Seronegative
spondyloarthropathy
(Read
and study the relevant articles in the course manual)
Group
of inter-related and overlapping inflammatory joint diseases that
all share a common pathology and strong association to the HLA-B27
antigen and are negative for rheumatoid factor (seronegative). Used
to be referred to as variants of rheumatoid arthritis as they share
many pathologic and radiologic features with rheumatoid arthritis.
‘Spondylos’ is Greek for vertebrae.
5-14%
of the general population have the HLA-B27 antigen – up to
90% of those with a seronegative spondyloarthropathy have it. To
a lessor extent, HLA-CW6 has an association.
Generally,
but inconsistently, all tend to have a peripheral arthritis (usually
lower limb and asymmetrical – rheumatoid is usually symmetrical);
sacroilitis (more common radiologically than clinically; presents
as bilateral discomfort in buttocks); enthesopathy; negative for
rheumatoid factor; have no nodules or the other extra-articular
features of rheumatoid arthritis; and have a marked familial aggregation.
Enthesopathy
is often manifested in the foot in these conditions as plantar heel
pain. Erosions are repaired by reactive bone --> bony spurs.
Ankylosing Spondylitis (Marie-Strumpell’s
disease)
Chronic
inflammatory disorder primarily affecting the axial skeleton, but
with significant involvement of the appendicular skeleton -->
affected joints tend to fibrosis and ankylosis.
Cause
unknown, but genetic factors play a role - strongly associated with
HLA-B27 histocompatibility antigen. Affects up to 1% of population.
Clinical
features:
* Usually insidious onset of intermittent hip, buttock
or back pain – associated with decreased mobility –
insidious onset often leads to delay in diagnosis.
*
Usually worse in morning and in middle of night – usually
15-35 years (there is a well recognised juvenile onset ankylosing
spondylitis).
*
The stiffness improves with movement and exercise. M:F = 3-5:1.
More severe outcome if age of onset is earlier.
*
In females, first symptoms can often occur during pregnancy.
*
25% have iritis; 4% develop cardiovascular disease; some have breathing
difficulties due to limited chest expansion. 50% have peripheral
arthritis at some stage during disease.
*
X-ray of sacroiliac joint --> erosions, sclerosis, joint space
narrowing – later get sclerosis and ankylosis.
*
Can also get C1-C2 subluxations.
Extra-articular
involvement:
- Aortic valve regurgitation; breathing difficulties from restriction
of rib cage; iritis; cauda equina syndrome; prostatitis (in men);
secondary amyloidosis
Course
is highly variable – characterised by exacerbations and remissions.
Less then 20% now go on to significant disability. Life expectancy
appears not to be reduced.
Involvement
of foot:
• foot is affected less frequently than in psoriatic
spondyloarthropathy and Reiter’s syndrome
• Arthritis is bilateral – can by symmetric or asymmetric
– usually MPJ’s and first tarsometatarsal joint. X-rays
? soft tissue swelling, joint space narrowing, erosions –
also get adjacent bone proliferation.
• up to 30% are considered to get plantar heel pain and/or
achilles tendonitis
• X-ray of calcaneus – plantar proliferation (from enthesopathy)
and posterosuperior erosions (from bursitis). Erosive changes are
generally found in early stages and the sclerotic proliferative
changes are more typical late in the disease (Lopez-Bote et al,
1989)
• Enthesopathy – at attachments of plantar fascia, achilles
tendon and peroneus brevis.
• Synovitis of metatarsophalangeal joints
• Lateral deviation of lessor digits (less severe than rheumatoid
arthritis)
• Bony ankylosis of small joints can occur
• Gait analysis has shown reduced stride length, decreased
flexion at knee and hip in those that are asymptomatic compared
to healthy controls (Zebouni et al, 1992)
• may seek Podiatric consultation due to difficulty in reaching
feet due to spinal problems
Management:
• In most cases, AS is relatively mild with reasonable
prognosis
• Aim of management is pain relief and prevention of deformity
? importance of early diagnosis
• Patient education (sleep on form mattress; exercises; avoid
smoking; patient support groups)
• NSAID’s (usually very effective for spinal pain);
sulfasalazine appears to be an effective second line drug. Methotrexate
if sulfasalazine is ineffective.
