Spondyloarthropathies Brian E. Daikh, MD 7/21/09.
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Transcript of Spondyloarthropathies Brian E. Daikh, MD 7/21/09.
Spondyloarthropathies
Brian E. Daikh, MD7/21/09
Case:
Hx: 20 y.o. male with months of left knee swelling.occasional mouth sores1 episode of bloody diarrhea with ibuprofen4 years of back stiffnessA brother has psoriasis
Exam:Left knee warm with a moderate effusionSpinal flexion limited
Question: What is the DDx and what further information is needed to
determine a diagnosis in this patient?
Spondyloarthropathy: Definitions
• A group of inflammatory arthridites Characterized by:– Synovitis– Enthesitis – inflam. Where tendon connects to bone– Spinal and Peripheral Joint Involvement– Genetic Predisposition– Probable Infectious Cause
• Categories– Ankylosing Spondylitis – Reactive Arthritis– Psoriatic Arthritis– Enteropathic Arthritis – Crohn’s disease, Ulcerative Colitis– Undifferentiated
Spondyloarthropathy: Clinical and Laboratory Features
• Sacroileitis or spondylitis (inflam of ligaments that connect to vert bodies)
• Peripheral arthritis:
– Typically asymmetric and involves the lower limb;
– Upper limb involvement often associated with Psoriatic Arthritis
• Enthesopathy -inflammation at the site of tendinous or ligamentous insertion
• Extra-articular manifestations occur in the minority
• By definition, patients are RF factor negative
• HLA-B27 is present in many individuals, depending on the type of arthritis.
ACR Diagnostic Criteria for Spondyloarthropathy
• Inflammatory Spinal Pain or Joint Synovitis (Asymmetric or predominantly lower limbs)
• AND 1 of the following:
– Positive family history– Psoriasis– IBD– Urethritis or Cervicitis (nongonococcal), or acute diarrhea within
1 month– Buttock pain– Enthesopathy– Sacroileitis
• Sensitivity 78.4% and specificity 89.6%
Differences between RA and Spondyloarthropathy
RA Spondy
Peripheral Arthritis polyarticular pauciarticularSacroileitis xSpondylitis xEnthesitis xSubcutaneous Nudules xRheumatoid Factor xSymmetry x
• Asymmetric peripheral arthritis
• Sausage digits
• Enthesopathy– Achilles tenosynovitis– Plantar fasciitis– Costochondritis
• Acute anterior uveitis/iridocyclitis
• Mucocutaneous lesions
• Nail involvement
• Fatigue, weight loss
• Amyloidosis
• Apical pulmonary fibrosis
• Immunoglobulin A nephropathy
• Cardiac involvement
Spondylarthropathies: nonvertebral manifestations
•Ankylosing spondylitis > 90% (white males)–with uveitis or aortitis ~100%
•Reactive arthritis 50-80%–with sacroiliitis or uveitis 90%
•Juvenile spondylarthropathy 80%•Inflammatory bowel disease–Peripheral Not increased–Axial
•Crohn’s disease 50%•Ulcerative colitis 70%
•Psoriasis –Peripheral Not increased–Axial 50%
HLA-B27 disease associations
HLA-B27
• A member of the MHC Class I gene family• Important in the presentation of processed
antigen to T-cells• Present in 9-11% of the caucasion
population.• A poor screening test; if absent, it is
unlikely the patient has ankylosing spondylitis, but if present, it does not mean the patient has disease.
Pathogenic Role of HLA-B27
• The mechanism is not well defined.
• Arthritogenic Peptide Theory: HLA-B27 may bind unique peptides of self or bacterial origin.
• Molecular Mimicry Theory: Antibodies directed against foreign antigens cross-react with HLA-B27.
• Aberrant Processing Theory: Abnormal folding of protein or expression of heavy chain dimers on the cell surface may lead to abnormal antigen presentation.
Enthesitis
Ankylosing Spondylitis
Ankylosing Spondylitis: Definition and Clinical Features
• A chronic inflammatory arthritis that mainly affects the axial skeleton
• Typical presentation is with low back pain of insidious onset
• Arthritis of the hips and shoulders and enthesopathies are common
• Extra-articular manifestations include: uveitis and rarely aortic valve disease and cauda equina syndrome
Ankylosing Spondylitis - Epidemiology
• Strong HLA-B27 association in all populations
• In Caucasians, AS occurs with a prevalence of 0.5-1.0%
• M:F 5:1
• Incidence and prevalence may be underestimated due to variance in clinical presentation
Characteristics of Back Pain
• Onset– Insidious– Often before age 40
• Duration greater than 3 months• Associated with prominent morning stiffness• Improves with activity
Ankylosing Spondylitis-Initial Management
• History and physical exam– Appropriate history of morning stiffness, measurement of spinal
mobility, examination of peripheral joints, eyes, mouth, skin.
• Laboratory evaluation– CBC, CRP, HLA-B27?
• X-rays– Lumbar spine and sacroiliac joints. C-spine if appropriate
• Other possible modalities-not standard of care at this time.– MRI of the lumbar spine and SI joints if plain x-rays are normal.
