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Behcet’s disease
1. Behcet’s DiseaseBehcet’s Disease
Iman GalalIman Galal
Pulmonary Medicine DepartmentPulmonary Medicine Department
Ain Shams UniversityAin Shams University
E-mail: dr.imangalal@gmail.comE-mail: dr.imangalal@gmail.com
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Historical Aspect:Historical Aspect:
In 1937,In 1937, Prof. Hulusi Behçet,Prof. Hulusi Behçet, aa
Turkish dermatologistTurkish dermatologist (1889-1948),(1889-1948),
described a syndrome characterized bydescribed a syndrome characterized by
recurrent oral ulcers, genital ulcers, &recurrent oral ulcers, genital ulcers, &
hypopyon uveitis of unknown cause.hypopyon uveitis of unknown cause.
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Epidemiology:Epidemiology:
Disease prevalence & expression vary geographically & itDisease prevalence & expression vary geographically & it
affects people in the Middle Eastern or Far East moreaffects people in the Middle Eastern or Far East more
often than those from other regions.often than those from other regions.
Turkey has the highest prevalence: 80-370 cases perTurkey has the highest prevalence: 80-370 cases per
100,000 population.100,000 population.
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Epidemiology:Epidemiology:
People of Mediterranean, Middle Eastern & Asia arePeople of Mediterranean, Middle Eastern & Asia are
thought to be at risk of developing Behçet's disease.thought to be at risk of developing Behçet's disease.
In those of Mediterranean & Middle Eastern origin,In those of Mediterranean & Middle Eastern origin,
Behçet's disease is more common in men than in women.Behçet's disease is more common in men than in women.
However, in people of Asia, Behçet's disease is moreHowever, in people of Asia, Behçet's disease is more
common in women.common in women.
The symptoms of Beçhet's disease tend to be more severeThe symptoms of Beçhet's disease tend to be more severe
in men.in men.
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Pathophysiology:Pathophysiology:
The underlying cause of Behçet's disease is unknown.The underlying cause of Behçet's disease is unknown.
As with other autoimmune diseases, the disorder mayAs with other autoimmune diseases, the disorder may
represent aberrant immune activity triggered by exposure torepresent aberrant immune activity triggered by exposure to
an agent, in patients with a genetic predisposition (HLA B51).an agent, in patients with a genetic predisposition (HLA B51).
Some environmental factors that have been suggested asSome environmental factors that have been suggested as
possibly being associated with Behçet's disease include:possibly being associated with Behçet's disease include:
Herpes virus,Herpes virus,
Hepatitis virus,Hepatitis virus,
Bacteria, andBacteria, and
Pollutants e.g., industrial waste or chemicals.Pollutants e.g., industrial waste or chemicals.
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1.1. Oral lesions:Oral lesions:
Oral ulceration is usually an initial symptom & is seen inOral ulceration is usually an initial symptom & is seen in
all patients at some time in the clinical course,all patients at some time in the clinical course,
sometimes precedes other manifestations by years.sometimes precedes other manifestations by years.
Oral aphthae which are grossly & histologically similarOral aphthae which are grossly & histologically similar
to common oral ulcers, but tend to be more extensive &to common oral ulcers, but tend to be more extensive &
often multiple.often multiple.
Lesions heal within about 10 days without scarring.Lesions heal within about 10 days without scarring.
Clinical ManifestationsClinical Manifestations
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2.2. Ocular lesions:Ocular lesions:
Ocular disease occurs in 25-75% of patients withOcular disease occurs in 25-75% of patients with
Behçet's disease & may progress to blindness.Behçet's disease & may progress to blindness.
Symptoms include blurred vision, eye pain, photophobiaSymptoms include blurred vision, eye pain, photophobia
& lacrimation.& lacrimation.
Uveitis is often the dominant feature of Behçet's disease.Uveitis is often the dominant feature of Behçet's disease.
It is typically bilateral & episodic.It is typically bilateral & episodic.
