BioPharma The Spondyloarthropathies Kathryn Dao, MD Arthritis Center September 15, 2005.
Seronegative Spondyloarthropthies. Definition -Spondyloarthropathies (SA) are cluster of...
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Seronegative Spondyloarthropthies
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Definition
-Spondyloarthropathies (SA) are cluster of interrelated and overlapping chronic inflammatory rheumatic disease.
-The primary pathologic sites are
-Enthesis
-Axial skeleton including the sacroiliac joints
-Limb joints
-Nonarticular structures: gut,skin,eye,aortic valve
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Etiology
SA occur in genetically predisposed persons and are triggered by enviromental factors.
SA are not associated with rheumatoid factor
There is strong association with HLA-B27
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Association of SA with HLA-B27
Disease HLA-B27 (%)
Ankylosing Spondylitis 90
Reactive Arthritis 40-80
Psoriatic Arthropathy 40-50
Enteropathic Arthritis 35-75
Undifferentiated SA 70
Healthy population (white) 8
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Classification Criteria for SAI. Inflammatory back pain or asymmetrical arhritis with lower
limb predominance
II. One or more of the following criteria:
-Positive family history: AS, reactive arthritis, psoriasis, IBD, uveitis
-Psoroasis
-IBD
-Uerthritis, cervicitis, diarrhea month before onset
-Buttock pains
-Enthesitis
-Sacroileitis
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Ankylosing Spondylitis (AS)
AS is a chronic inflammatory disease of unknown etiology.
Affects mainly the axial skeleton (spine & sacroiliac joints).
Strong association with HLA-B27
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Epidemiology
Annual prevalence(USA white): 6.6/105
HLA-B27 +: 1-2%
+ 10 degree affected relative: 10-20%
M>F X2-3
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Clinical Presentation
1. Mild constitutional symptoms: anorexia, weight loss, fever 2. Inflammatory back pain- 75%
-Insidious onset
-3 months
-Morning stifness
-Worsening with inactivity
-Improvement with physical exercise, hot tub
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Clinical Presentation
3. Involvement of hip and shoulder joints- 33%
4. Peripheral arthritis- 33%
Asymmetrical, non-erosive, lower limbs
5. Enthesopathy- plantar fascia, Achilles, patella, pelvis
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Clinical Presentation
6. Extra-articular manifestations:
-Acute anterior uveitis- 25-30%
-Cardiovascular: Aortic insufficiency, ascending aortitis, conduction disturbances
-Lung fibrosis- apex
-Neurological manifestations due to cervical spine (+fractures) involvement
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Physical examination
1. Sacroileitis
2. Limited spine movements (hyperextension, lateral flexion)
3. Loss of lumbar lordosis+ thoracic kyphosis
4. Limited chest expansion
5. Peripheral arthritis
6. Enthesitis
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Diagnostic Criteria
1. LBP3 months improved with exercise, not relieved by rest
2. Limited lumbar spine motion
3. Decreased chest expansion
4. Sacroiileitis
Definite AS= 4+ any one
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Reactive Arthritis
Aseptic peripheral arthritis occurring within 1 month of a primary infection elsewhere in the body.
Triggering infection:
1. Genitourinary infection- Chlamydia trachomatis
2. Enteritis due to gram negative enterobacteria: Salmonella, Shigella, Yersinia, Campylobacter
3. Treatment with BCG injection for bladder cancer
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Epidemiology
Annual Prevalence: 30-40/105
HLA-B27: 40-80%
HLA-B27+ X50 risk for developing the disease
F=M.
Usually young adults
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Clinical Presentation
1. General symptoms: malaise, fatigue, fever
2.Musculoskeletal symptoms
-Monoarthritis or asymmetyric olygoarthritis
Weight bearing joints: Knees,ankles,hips
- Enthesitis:Achilles tendonitis, plantar fasciitis
- Dactylitis (“sausage digits”)
15-30% develop chronic/recurrent arthritis sacroiileitis
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Clinical Presentation
3. Genitourinary symptoms:
- Urethritis, cystitis,
- Cervicitis, prostatitis
4. Ocular lesions:
- Conjuctivitis (33%)
2. Anterior uveitis (5%)
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Clinical Presentation5. Mucocutaneous lesions
- Keratoderma Blenorrhagicum
- Circinate Balanitis/ Vulvitis
- Painless ulcer in the mouth
- Nail lesions
6. Cardiac involvement-rare
- Carditis
-Conduction disturbances
Reiter syndrome= arthritis+urethritis+conjuctivitis
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Psoriatic Arthritis (PsA)
Inflammatory arthritis associated with psoriasis
Prevalence of psoriasis: 1-3%
Prevalence of arthritis in psoriasis: 7-42%
-75% psoriasis precedes PsA
-15% synchronous onset
-10% arthritis precedes psoriasis
M=F
PsA usually begins between 30- 50 years
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Clinical presentation
I. Articular patterns:
1. Asymmetric oligoarthritis- most common
2. Arthritis of distal interphalangeal joints
3. Symmetric polyarthritis (dd: RA)
4. Arthritis mutilans
5. Spondyloarthropathy
II. Dactylitis- 30%
III. Enthesopathy
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Enteropathic Arthritis
Inflammatory arthritis associated with:
1. Inflammatory bowels disease(Crohn’s disease, ulcerative colitis)
2. Infectious enterocolitis
3. Whipple’s disease
4. Intestinal bypass surgery
5. Coeliac disease
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Clinical Presentation
I. Articular manifestations
1. Monoarthritis, asymmetrical olygoarthritis:2-20%
large+small joints of lower limbs
less frequent- hips, shoulders
+enthesopathy
correlates with GI manifestations
M=F
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Clinical Presentation
I. Articular manifestations
2. Axial involvement: 5-12%
sacroiileitis,spondylitis
no correlation with GI
M.>F X3
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Clinical Presentation
II. Acute anterior uveitis 3-11%
HLA-B27 +
+ Axial involvement
III. Skin lesions: 10-25%
1. Erythema nodosum
2. Pyoderma gangrenosum
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Investigations in SA
I. Lab tests
1. ESR, CRP- 75%
2. Mild normocytic anemia- 15%
3. IgA
4. ALP
5. RF, ANA, C- normal
6. HLA-B27 (not diagnostic)
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Investigations in SA
II. X-ray film
1. Sacroiileitis- postage stamp, pseudowidening, sclerosis, ankylosis
2. Spondylitis- squaring, syndesmophytes, bamboo spine, osteoporosis
3. Enthesitis
III. Bone scan- sacroiileitis?
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Management in SA
Goals:
1. Relief of pain & rigidity
2. Maintaining posture & movement
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Management in SA
I. Drug therapy
1. NSAID !
2. Steroids- for short term, local injections
3. Second line therapy: sulfasalazine, methotrexate
4. Anti-TNF-
II. Physical exercise (swimming!)
III. Physiotherapy (hydrotherapy, passive streching etc.)