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Unformatted text preview: Behcet’s Disease
AM Report Behcet’s: Epidemiology
Behcet’s: Epidemiology Disease prevalence and expression vary geographically and it affects people of Middle Eastern or Far Eastern ancestry more often than those from other regions.
Turkey has the highest prevalence: 80 to 370 cases per 100,000 population. Behcet’s: Epidemiology
Behcet’s: 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 the United States. Behcet’s: Pathophysiology
Behcet’s: Pathophysiology The underlying cause of Behcet's disease is unknown. As with other autoimmune diseases, the disorder may represent aberrant immune activity triggered by exposure to an agent, perhaps infectious, in patients with a genetic predisposition to develop the disease. Behcet’s: Pathophysiology
Behcet’s: Pathophysiology The prevalence of the HLAB51 allele is high among patients with Behçet's disease who are of Middle Eastern or Far Eastern ancestry (up to 81 percent of Asian patients have the allele) but not among white patients who live in Western countries (13 percent). Studies suggest a possible pathogenic role of certain bacterial/viral antigens that have cross
reactivity with human peptides. Behcet’s: Pathophysiology
Behcet’s: Pathophysiology Endothelial activation in affected blood vessels has been proposed as a mediator of vascular inflammation as well as thrombosis in Behcet's.
Hyperfunction of neutrophils noted within active lesions Clinical Manifestations: Oral 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 other manifestations by a number of years. Oral aphthae which are grossly and histologically similar to common oral ulcers, but tend to be more extensive and often multiple Lesions heal within about 10 days without scarring. Clinical Manifestations: Urogenital Clinical Manifestations: Urogenital Lesions Genital ulceration occur in 75 percent or more of patients with Behcet's disease. The ulcers are similar in appearance to the oral aphthae. Genital ulcers are most commonly found on the scrotum in men and the vulva in women Recurrence is typically less frequent than with oral ulcerations. Clinical Manifestations: Cutaneous Clinical Manifestations: Cutaneous Lesions Cutaneous lesions also occur in over 75 percent of patients with Behcet's disease.
May include acneiform lesions, erythema nodosum, pyoderma gangrenosumtype lesions, and palpable purpura. Clinical Manifestations: Cutaneous 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 a needle. Less common in European decent patients with Behcet'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. Clinical Manifestations: Arthritis
Clinical Manifestations: Arthritis Nonerosive, asymmetric, usually nondeforming arthritis occurs in about onehalf of patients with Behcet's disease, particularly during exacerbations. Most commonly affects the medium and large joints, including the knee, ankle, and wrist. Clinical Manifestations: Ocular
Clinical Manifestations: Ocular Ocular disease occurs in 25 to 75 percent of 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 of Behcet's disease. It is typically bilateral and episodic. Clinical Manifestations: Ocular
Clinical Manifestations: Ocular Hypopyon, a visible layer of pus in the anterior ocular chamber, is characteristic of Behçet's disease The most serious ocular problem in patients with Behçet's disease is retinal disease, as a result of vasoocclusive lesions. Clinical Manifestations: 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 Behçet's disease are indistinguishable from those of ulcerative colitis.
It is often difficult to distinguish between Behçet's disease and inflammatory bowel diseases, because of the similarity in extraintestinal symptoms Clinical Manifestations: Clinical Manifestations: Gastrointestinal The other parts of the gastrointestinal system and liver (except in BuddChiari syndrome), pancreas, and spleen are rarely involved.
A large Japanese autopsy registry of subjects with Behcet’s, reported 5 cases of histologic pancreatitis in a total 170 cases. Clinical Manifestations: Neurologic
Clinical Manifestations: Neurologic Neurologic disease occurs in fewer than onefifth of patients with Behcet's disease, more frequently in men than women. Classically, meningitis or meningoencephalitis, neurologic deficits such as motor disturbances and brainstem symptoms, and psychiatric symptoms including personality changes develop more than five years after Behçet's disease is diagnosed. Clinical Manifestations: Neurologic
Clinical Manifestations: Neurologic Symptoms have exacerbations and remissions and gradually cause irreversible disability. In the terminal stage, dementia becomes evident in about 30 percent of affected patients Clinical Manifestations: Vascular
Clinical Manifestations: Vascular Smallvessel vasculitis is common and accounts for much of the pathologic process in Behçet's disease. Large vessel vascular involvement occurs in approximately onethird of patients with Behcet's disease.
Superficial and deep venous thrombosis are common. Clinical Manifestations: Vascular
Clinical Manifestations: Vascular This may lead to stenosis or aneurysm formation. Rupture of such aneurysms may be fatal. Vascular lesions in the lung, including thrombosis, aneurysm, and arteriobronchial fistula, cause recurrent episodes of dyspnea, cough, chest pain, and hemoptysis. Vascular disease in 728 patients with Behcet's disease
Number of patients
Deep 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 Behcet’s Disease: Diagnosis
Behcet’s Disease: Diagnosis There are no pathognomonic symptoms or laboratory findings, the diagnosis is made on the basis of clinical findings
New international criteria were published in 1990 Behcet’s Disease: Treatment
Behcet’s Disease: Treatment The choice of the treatment depends on the patient's clinical manifestations. Significant ocular, neurologic, gastrointestinal, vascular, or other serious end organ manifestations typically require treatment with steroids and other immunosuppressive agents. Behcet’s Disease (Systemic): Behcet’s Disease (Systemic): Treatment High dose steroids and… Azathioprine
Cyclosporine There is no consensus as to which immunosuppressive agent to use with steroids. The choice depends upon the severity of disease, type of organ involvement, and clinician experience with available agents. Behcet’s Disease Behcet’s 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 immunosuppressive agents. Behcet’s Disease (Ocular): Behcet’s Disease (Ocular): Treatment Ocular lesions are treated with topical mydriatics, glucocorticoids.
Relapses and/or progressive disease may occur with steroid treatment alone. Combination therapy, as for systemic disease, is often necessary.
There is evidence of benefit from cyclosporine and azathioprine. No efficacy for cyclophosphamide or colchicine. Behcet’s Disease (Neurologic): Behcet’s Disease (Neurologic): Treatment Aseptic acute meningitis or meningoencephalitis in the early phase of the disease responds well to treatment with corticosteroids. In contrast, chronic progressive central nervous system disease is resistant to all the currently available therapies. Behcet’s Disease (Arterial): Behcet’s Disease (Arterial): Treatment Arterial lesions are generally treated with steroids and another systemic immunosuppressive agent and may require surgical repair. Although surgery is ideally performed when the patient's disease is quiescent, it is often necessary to operate urgently or emergently due to enlarging or ruptured true or pseudoaneurysms or limb or viscerathreatening ischeia Behcet’s Disease (Venous): Behcet’s 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 pulmonaryvessel disease because of the risk of potentially fatal hemoptysis. Behcet’s Disease: Prognosis
Behcet’s Disease: Prognosis Behcet's disease has an undulating course of exacerbations and remissions, and may become less severe after approximately 20 years.
The disease appears to be more severe in young, male, and Middle Eastern or Far Eastern patients. Behcet’s Disease: Prognosis
Behcet’s Disease: Prognosis Mucocutaneous, articular and ocular disease 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. Behcet’s Disease: Prognosis
Behcet’s Disease: Prognosis In one study, 20 percent of patients with chronic neurologic involvement died within seven years.
Half of patients die within three years after the onset of hemoptysis. ...
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This note was uploaded on 01/02/2012 for the course STEP 1 taught by Professor Dr.aslam during the Fall '11 term at Montgomery College.
- Fall '11