Clinical ManifestationsClinical Manifestations
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2.2. Ocular lesions:Ocular lesions:
Hypopyon; a visible layer of pus in the anterior ocularHypopyon; a visible layer of pus in the anterior ocular
chamber, is characteristic of Behçet's diseasechamber, is characteristic of Behçet's disease
The most serious ocular problem in patients withThe most serious ocular problem in patients with
Behçet's disease is retinal disease, as a result of vaso-Behçet's disease is retinal disease, as a result of vaso-
occlusive lesions.occlusive lesions.
Clinical ManifestationsClinical Manifestations
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3.3. Uro-genital lesions:Uro-genital lesions:
Genital ulceration occur in 75% of patients with Behçet'sGenital ulceration occur in 75% of patients with Behçet's
disease.disease.
The ulcers are similar in appearance to the oral aphthae.The ulcers are similar in appearance to the oral aphthae.
Genital ulcers are most commonly found on the scrotumGenital ulcers are most commonly found on the scrotum
in men & the vulva in women.in men & the vulva in women.
Recurrence is typically less frequent than with oralRecurrence is typically less frequent than with oral
ulcerations.ulcerations.
Clinical ManifestationsClinical Manifestations
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4.4. Cutaneous lesions:Cutaneous lesions:
Cutaneous lesions also occur in over 75% of patientsCutaneous lesions also occur in over 75% of patients
with Behçet's disease.with Behçet's disease.
They include acneiform lesions, erythema nodosum,They include acneiform lesions, erythema nodosum,
pyoderma gangrenosum-type lesions, & palpablepyoderma gangrenosum-type lesions, & palpable
purpura.purpura.
Clinical ManifestationsClinical Manifestations
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4.4. Cutaneous lesions:Cutaneous lesions:
PathergyPathergy refers to an erythematous papular orrefers to an erythematous papular or
pustular response to local skin injury.pustular response to local skin injury.
PathergyPathergy is defined as ais defined as a > 5 mm lesion> 5 mm lesion that appearsthat appears
24-48 hours24-48 hours after skin prick by a needle.after skin prick by a needle.
Pathergy testPathergy test is a non-specific test.is a non-specific test.
Clinical ManifestationsClinical Manifestations
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5.5. Arthritis:Arthritis:
Non-erosive, asymmetric, usually non-deformingNon-erosive, asymmetric, usually non-deforming
arthritis occurs in about one-half of patients witharthritis occurs in about one-half of patients with
Behçet's disease, particularly during exacerbations.Behçet's disease, particularly during exacerbations.
Most commonly affects the medium & large joints,Most commonly affects the medium & large joints,
including the knee, ankle, & wrist.including the knee, ankle, & wrist.
Clinical ManifestationsClinical Manifestations
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6.6. Gastrointestinal:Gastrointestinal:
Symptoms include abdominal pain, diarrhea, melena, &Symptoms include abdominal pain, diarrhea, melena, &
sometimes perforation.sometimes perforation.
Gastrointestinal ulcerations occur most often in theGastrointestinal ulcerations occur most often in the
terminal ileum, cecum, & ascending colon.terminal ileum, cecum, & ascending colon.
Histologically, the intestinal ulcers of Behçet's diseaseHistologically, the intestinal ulcers of Behçet's disease
are indistinguishable from those of ulcerative colitis.are indistinguishable from those of ulcerative colitis.
Clinical ManifestationsClinical Manifestations
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Clinical ManifestationsClinical Manifestations
7.7. Neurological:Neurological:
Occurs in < 20 % of patients being more common in men.Occurs in < 20 % of patients being more common in men.
Classically, meningitis or meningoencephalitis, motorClassically, meningitis or meningoencephalitis, motor
neurologic deficits, loss of bladder control, headache, &neurologic deficits, loss of bladder control, headache, &
psychiatric symptoms including personality changespsychiatric symptoms including personality changes
develop more than five years after the diagnosis ofdevelop more than five years after the diagnosis of
Behçet's disease.Behçet's disease.
In the terminal stage, dementia becomes evident in aboutIn the terminal stage, dementia becomes evident in about
30% of affected patients.30% of affected patients.
