Behcet’s syndrome

And
Polymyalgia rheumatica

Dr.Faiq Isho Gorial

Professor and consultant rheumtologist
Objectives

Behcet’s Syndrome
1. To define Behcet’s syndrome (BS)
2. To know the prevalence and etiology of BS .
3. To know the Diagnostic criteria, and Rx of of BS
Polymyalgia rheumatica (PMR)
1. To define PMR
2. To know the prevalence and etiology of PMR .
3. To know the Diagnostic criteria, and Rx of PMR
Behcet’s syndrome ( Disease)

Definition
• Vasculitis that Involve blood vessels of nearly all sizes and types,
ranging from small arteries to large ones, and involving veins as
well as arteries.
Prevalence

• More common and severe in the eastern Asia and Mediterranean area).

• Most common in Turkey (80 to 370 cases per 100,000) while the
prevalence ranges from 13.5 to 35 per 100,000 in Japan, Korea, China, Iran,
Iraq, and Saudi Arabia

• Male: Female ratio is equal in these areas.

• .Females are affected more commonly in United States and northern

Europe.
• Age: young adults 20 to 40 years of age but can be seen in children
• More severe in young male form Middle- or Far-Eastern Asia

Etiology
• Unknown etiology
• HLA–B51 is a risk factor
• Other: -Infections
-Environmental exposures.
Treatment of BS
• Mucocutaneous regions : topical steroids and colchicine
• Erythema nodosum and arthralgia: Colchicine, MTX .
• Resistant oral and genital ulceration:Thalidomide (teratogenic and
neurotoxic.)
• Systemic disease: Steroids and immunosuppressives.
• Interferon–alpha has demonstrated some promise in Behcet’s.
• Biological Tx( Anti-TNFα)
Polymyalgia rheumatica (PMR)
Definition
• Inflammatory disorder that causes
muscle pain and stiffness, primarily in the neck,
shoulders, upper arms, hips and thighs.

Prevalence
• Symptoms of polymyalgia rheumatica usually begin quickly over a
few days.
• almost exclusively a disease of adults over the age of 50 years.
• Common in ages older than 65 years
• Women are two times more likely to develop the disorder.
Etiology
• The exact cause is unknown.
• Related to and may coexist with giant cell arteritis.
• Pathogenic similarities between both PMR and GCA.
• PMR can precede, accompany, or follow GCA.
Differential diagnoses
• Fibromyalgia
• Hypothyroidisim
• Cervical spondylosis
• Rheumatoid arthritis
• Systemic vasculitis
• Inflammatory myopathy
• Malignancy

Investigations
• High ESR & CRP
• Anemia
Treatment

• Low dose of oral corticosteroid, such as prednisone: 10-20 mg.

for 2-3 years.