The Spondyloarthropathies Flashcards

1
Q

Ankylosing Spondylitis

A

Ankylosing spondylitis is a HLA-B27 associated spondyloarthropathy. It typically presents in males (sex ratio 3:1) aged 20-30 years old

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2
Q

Ankylosing Spondylitis - Ix

A

Inflammatory markers (ESR, CRP) are typically raised although normal levels do not exclude ankylosing spondylitis.

HLA-B27 is of little use in making the diagnosis as it is positive in:
90% of patients with ankylosing spondylitis
10% of normal patients

Plain x-ray of the sacroiliac joints is the most useful investigation in establishing the diagnosis. Radiographs may be normal early in disease, later changes include:
- sacroilitis: subchondral erosions, sclerosis
NB: Sacroiliitis may present as sclerosis of joint margins which can be asymmetrical at early stage of disease, but is bilateral and symmetrical in late disease.
- squaring of lumbar vertebrae
- ‘bamboo spine’ (late and uncommon)
- syndesmophytes: due to ossification of outer fibers of annulus fibrosus
chest x-ray: apical fibrosis

Spirometry may show a restrictive defect due to a combination of pulmonary fibrosis, kyphosis and ankylosis of the costovertebral joints.

A syndesmophyte is a bony growth originating inside a ligament, commonly seen in the ligaments of the spine, specifically the ligaments in the intervertebral joints leading to fusion of vertebrae.

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3
Q

Ankylosing Spondylitis - Mx

A

Management

The following is partly based on the 2010 EULAR guidelines (please see the link for more details):
encourage regular exercise such as swimming
physiotherapy
NSAIDs are the first-line treatment
the disease-modifying drugs which are used to treat rheumatoid arthritis (such as sulphasalazine) are only really useful if there is peripheral joint involvement
the 2010 EULAR guidelines suggest: ‘Anti-TNF therapy should be given to patients with persistently high disease activity despite conventional treatments’
research is ongoing to see whether anti-TNF therapies such as etanercept and adalimumab should be used earlier in the course of the disease

NSAIDs are the first-line drug treatment in an ankylosing spondylitis. Regular exercise is also very important. The role of anti-TNF therapies is increasing but they are not currently first-line and the 2008 NICE guidelines specifically advise against using infliximab

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4
Q

Ankylosing Spondylitis - Morning back pain

A

Note the history of morning pain is typical for an inflammatory arthritis such as ankylosing spondylitis.

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5
Q

Ankylosing Spondylitis - Initial Mx: Example Question

A

A 23-year-old Sri Lankan male presents with 6 months of gradual onset low back pain, worse before waking. He describes increasing stiffness in his right wrist and left third metacarpal joints. On examination, you note reduced spinal movements in lateral spinal flexion and rotation and a positive Schober’s test. He has not received any previous treatments for his back pain and has no other past medical history. What is the most appropriate initial management?

	Start sulphasalazine
	Start infliximab
	Start etanercept
	> Physiotherapy and NSAIDs
	No treatment

The patient gives a classic description of new onset ankylosing spondylitis. He presents from the typical age group of between 15-25. NSAIDs and physiotherapy should be the first line treatment for all symptomatic AS patients, allowing up to 4 weeks for assessment of effect. Up to 70% of AS patients receive sufficient symptomatic relief with NSAIDs alone, with the most recent EULAR guidelines recommending continuous NSAIDs therapy for those with active persistent symptoms1. There is also evidence that this reduces radiological progression of the disease.

Systemic glucocorticoids have no place for AS management but intra-articular steroid injections may be indicated in peripheral joints or enthesitis. Of traditional DMARDs, sulphasalazine is the only DMARD with evidence of efficacy in peripheral joint involvement but is not effective in those with axial joint involvement2. TNF-alpha inhibitors are recommended on those with AS symptoms insufficiently controlled by NSAIDs alone. There appears to be no difference in efficacy between etanercept, infliximab or adalimumab

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6
Q

Ankylosing Spondylitis - Features

A

Ankylosing spondylitis is a HLA-B27 associated spondyloarthropathy. It typically presents in males (sex ratio 3:1) aged 20-30 years old.