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Clinical ManifestationsClinical Manifestations
8.8. Vascular lesions:Vascular lesions:
Vasculitis in BehçetVasculitis in Behçet’’s disease is Immune-complexs disease is Immune-complex
mediated.mediated.
Small-vessel vasculitis is common & accounts for much ofSmall-vessel vasculitis is common & accounts for much of
the pathologic process in Behçet's disease.the pathologic process in Behçet's disease.
Large vessel vascular involvement occurs inLarge vessel vascular involvement occurs in
approximately one-third of patients with Behçet'sapproximately one-third of patients with Behçet's
disease.disease.
Superficial & deep venous thrombosis are common.Superficial & deep venous thrombosis are common.
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Intracardiac ThrombosisIntracardiac Thrombosis
(a)(a)
Before treatmentBefore treatment
(b)(b)
After treatmentAfter treatment
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Clinical ManifestationsClinical Manifestations
8.8. Vascular lesions:Vascular lesions:
Vascular lesions may lead to stenosis or aneurysmVascular lesions may lead to stenosis or aneurysm
formation (may rupture fatal).formation (may rupture fatal).
Vascular lesions in the lung, including thrombosis,Vascular lesions in the lung, including thrombosis,
aneurysm, & arteriobronchial fistula, causing recurrentaneurysm, & arteriobronchial fistula, causing recurrent
episodes of dyspnea, cough, chest pain & hemoptysis.episodes of dyspnea, cough, chest pain & hemoptysis.
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Left Ventricular Pseudo-aneurysmLeft Ventricular Pseudo-aneurysm
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Treatment:Treatment:
The choice of the treatment depends on the patient'sThe choice of the treatment depends on the patient's
clinical manifestations.clinical manifestations.
Significant ocular, neurologic, gastrointestinal, vascular,Significant ocular, neurologic, gastrointestinal, vascular,
or other serious end organ manifestations typicallyor other serious end organ manifestations typically
require treatment with steroids & otherrequire treatment with steroids & other
immunosuppressive agents.immunosuppressive agents.
Secondary thrombosis may not be preventable bySecondary thrombosis may not be preventable by
anticoagulation, instead 80 mg/day of Aspirin is moreanticoagulation, instead 80 mg/day of Aspirin is more
favorable.favorable.
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Treatment:Treatment:
Prednisone alone may not be sufficient to control thePrednisone alone may not be sufficient to control the
vasculitis & the addition of other drugs e.g.,vasculitis & the addition of other drugs e.g.,
immunosuppressive agents is recommended.immunosuppressive agents is recommended.
Types of Intravenous ImmunosuppressantsTypes of Intravenous Immunosuppressants::
Traditional immunosuppressantsTraditional immunosuppressants::
Chlorambucil, methotrexate, cyclosporin, or azathioprin.Chlorambucil, methotrexate, cyclosporin, or azathioprin.
Monoclonal antibodies:Monoclonal antibodies:
Infliximab & interferon-Infliximab & interferon-αα..
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Treatment: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..
The presence of pulmonary arteritis rules out the usage ofThe presence of pulmonary arteritis rules out the usage of
anticoagulation.anticoagulation.
Embolization therapy may be used for both treatment &Embolization therapy may be used for both treatment &
prevention of hemorrhage from pulmonary arteryprevention of hemorrhage from pulmonary artery
aneurysms,aneurysms,
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Prognosis:Prognosis:
Behcet's disease has an undulating course ofBehcet's disease has an undulating course of
exacerbations & remissions, and may become less severeexacerbations & remissions, and may become less severe
after approximately 20 years.after approximately 20 years.
The disease appears to be more severe in young, male, &The disease appears to be more severe in young, male, &
Middle Eastern or Far Eastern patients.Middle Eastern or Far Eastern patients.
Mucocutaneous, articular and ocular manifestations areMucocutaneous, articular and ocular manifestations are
often at their worst in the early years of the disease.often at their worst in the early years of the disease.
Half of patients die within three years after the onset ofHalf of patients die within three years after the onset of
hemoptysis.hemoptysis.
