Spondyloarthropathies (Seronegative Arthritis)
·
Rheumatoid factor is negative –
but exclude seronegative RA
·
Clinical overlap between the
conditions
·
Acronym: PEAR: Psoriasis, Enteropathic, Ankylosing
Spondolytis, Reactive/Reiter‟s
·
Have in common:
o Involvement of spine and sacroiliac joints (= axial arthritis)
o Usually asymmetrical large joint mono or oligo-arthritis
o Inflammation then calcification of tendon insertions (enthesopathy)
o Extra-articular manifestations: uveitis, aortic regurgitation, upper
zone pulmonary fibrosis
o Familial tendency + HLA-B27 +ive predisposition
·
If type not clear then classified
as „Undifferentiated spondyl-arthropathy‟:
·
= Chronic systemic inflammatory
disorder of the axial skeleton, affecting SI joints and spine
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Ankylosing = fibrous replacement
of the joint ® bony fusion
·
Epidemiology:
o Prevalence: 2 – 5 per 1,000 males.
Men have more progressive disease
o Men more common and present earlier (6:1 at 16 years, 2:1 at 30 years)
o Onset usually between 15 – 40 years
o Closely linked to HLA-B27:
§ 5 – 20% risk for positive individual
§ 11 HLA subtypes identified with different disease susceptibilities
§ Strong ethnic variances in HLA prevalence: present in Caucasians, absent
in indigenous people of South America and Australia, high prevalence in
Eskimos….
·
Clinical presentation:
o 75% first present with insidious onset of dull back ache, worse at night, improved by exercise
o Morning stiffness, backache, sacroiliac pain, loss of spinal movement
(spinal ankylosis, distraction of < 10 cm on flexion with Schober‟s test)
o Leading to flattening of lumber spine, thoracic kyphosis, neck
hyperextension
o Fatigue common
·
Distribution:
o Sacroiliac joints and spine (lumbar to start with, C-spine later):
§ Bilateral sacro-iliac joint tenderness
§ Tenderness of the lumbar vertebrae
§ Loss of thoracic kyphosis and lumbar lordosis
§ Early restriction in lateral flexion of the spine – test by seeing how far they can slide their hand down the side of their leg without bending forward. Later loss of movement in all directions
o Hips (30%), also knees and shoulders
o Peripheral arthritis infrequent
·
Other features:
o Commonly:
§ Enthesitis
§ Iritis/Anterior Uveitis (25 – 30%): unilateral, acute, painful, with
photophobia and blurred
§ vision. To test: shining light in
opposite eye causes pain in the affected eye
§ Costocondriasis + chest pain referred from thoracic vertebrae
§ Chest wall rigidity ® ¯VC
§ Plantar fasciitis
o Rare:
§ Neurological involvement: secondary to spinal fracture (eg C-spine), atlanto-axial subluxation, cauda equina syndrome
§ Amyloidosis
§ Carditis and aortic regurgitation due to fibrosis of the aortic valve
(can also affect AV bundle ® arrhythmias)
§ Apical lung fibrosis (rare)
·
Pathogenesis:
o Cross reactivity between Klebsiella pneumoniae antigens and HLA B27
o Antibody complexes cause synovitis, enthesopathy (including tendon
attachment calcification) ® capsular ossification, ankylosis (bony fusion) of the sacroiliac joint,
inflammatory arthritis of the synovial joints in the spin and ossification of
spinal ligaments
·
Diagnosis is clinical: History of
inflammatory spine disease + SI tenderness, ¯L-spine mobility, ¯chest
expansion
·
Differential from RA:
o Spine rarely affect in RA
o Small peripheral joints rarely affected in AS
o In AS there are no subcutaneous nodules and no RF (but there may not be
in RA either)
· Investigations:
o X-rays: „bamboo‟ or „railroad‟ spine, squaring of vertebrae,
syndesmophytes, erosions of the apophyseal joints (between rib tuberosities and
spinal processes), eventually bony ankylosis of the
o SI joints (also seen in Reiter‟s and Crohn‟s diseases)
o Bloods:
§ FBC (mild normochromic anaemia in 15%)
§ ÂESR and
CRP
·
Treatment:
o Physiotherapy/Exercise (not rest) to maintain posture and mobility
o NSAIDs to relieve pain and stiffness (especially phenylbutazone). If ineffective try sulphasalazine
o Local corticosteroids for uveitis, enthesitis, peripheral synovitis
o Disease Modifying drugs if severe
·
Epidemiology: occurs in 5 – 7% of
psoriasis patients, age 35 – 45, male = female
·
Pathology:
o Can have a reactive type presentation due to a host of possible infective/inflammatory agents
o Primary lesion = synovitis (similar to RA): hypertrophic villi, T-cell
infiltration, aggregates of T cells. But usually only minimal joint impairment.