• Physiotherapy -postural exercises, joint mobility; activity
and range of motion exercises to prevent spinal rigidity (swimming
helpful); breathing exercises
• Surgery in advanced cases for deformity (hip replacement;
vertebral osteotomy)
Psoriatic
Arthritis (PsA)
*
Inflammatory arthritis in 2-7% of those with psoriasis (psoriasis
affects 1-2% of population).
*
Most have previous long history of psoriasis – all forms of
psoriasis can develop arthritis.
*
High association with HLA-B27 and HIV infection.
5
broad clinical types:
1) Oligoarticular & polyarthritis with distal interphalangeal
joint involvement and other ‘scattered’ joints (often
less than four) - asymmetric – usually get the classic ‘sausage’
digits (affects 50-70%).
2) Asymmetric involvement of distal interphalangeal joints of hands
and feet – digits affected - usually have nail changes; progressive
bone erosions frequently occur (affects 5-10%)
3) Symmetric seronegative polyarthritis resembling rheumatoid arthritis
(in 15-25%; morning stiffness and fatigue are common) – usually
milder/less destructive than rheumatoid arthritis and does not develop
extra-articular manifestations.
4) Sacroilitis and spondylitis (in 5%; resembles ankylosing spondylitis)
– spinal involvement is predominant feature
5) Arthritis mutilans (widespread destructive polyarthritis with
marked bone resorption; in about 1-5%; often get osteolysis of bones
in fingers and toes; frequently get back pain)
*May
also hypothetically get a coincidental rheumatoid arthritis and
psoriasis (due to prevalence of both diseases in community)
Clinical
features:
* M=F, usually 35-50 years (juvenile form, usually 9-12
years).
*
Symptoms are variable due to different clinical types.
*
IPJ’s of fingers and toes most commonly involved – knee
and ankle are occasionally involved.
* Arthritis precedes or occurs simultaneously with the onset of
the psoriasis in about a third of cases.
* No relationship between severity of psoriasis and psoriatic arthritis,
but the arthritis is more common in those with more severe skin
lesions.
*
There is a relationship between severity of psoriatic nail changes
and arthritis.
* Occasionally have fatigue, fever, conjunctivitis and iritis
* Crystal induced arthropathy has a higher incidence in those with
psoriatic arthritis.
Laboratory
tests – raised ESR, mild neutropenia, raised gammaglobulins,
no RA factor, mild anaemia, high neutrophil count in synovial fluid.
Differential
diagnosis – Reiter syndrome, gout, rheumatoid arthritis.
Involvement
of foot:
• Heel pain is presenting feature in up to 10%
• Usually bilateral and asymmetric – foot is one of
most common sites of arthritis and may be initial presenting feature
of psoriatic arthritis – usually IPJ’s.
• Posterior tibial dysfunction may be presenting feature of
psoriatic arthritis – other tendon sheaths can also be commonly
involved
• X-ray of foot - marginal erosions (bilateral and symmetrical),
bone proliferation, joint space loss (often severe), osteolysis
of distal tufts, destruction of IPJ’s, ‘pencil in a
cup’ deformity of phalanges. Calcaneus will generally show
proliferative and erosive changes of posterior and inferior aspects.
Achilles tendon may be thickened.
• “Sausage toe” – toe is swollen (dactylitis)
– due to involvement of MPJ, PIPJ and DIPJ’s –
shows up on bone scan as a ‘hot toe’.
• up to 70% get plantar heel pain – other entheses also
get affected
• distal involvement of the plantar fascia can also occur
• Tarsal tunnel syndrome
• Nail changes – (in 80%) - pitting, ridging, hyperkeratosis
and onycholysis
Management:
• Managed similar to rheumatoid arthritis, but prognosis
is better.
• Physical therapy & rehabilitation (preserve joint range
of motion and strength muscle)
• Drugs (NSAID’s; gold salts; antimalarials, sulfasalazine;
immunosuppressive drugs; corticosteroids)
Reactive Arthritis (ReA)
(Reiter’s syndrome)
*
Post-infectious disorder (not a local infection, but a reaction).
*
Classic triad (Reiter – 1916) of arthritis, urethritis and
conjunctivitis following infection.
* Reiter’s syndrome is now not the preferred name (Reiter
was involved with Nazi politics and medical experiments).