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References:References:
Fishman AP, Elias JA, Fishman JA, Grippi MA, Senior RM & Pack AI. In Fishman’s pulmonary diseases &Fishman AP, Elias JA, Fishman JA, Grippi MA, Senior RM & Pack AI. In Fishman’s pulmonary diseases &
disorders (2008).disorders (2008).
MacCormack M & Phillips T. Behcet’s disease: a clinical review. Wounds 14(8): 275-283, 2002.MacCormack M & Phillips T. Behcet’s disease: a clinical review. Wounds 14(8): 275-283, 2002.
kman-Demir, G.(1999). Clinical patterns of neurological involvement in Behcet's disease: Evaluation of 200kman-Demir, G.(1999). Clinical patterns of neurological involvement in Behcet's disease: Evaluation of 200
patients.patients. BrainBrain, 122(11), 2171-82, 122(11), 2171-82
Al-Otaibi, L.M. (2005). Behçet's Disease:Al-Otaibi, L.M. (2005). Behçet's Disease: A Review. Journal of Dental ResearchA Review. Journal of Dental Research, 84(3), 209-22., 84(3), 209-22.
Alpsoy, E. et al (2002). Interferon Alfa-2a in the Treatment of Behcet Disease: A Randomized Placebo-Alpsoy, E. et al (2002). Interferon Alfa-2a in the Treatment of Behcet Disease: A Randomized Placebo-
Controlled and Double-blind Study.Controlled and Double-blind Study. Arch DermatolArch Dermatol. 2002;138(4):467-471.. 2002;138(4):467-471.
Gül, U., Gonoul, M. (2007). Oral and Genital Pathergy in Behçet's Disease.Gül, U., Gonoul, M. (2007). Oral and Genital Pathergy in Behçet's Disease. DermatologyDermatology, 215(1), 80-81., 215(1), 80-81.
Hatemi, G., Silman, A., Bang, D., Bodaghi, B., Chamberlain, A. M., Gul, A., Houman, M. H., Kotter, I.,Hatemi, G., Silman, A., Bang, D., Bodaghi, B., Chamberlain, A. M., Gul, A., Houman, M. H., Kotter, I.,
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(2008). EULAR recommendations for the management of Behcet's disease: report of a task force of the(2008). EULAR recommendations for the management of Behcet's disease: report of a task force of the
European Standing Committee for International Clinical Studies Including Therapeutics (ESCISIT).European Standing Committee for International Clinical Studies Including Therapeutics (ESCISIT). AnnAnn
Rheum DisRheum Dis 2008 0: ard.2007.0804322008 0: ard.2007.080432
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pp291-311,pp291-311,
Niccoli, L., Nannini, C, Benucci, M., Chindamo, D., Cassara, E., Salvarani, C., Cimino, L, Gini, G., Lenzetti,Niccoli, L., Nannini, C, Benucci, M., Chindamo, D., Cassara, E., Salvarani, C., Cimino, L, Gini, G., Lenzetti,
I., Cantinit, F. (2007). Long-term efficacy of infliximab in refractory posterior uveitis of Behçet's disease: aI., Cantinit, F. (2007). Long-term efficacy of infliximab in refractory posterior uveitis of Behçet's disease: a
24-month follow-up study.24-month follow-up study. RheumatologyRheumatology, 46(7), 1161-1164., 46(7), 1161-1164.
Sakane, T., Takeno, M., Suzuki, N., Inaba, G.(1999). Behcet's disease.Sakane, T., Takeno, M., Suzuki, N., Inaba, G.(1999). Behcet's disease. The New England Journal ofThe New England Journal of
MedicineMedicine, 341(17), 1284-91., 341(17), 1284-91.
Sfikakis, P.P., et al (2007). Anti-TNF therapy in the management of Behçet's disease--review and basis forSfikakis, P.P., et al (2007). Anti-TNF therapy in the management of Behçet's disease--review and basis for
recommendations.recommendations. RheumatologyRheumatology, 46(5), 736-741., 46(5), 736-741.
Takamoto, M. et al (2007). Long-Term Infliximab Treatment for Behçet's Disease.Takamoto, M. et al (2007). Long-Term Infliximab Treatment for Behçet's Disease. Japanese Journal ofJapanese Journal of
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