Seronegative Spondyloarthropthies

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

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

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

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

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

Epidemiology

Annual prevalence(USA white): 6.6/105

HLA-B27 +: 1-2%

+ 10 degree affected relative: 10-20%

M>F X2-3

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

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

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

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

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

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

Epidemiology

Annual Prevalence: 30-40/105

HLA-B27: 40-80%

HLA-B27+ X50 risk for developing the disease

F=M.

Usually young adults

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

Clinical Presentation

3. Genitourinary symptoms:

- Urethritis, cystitis,

- Cervicitis, prostatitis

4. Ocular lesions:

- Conjuctivitis (33%)

2. Anterior uveitis (5%)

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

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

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

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

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

Clinical Presentation

I. Articular manifestations

2. Axial involvement: 5-12%

sacroiileitis,spondylitis

no correlation with GI

M.>F X3

Clinical Presentation

II. Acute anterior uveitis 3-11%

HLA-B27 +

+ Axial involvement

III. Skin lesions: 10-25%

1. Erythema nodosum

2. Pyoderma gangrenosum

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)

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?

Management in SA

Goals:

1. Relief of pain & rigidity

2. Maintaining posture & movement

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.)