Articular destruction in a subset (25%) with panus formation, cartilage
erosion, etc = Arthritis Mutilans
·
Clinical presentation:
o Usually psoriasis develops first, then arthritis, but 15% go the other
way
o Usually insidious but can present acutely
o Check for nail pitting, transverse ridging, oncyholysis
o Extra-articular manifestations are uncommon (except for conjunctivitis
and iritis)
·
Distribution:
o Often asymmetric, mainly oligo but can be poly arthritis
o Often upper limb
o DIP joints in hands and feet especially affected – unique to PA
o Sacroiliac joints and spine (20 – 40%) – asymmetric involvement common
o Rheumatoid pattern
o Inflammation of digital tendon sheaths ® sausage
finger (dactylitis)
o Enthesitis: Achilles tendonitis and plantar fasciitis
·
Diagnosis: Psoriasis (exclude
seborrhoeic dermatitis and fungal infections) or psoriatic nail involvement +
sero-negative arthritis. Increased likelihood in B27 +ive
·
Investigations: X-ray of hands ® DIP
involvement + resorption of the terminal phalanges
·
Treatment:
o NSAIDs for pain – but may worsen skin lesions
o Corticosteroid injections for local synovitis
o If severe: methotrexate, cyclosporin, sulphasalazine, gold etc
·
Differentiating from RA:
o Presence of skin rash
o Asymmetric
o DIP and PIP involvement
o Can overlap with RA and present as a symmetrical, destructive arthritis.
Look for psoriasis and nail changes
·
Classic triad: urethritis,
conjunctivitis and seronegative arthritis. Recurrence in 50%, attacks can last
several months
·
Caused by sterile synovitis
following chlamydia/NSU/shigella infection
·
Distribution of arthritis usually
lower limb (may be chronic or relapsing):
o Sacroiliac joints and spine
o Hips
o Knees
o Ankles and most of the joints of the feet
·
Other features:
o Iritis
o Keratoderma blenorrhagica (brown, aseptic abscesses on soles and palms)
o Mouth ulcers
o Circinate balanitis (painless serpiginous penile rash)
o Enthesopathy (plantar fasciitis, Achilles tendonitis)
o Not onycholysis (differentiates from psoriasis)
·
Investigations:
o Chlamydia: First of 2 glass urine test shows more debris in the first
glass in urethritis (cf prostatitis where there is more in the 2nd)
o Anti-chlamydial antibodies
o Neutrophils in synovial fluid
o X-rays: periosteitis at ligamentous insertions. Rheumatoid like changes if chronic
·
Pathogenesis: following
non-specific urethritis, Chlamydia or Shigella infection in those genetically
pre-disposed (ie HLA B27). Hyperaemic synovial membrane, but no panus or
cartilage erosion (except if progressive). Profuse osteolysis and formation of
new periosteal bone
·
Management: treat causal agent,
rest, NSAIDs, steroid injections, recovery may be slow
·
Infective causes: Yersinia,
Chlamydia, Campylobacter, Salmonella, Shigella, Clostridium difficile…
· (all have lipopolysaccharide in their outer cell membrane)
·
® Sterile immunological reaction in joints due to cross reactivity of
antigens
·
Usually B27+
· Presentation:
o Acute asymmetrical polyarthritis (esp of lower limb) 1-2 weeks post
infection lasting for 3 – 6 months
o Can become chronic with relapsing and remitting course
o Enthesitis is common (eg ® plantar fasciitis or Achilles
tendonitis)
o Can also get:
§ Skin lesions resembling psoriasis: Circinate balanitis, keratoderma
blenorrhagica and nail dystrophy
§ Iritis
·
Investigations:
o Causative agent: Blood culture/serology for antibodies/stool culture
o HLA-B27, X-ray, ESR, joint aspiration for sceptic arthritis
·
Diagnosis is clinical
·
Management:
o Treating persisting infection has little impact on course
o NSAIDs/corticosteroids
o Sulphasalazine etc if necessary
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Also in leukaemia, endocarditis,
acne, acromegaly, Wilson‟s disease, sarcoid, sickle cell, haemochromatosis
·
Associations:
o Inflammatory bowel disease (15% of Crohn‟s and UC get arthritis)
o Also associated with intestinal bypass surgery and Whipple‟s Disease
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Asymmetrical lower large joint
mono- or oligo arthropathy
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No joint destruction
·
Sacroiliitis or Spondylitis in 5%
(70% of these have HLA-B27)
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Manage underlying condition:
o Sulphasalazine for both bowel disease and arthritis
o NSAIDs and steroid injections for monoarthritis
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