Features
typically a young man who presents with lower back pain and stiffness of insidious onset
stiffness is usually worse in the morning and improves with exercise
the patient may experience pain at night which improves on getting up

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7
Q

Ankylosing Spondylitis - Clinical Examination

A

Clinical examination
reduced lateral flexion
reduced forward flexion - Schober’s test - a line is drawn 10 cm above and 5 cm below the back dimples (dimples of Venus). The distance between the two lines should increase by more than 5 cm when the patient bends as far forward as possible
reduced chest expansion

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8
Q

Ankylosing Spondylitis - THE As!

A
Other features - the 'A's 
Apical fibrosis
Anterior uveitis
Atlantoaxial Subluxation
Aortic regurgitation
Achilles tendonitis
AV node block
AA Amyloidosis
Arthritis (peripheral) 
Arachnoiditis
IgA Nephropathy
and cauda equina syndrome
peripheral arthritis (25%, more common if female)

Arachnoiditis is a pain disorder caused by the inflammation of the arachnoid, one of the membranes that surrounds and protects the nerves of the spinal cord. It is characterized by severe stinging, burning pain, and neurological problems

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9
Q

Ankylosing Spondylitis - Cardiac Cx: Example Question

A

A 75 year old male presents to A&E with two episodes of loss of consciousness over the last 48 hours. Both episode were witnessed by his wife, onset while sitting in his chair at home, without any witnessed limb jerking, urinary incontinence or tongue biting. He denies any chest pain or shortness of breath normally but reports gradually being able to walk increasingly shorter distances, which he attributed to old age. He has no other significant past medical history, lives with his wife and is a lifelong non-smoker. On examination, he has a significant thoracic kyphosis. On flexion of the lower back, the marked distance increased from 15 cm to 18 cm. He also has a poverty of spinal lateral flexion and bilateral spinal rotation. His cardiovascular examination reveals heart sounds I and II with an early diastolic murmur. Respiratory examination reveals fine inspiratory crackles at both apices. His lying and standing blood pressures are unremarkable. A CT head demonstrated only mild microangiopathic disease. The patient is currently comfortable and alert, requesting to go home. He is attached to cardiac telemetry. What do you expect his ECG to show?

Sinus bradycardia
Trigeminy
Fast atrial fibrillation with ventricular response greater than 100
Atrial flutter
> Bradycardia with 1st degree heart block

The clinical description is of a patient with ankylosing spondylitis, associated with multiple extra-articular features, commonly remembered by MRCP candidates as the As. They classically include atlano axial subluxation, arachnoiditis, apical fibrosis, anterior uveitis, aortitis, aortic regurg, AV block, amyloidosis, IgA nephropathy, Achilles tendonitis and associations with plantar fasciitis and inflammatory bowel disease (the last two are a bit of a stretch!). In this case, the patient is Schobers positive with a murmur of aortic regurgitation from aortitis. The most likely cause of these episodes of syncope are cardiac in origin. AV node block results in first degree heart block on ECG, resulting in symptoms if bradycardia leads to transient cerebral hypoperfusion.

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10
Q

Ankylosing Spondylitis in a female pt - Example Question

A

A 26-year-old woman presents with a four month history of back pain. The pain is located around the lower lumbar vertebrae and spreads to both buttocks. Ibuprofen and walking seem to improve the pain. A lumbar spine film is requested:

SEE PASSMED

What is the most likely cause of this patients back pain?

	Marble bone disease
	Alkaptonuria
	> Ankylosing spondylitis
	Facet-joint dysfunction
	Rheumatoid arthritis

Ankylosing spondylitis with well formed syndesmophytes are seen on the lumbar spine film.

The first thing to address is the sex of the patient. Of course ankylosing spondylitis is more common in men but the male-to-female ratio is only 3:1. This means it is reasonable to be asked about female patients in questions, particularly if there is accompanying ‘hard evidence’ such as x-rays.

Marble bone disease (osteopetrosis) results in dense, thick bones that are prone to fracture. Syndesmophytes are not a feature.

