onthe patient's clinical manifestations.

Significant ocular, neurologic,gastrointestinal, vascular, or other seriousend organ manifestations typically require

treatment with steroids and otherimmunosuppressive agents.

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Behcets Disease (Systemic):

Treatment High dose steroids and

Azathioprine

Methotrexate

Cyclophosphamide

Cyclosporine

There is no consensus as to which

immunosuppressive agent to use with steroids.The choice depends upon the severity ofdisease, type of organ involvement, and clinicianexperience with available agents.

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Behcets Disease (Mucocutaneous):

Treatment Treated with topical anesthetics, high

potency glucocorticoids, intralesional

steroids, colchicine. Ulcers that are refractory to these

treatments, require systemic steroids,

thalidomide, or the immunosuppressiveagents.

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Behcets Disease (Ocular):

Treatment Ocular lesions are treated with topical

mydriatics, glucocorticoids.

Relapses and/or progressive disease may occurwith steroid treatment alone.

Combination therapy, as for systemic disease, isoften necessary.

There is evidence of benefit from cyclosporineand azathioprine. No efficacy forcyclophosphamide or colchicine.

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Behcets Disease (Neurologic):

TreatmentAseptic acute meningitis or

meningoencephalitis in the early phase of

the disease responds well to treatmentwith corticosteroids.

In contrast, chronic progressive central

nervous system disease is resistant to allthe currently available therapies.

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Behcets Disease (Arterial):

Treatment Arterial lesions are generally treated with

steroids and another systemicimmunosuppressive agent and may require

surgical repair. Although surgery is ideally performed when the

patient's disease is quiescent, it is oftennecessary to operate urgently or emergently dueto enlarging or ruptured true orpseudoaneurysms or limb or viscera-threateningischeia

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Behcets Disease (Venous):

Treatment Low dose aspirin is probably reasonable in

patients with Behcet's associated superficial

thrombophlebitis. Systemic anticoagulation is often used in

patients with deep venous thrombosis.

Anticoagulant drugs should be given carefully in

patients with pulmonary-vessel disease becauseof the risk of potentially fatal hemoptysis.

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Behcets Disease: Prognosis

Behcet's disease has an undulating courseof exacerbations and remissions, and may

become less severe after approximately 20years.

The disease appears to be more severe in

young, male, and Middle Eastern or FarEastern patients.

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Mucocutaneous, articular and oculardisease are often at their worst in the

early years of disease. Central nervous system and large vessel

disease, if they develop, typically do so

later in the disease course.

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In one study, 20 percent of patients withchronic neurologic involvement died within

seven years. Half of patients die within three years

after the onset of hemoptysis.

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