AS: Management
• Early diagnosis, patient education, and physical therapy are essential
• Goals of PT are to restore and maintain posture and movement to as near to normal as possible
• Self-management with exercise must be lifelong
• NSAIDS relieve pain and stiffness, but are not disease-modifying
• Sulfasalazine and Methotrexate may be effective (no controlled clinical trials)
• Anti-TNFα agents are very effective in controlled trials. These are the only FDA approved therapies.
Psoriatic Arthritis
Psoriatic Arthritis - Definition
• An inflammatory arthritis associated with psoriasis
• May occasionally be present in the absence of clinically evident psoriasis
Psoriatic Arthritis: Imaging
• Common involvement of wrists, hands, feet, and shoulders.
• In contrast to RA, osteopenia is not observed and DIP joint involvement is common.
• Classic “pencil-in-cup” deformity • May have erosion adjacent to ankylosis or
new bone formation• Periostitis
Psoriatic arthritis-initial evaluation
• History and physical exam– Close attention to the subtle findings of psoriasis, e.g. scalp
involvement, nail pitting. Complete joint exam, including spinal mobility.
• Laboratory evaluation– CBC, chemistries, CRP, RF, anti-CCP antibody (these are to
exclude RA, really)
• Baseline x-rays if appropriate– If the disease is of fairly early onset, baseline x-rays may be
normal.
Psoriatic Arthritis - Treatment
• NSAIDS – mild disease, symptom relief• Intra-articular corticosteroids• DMARDS
– Plaquenil – mild disease– Sulfasalazine – mild disease– MTX – moderate-severe disease– Anti-TNFα agents (These are the only drug
approved by the FDA for the treatment of PsA!) – used in methotrexate nonresponders.
Reactive Arthritis
Reactive Arthritis: Definitions
• Sterile joint inflammation that develops after a previous infection
• The disease is systemic and not limited to the joints
• Triggering infections most commonly originate in the throat, urogenital organs, or GI tract
Epidemiology of Reactive Arthritis
• Most commonly affects young adults
• M = F
• Annual incidence 30-40/100,000
• Worldwide distribution
• Genetic association – HLA-B27
• Frequently associated with infections
Reactive Arthritis: Clinical Features
• Arthritis, enthesitis, tendonitis, tenosynovitis, periostitis, and muscle pain
• Skin and mucous membrane lesions are frequent – oral ulcers and keratoderma blenorrhagicum
• Eye inflammation (uveitis and conjunctivitis)
• Visceral involvement (nephritis and carditis) is rare
• Severity ranges from mild arthralgias to disabling disease
• Spontaneous recovery is common and the prognosis is, in general, good
• Recurrences are not uncommon
• Susceptibility to the disease is strongly linked to HLA-B27 antigen positivity.
Reactive Arthritis: Triggering Infections
• Urogenital Tract– Chlamydia trachomatis– Ureaplasma urealyticum
• Gastrointestinal Tract– Yersinia enterocolitica– Yersinia pseudotuberculosis– Salmonella– Shigella– Campylobacter
• Respiratory Tract– Chlamydia pneumoniae
Reactive arthritis-initial evaluation
• History and physical exam– Appropriate questioning for prodromal illness
• Laboratory evaluation– CBC, chemistries, CRP, urethral or cervical
swabs, stool culture, throat culture.
Reactive arthritis-clinical course
• The clinical course is extremely variable.• The majority of patients have a relatively short, self-
limited course. These patients are often treated successfully with NSAIDs, corticosteroids, and sometimes a short courses of DMARD’s.
• Alternative courses include a waxing and waning course over a period of months or years more chronic, persistent inflammatory arthritis. These patients require treatment with DMARD’s.
Reactive Arthritis: Treatment
• Antibiotics – probably not helpful
• NSAIDS – symptomatic relief
• Sulfasalazine – may be disease modifying, peripheral joints > axial skeleton
• Methotrexate – May be disease modifying
• Anti-TNFα Agents – may be very effective
Conclusions
• The Spondyloarthropathies are a diverse group of inflammatory arthropathies that share the characteristics of arthritis and enthesitis.
• HLA-B27 likely plays a pathogenic role in many of these conditions.
• Extraarticular manifestations are uncommon, but may be severe.
Spondyloarthropathies – Clinical Pearls
• All of these conditions are diagnosed primarily based on clinical features.
• Extra-articular manifestations (skin, eye, GI) may provide important clues.
• X-rays (sacroileitis, spondylitis, erosions) may also provide clues to the Dx.
• Lab tests will not make the Dx
Spondyloarthropathies – Clinical Pearls
• Mild disease (low grade swelling, normal acute phase labs – NSAID, Plaquenil, Sulfasalazine
• Mild-Moderate disease – Sulfasalazine or Methotrexate – except spine – consider TNF blocker.
• Moderate – Severe disease – begin with Methotrexate
• Plaquenil and Sulfasalazine will not affect the skin in Psoriatic Arthritis