Facet-joint dysfunction is a common cause of back pain but it would not explain the x-ray findings.

Rheumatoid arthritis of course does not commonly present with back pain. The following x-ray changes are typically seen:
loss of joint space
juxta-articular osteoporosis
soft-tissue swelling
periarticular erosions
subluxation
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11
Q

Ankylosing Spondylitis - Classical Fx

A
  • Typically a young male who presents with lower back pain and stiffness
  • Stiffness is worse in morning
  • Stiffness improves with activity
  • Peripheral arthritis (25% more common if F)
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12
Q

Psoriatic Arthritis

A

Psoriatic arthropathy correlates poorly with cutaneous psoriasis and often precedes the development of skin lesions. Around 10-20% percent of patients with skin lesions develop an arthropathy with males and females being equally affected

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13
Q

Psoriatic Arthritis - Types

A

Types*
rheumatoid-like polyarthritis: (30-40%, most common type)
asymmetrical oligoarthritis: typically affects hands and feet (20-30%)
sacroilitis
DIP joint disease (10%)
arthritis mutilans (severe deformity fingers/hand, ‘telescoping fingers’)

*Until recently it was thought asymmetrical oligoarthritis was the most common type, based on data from the original 1973 Moll and Wright paper. Please see the link for a comparison of more recent studies

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14
Q

Psoriatic Arthritis - Hand XR

A

SEE PASSMED X-RAY

X-ray showing some of changes in seen in psoriatic arthropathy.
Note that the DIPs are predominately affected, rather than the MCPs and PIPs as would be seen with rheumatoid. Extensive juxta-articular periostitis is seen in the DIPs but the changes have not yet progressed to the classic ‘pencil-in-cup’ changes that are often seen with psoriatic arthritis.

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15
Q

Psoriatic Arthritis - Mx

A

Management

  • treat as rheumatoid arthritis
  • but better prognosis
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16
Q

Psoriatic Arthritis and Methotrexate

A

Methotrexate is very effective at improving psoriatic arthritis but in addition to this it also has a dramatic effect on skin disease to a much greater extent then the other disease modifying anti-rheumatic drugs (DMARDs) listed. Therefore it is the DMARD of choice in psoriatic arthritis.

If there was a contraindication to methotrexate leflunomide would be used second line for peripheral psoriatic arthritis.

You would only use infliximab or another anti-TNF drugs first line if the patient had a predominantly axial spondyloarthropathy. This is according to the European League Against Rheumatism (EULAR) guidelines

Hydroxychloroquine can worsen skin disease and has little efficacy on psoriatic arthritis and is therefore not used routinely in the condition.

Example Question:

A 24 year old who is known to have psoriasis presents with arthralgia. She has noticed that her knuckles have become swollen and her psoriasis has got much worse over the last four months. On examination, she has severe plaque psoriasis on her extensors and scalp leading to alopecia. Her metacarpophalangeal joints are clearly swollen and tender. She is currently on naproxen 500mg BD, paracetamol 1g TDS, topical steroids and calcipotriol. What medication would you add?

	Leflunomide
	Sulfasalazine
	Hydroxychloroquine
>	Methotrexate
	Infliximab
17
Q

Psoriatic Arthritis - Example Question

A

A 35-year-old woman presents to rheumatology clinic with a 2-month history of symmetrical swelling of the ankles and fingers. She also complains of joint pain and stiffness. The stiffness is primarily worse in the early morning and eases with use. Apart from a recent sore throat, she is otherwise well. She has a family history of type 1 diabetes mellitus. She does not take any prescribed medication but has found herself relying on over-the-counter analgesics to get through the day.

On examination, she has bilateral swelling of the index, ring and middle fingers and bilateral ankle swelling. She has a full range of movement in the fingers, wrists and ankles. There is marked swelling and tenderness to palpation at the distal interphalangeal joints in the index, middle and ring fingers on both sides. There are no skin changes, but yellowing and pitting of the nails are noted.