Syndrome
now considered consisting of:
1) Arthritis
2) Urethritis
3) Conjunctivitis
4) Mucocutaneous lesions
However, not all patients will exhibit all of these features.
Usually
follows infection with Yersina, Salmonella, Campylobacter (gastrointestinal
infections) and Chlamydia (genitourinary infection).
Clinical
features:
* M>F. Usually 15 to 35 yrs.
*
Abrupt onset with urethritis is usually first manifestation with
low-grade fever.
*
Urethritis in some may be asymptomatic.
*
History should reveal recent infection.
*
Conjunctivitis (usually mild) and arthritis follows urethritis.
* Asymmetric arthritis – oligoarticular and usually lower
extremity (knees, ankles and MPJ’s) – joints are tender
and warm; joint stiffness is early predominant feature.
* Some develop mucocutaneous lesions – small mouth ulcers
Involvement
of foot:
• Asymmetric involvement of MPJ’s and IPJ’s.
• Achilles tendonitis and plantar fasciitis are common and
may be severe (enthesitis).
• Keratoderma blennorrhagia – psoriasis like lesion
on sole of foot (in 5-30%) – appears as small reddish to yellow
brown vesicles
• X-rays may show calcaneal erosions and ‘fluffy’
spurs; joint space loss, poorly joint defined erosions, periarticular
osteoporosis, soft tissue swelling
• “Sausage toe” – toe is swollen (dactylitis)
– shows up on bone scan as a ‘hot toe’.
• 50% have some form of polyarthritis in foot joints or heel
pain
• Ankle and first metatarsophalangeal joint involvement are
the most common joints affected in foot.
Treatment:
* Antibiotics; symptomatic (eg NSAID’s); immunosuppressive;
corticosteroids
* Physical therapy
* Eye drops.
Enteropathic Spondylitis (Enteropathic synovitis)
*
Joint manifestations associated with chronic inflammatory bowel
diseases – usually ulcerative colitis (UC) and Crohn’s
disease (CD).
*
Can also be associated with intestinal bypass surgery (bypass arthritis-dermatitis
syndrome), infectious gastroenteritis, pancreatic disease, biliary
cirrhosis, coeliac disease (gluten sensitive enteropathy) and Whipple’s
disease.
Clinical
features:
* Peripheral arthritis – in 12% of UC and 20% of
CD;
*
abrupt onset; usually 5 joints or less; migratory; asymmetric; knees,
ankles and MPJ’s most common.
*
Peripheral arthritis is more likely if colon is extensively involved
– first attack usually occurs within 2 years of onset of bowel
disease – the attacks coincide with exacerbation of bowel
disease in up to 70% of the time.
* Axial arthritis/spondylitis – similar to ankylosing spondylitis
symptoms – occasionally occurs before bowel disease.
In
foot
*
get asymmetric involvement of proximal IPJ’s.
*
Foot involvement more likely in UC.
*
Achilles tendonitis and plantar fasciitis (enthesopathy) can also
occur.
* Attacks of arthritis are usually self-limiting (resolve in 1-2
months) and permanent joint abnormality is not common.
Treatment:
Treatment of bowel disease; NSAID’s; physical therapy;
sulfasalazine; infliximab
Undifferentiated spondylitis
These
are subsets of patients who have some of the features of seronegative
spondyloarthropathy, but do not meet the criteria for the well recognised
conditions.
They
may be an early stage of a known spondyloarthropathy, an atypical
variant of a known spondyloarthropathy or an unknown type of spondyloarthropathy.
In
a cohort of 22 patients followed for 11 years, 15 (68%) developed
ankylosing spondylitis, 1 developed psoriatic arthritis, 4 remain
undifferentiated and 2 went into remission (Kumar et al, 2001).
Prevalence
is not known, but is assumed to be more common than the other seronegative
spondyloarthropathy’s.
Can
have – sausage digits/dactylitis; insertional tendonitis (eg
achilles); asymmetrical sacroilitis; periostitis; spurs; peri-insertional
osteoporosis and erosions
Online
links:
ePodiatry's
links to articles on seronegative
spondyloarthropathy
Full
text online resource on the seronegative arthropathies from the
South Australian Orthopaedic Registrars Notebook
Medical Hubs links on Ankylosing Spondylitis |
