Spondyloarthropathies

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Spondyloarthropathies

• Brian E. Daikh, MD
• 7/21/09

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Case

• Hx
• 20 y.o. male with months of left knee swelling.
• occasional mouth sores
• 1 episode of bloody diarrhea with ibuprofen
• 4 years of back stiffness
• A brother has psoriasis
• Exam
• Left knee warm with a moderate effusion
• Spinal flexion limited
• Question What is the DDx and what further
  information is needed to determine a diagnosis in
  this patient?

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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 Crohns disease,
  Ulcerative Colitis
• Undifferentiated

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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.

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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

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Differences between RA and Spondyloarthropathy
RA Spondy Peripheral
Arthritis polyarticular pauciarticular Sacroileit
is x Spondylitis x Enthesitis x Sub
cutaneous Nudules x Rheumatoid
Factor x Symmetry x
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Spondylarthropathies nonvertebral manifestations

• 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

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HLA-B27 disease associations

• Ankylosing spondylitis gt 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
• Crohns disease 50
• Ulcerative colitis 70
• Psoriasis
• Peripheral Not increased
• Axial 50

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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.

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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.

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Enthesitis
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Ankylosing Spondylitis
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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

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Ankylosing Spondylitis - Epidemiology

• Strong HLA-B27 association in all populations
• In Caucasians, AS occurs with a prevalence of
  0.5-1.0
• MF 51
• Incidence and prevalence may be underestimated
  due to variance in clinical presentation

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Characteristics of Back Pain

• Onset
• Insidious
• Often before age 40
• Duration greater than 3 months
• Associated with prominent morning stiffness
• Improves with activity

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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.

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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-TNFa agents are very effective in controlled
  trials. These are the only FDA approved
  therapies.

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Psoriatic Arthritis
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Psoriatic Arthritis - Definition

• An inflammatory arthritis associated with
  psoriasis
• May occasionally be present in the absence of
  clinically evident psoriasis

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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

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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.

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Psoriatic Arthritis - Treatment

• NSAIDS mild disease, symptom relief
• Intra-articular corticosteroids
• DMARDS
• Plaquenil mild disease
• Sulfasalazine mild disease
• MTX moderate-severe disease
• Anti-TNFa agents (These are the only drug
  approved by the FDA for the treatment of PsA!)
  used in methotrexate nonresponders.

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Reactive Arthritis
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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

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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

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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.

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Reactive Arthritis Triggering Infections

• Urogenital Tract
• Chlamydia trachomatis
• Ureaplasma urealyticum
• Gastrointestinal Tract
• Yersinia enterocolitica
• Yersinia pseudotuberculosis
• Salmonella
• Shigella
• Campylobacter
• Respiratory Tract
• Chlamydia pneumoniae

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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.

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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
  DMARDs.
• 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 DMARDs.

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Reactive Arthritis Treatment

• Antibiotics probably not helpful
• NSAIDS symptomatic relief
• Sulfasalazine may be disease modifying,
  peripheral joints gt axial skeleton
• Methotrexate May be disease modifying
• Anti-TNFa Agents may be very effective

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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.

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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

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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