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 SA I. 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. LBP3 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 Presentation 5. 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.)