Blood tests show:

Hb 11.1 g/dl
Platelets 305 * 109/l
WBC 7.8 * 109/l

Na+ 141 mmol/l
K+ 4.2 mmol/l
Urea 5.8 mmol/l
Creatinine 64 µmol/l

Bilirubin	13 µmol/l
ALP	83 u/l
ALT	15 u/l
ESR	50mm/hr
CRP	39 mg/L
Rheumatoid factor	negative

Hand X-ray shows mild erosion at the distal interphalangeal joints of the index, middle and ring fingers on both hands.

What is the diagnosis?

	Rheumatoid arthritis
	Reiters syndrome
	Ankylosing spondylitis
	Yellow nail syndrome
	Psoriatic arthritis

Although the patient does not have a psoriatic rash, she has classic symptoms of psoriatic arthritis. She has dactylitis and distal interphalangeal swelling, as well as ankle involvement. Nail signs are well-documented in psoriasis. The diagnosis is clinched by the negative rheumatoid factor and raised inflammatory markers. A slightly low haemoglobin is also a common feature of the disease. The pattern of joint involvement points more towards psoriatic arthritis than rheumatoid arthritis, in which the metacarpophalangeal joints and wrists are more commonly affected. Reiter’s syndrome is a reactive arthritis that typically follows a gastrointestinal or venereal infection. Conjunctivitis and urethritis are seen alongside arthritis. Back pain would be expected to accompany ankylosing spondylitis. Yellow nail syndrome is a rare disorder of uncertain pathogenesis. It presents with nail discolouration, lymphoedema and pleural effusions.

Psoriatic arthritis can manifest in the absence of skin signs, particularly if the patient has a family history of psoriasis. The patient may develop a rash later or have signs limited to the nails.

18
Q

Mx of uncontrolled psoriasis in Psoriatic Arthropathy

A
  • Early instigation of biological therapy

TNF-alpha is a pro-inflammatory cytokine closely linked to the severity of psoriasis

Etanercept = TNF-alpha antagonist is therefore the most appropriate intervention

Brodalumab = anti-IL7 monoclonal antibody which has completed trials and is reserved for uncontrolled cases

NB: TB and Viral hepatitis should be ruled out prior to starting therapy

19
Q

Dactylitis

A

Describes the inflammation of a digit (finger or toe)

Causes include: 
- Spondyloarthritis eg Psoriatic and Reactive arthritis 
- Sickle Cell Disease
- Other rare causes:
TB
Sarcoidosis
Syphilis

Classical description = ‘sausage shaped’ digit

Causes = all the Ss - sausages shaped, pSoriatic, sickle cell, sarcoidosis, syphilis + TB

20
Q

Reactive Arthritis

A

= one of the HLA -B27 associated spondyloarthropathies
Encompasses Reiter’s syndrome

Reactive arthritis = defined as an arthritis that develops following an infection where the organism cannot be recovered from the joint

21
Q

Reiter’s Syndrome

A

= Classic Triad of URETHRITIS + CONJUNCTIVITIS + ARTHRITIS 2dry to a dysenteric illness during WWII. Later it was identified in patients following STI.

‘Can’t see, can’t pee, can’t climb a tree’

22
Q

Reactive Arthritis - Features and Prognosis

A

Features

  • Typically develops within 4w of initial infection and Sx generally last 4-6m
  • Arthritis is typically an asymmetrical oligoarthritis of lower limbs
  • Dactylitis
  • Sx of Urethritis
  • Eyes= Conjunctivitis (seen in 50%), anterior uveitis
  • Skin= Circinate balanitis (painless vesicles on coronal margin of foreskin)

Prognosis

  • 25% have recurrent episodes
  • 10% patients develop CKD
23
Q

Reactive Arthritis - Organisms

A

Post-dysenteric Organisms

  • Shigella Flexneri
  • Salmonella Tympimurium
  • Yersinia Enterocolitica
  • Campylobacter

Post-STI
- Chlamydia-trachomatis

NB Post STI form is more common in men (10:1)
Post-dysenteric form = equal sex incidence

24
Q

Reactive Arthritis - Mx

A

Symptomatic: Analgesia, NSAIDs, Intra-articular steroids
Sulfasalazine and Methotrexate used for persistent disease
Sx rarely last more than 12 months

25
Q

Reactive Arthritis - Features

A

Reactive arthritis: features

Reactive arthritis is one of the HLA-B27 associated seronegative spondyloarthropathies. It encompasses Reiter’s syndrome, a term which described a classic triad of urethritis, conjunctivitis and arthritis following a dysenteric illness during the Second World War. Later studies identified patients who developed symptoms following a sexually transmitted infection (post-STI, now sometimes referred to as sexually acquired reactive arthritis, SARA).

Reactive arthritis is defined as an arthritis that develops following an infection where the organism cannot be recovered from the joint.

Features
typically develops within 4 weeks of initial infection - symptoms generally last around 4-6 months
arthritis is typically an asymmetrical oligoarthritis of lower limbs
dactylitis
symptoms of urethritis
eye: conjunctivitis (seen in 50%), anterior uveitis
skin: circinate balanitis (painless vesicles on the coronal margin of the prepuce), keratoderma blenorrhagica (waxy yellow/brown papules on palms and soles)

Around 25% of patients have recurrent episodes whilst 10% of patients develop chronic disease

‘Can’t see, pee or climb a tree’

26
Q

Reactive Arthritis - Example Question

A

A 40-year-old man is stable on warfarin therapy for the treatment of atrial fibrillation. Whilst on a stag party in Spain he develops scrotal pain and itching. Whilst in Spain, he is treated with a course of ciprofloxacin for a presumed urinary tract infection. Two weeks later he develops a hot, red, swollen and painful knee and both elbows become inflamed. On examination in the emergency department you identify localised tenderness of the knee and painful movement in all directions. The knee is red and hot. Both elbows are mildly warm, with painful movement on flexion and extension. His conjunctiva are also red. He is afebrile. Examination of his external genitalia is essentially normal however there is evidence of excoriation around the scrotum.
What is the cause of his knee pain?

	> Reactive arthritis
	Still's disease
	Septic arthritis
	Gout
	Haemarthrosis

Reactive arthritis affects the joints, eyes and genitourinary/gastrointestinal system. It has been associated with gastrointestinal (GI) infections including Shigella, Salmonella, Campylobacter, and other organisms, as well as with genitourinary (GU) infections (especially with Chlamydia trachomatis). The clinical triad commonly occurs 1-4 weeks following exposure.

Ciprofloxacin interacts with warfarin causing prolonged INR which is a risk factor for haemarthrosis, but the history of conjunctivitis and GU symptoms does not support this

27
Q

Schober’s Test

A

Technique

Patient stands erect with normal Posture
Identify level of posterosuperior iliac spine
Mark midline at 5 cm below iliac spine
Mark midline at 10 cm above iliac spine
Patient bends at waist to full forward flexion
Measure distance between 2 lines (started 15 cm apart)

Interpretation

Normal: distance between 2 lines increases to >20 cm
Abnormal: distance does not increase to >20 cm
Suggests decreased Lumbar Spine range of motion
May suggest Ankylosing Spondylitis

28
Q

Psoriatic arthritis vs RA vs Reactive - Pattern of joint involvement

A

PA: DIP joints of fingers

RA: MCP and PCP joints and wrists are more commonly affected.

Reactive: Arthritis is typically an asymmetrical oligoarthritis of lower limbs

29
Q

Seronegative Spondyloarthropathies - Common Fx

A
  • Assoc w HLA-B27
  • Rheumatoid factor negative
  • Peripheral arthritis usually asymmetrical
  • Sacroilitis
  • Enthesopathy = Disorder involving attachment of a tendon or ligament to a bone e.g. Achilles tendonitis, Plantar fasciitis
  • Extra-Articular manifestations:
    > Uveitis
    > Pulmonary fibrosis (apical)
    > Amyloidosis
    > Aortic Regurgitation
30
Q

The Spondyloarthropathies

A

Ankylosing spondylitis
Psoriatic Arthritis
Reiter’s Syndrome inc reactive arthritis
Enteropathic Arthritis (assoc with IBD)