Week 2 - F - Polymalgia rheumatica and large vessel vasculitis - giant cell arterititis and takayasu's arteritis

Question 1

Polymyalgia rheumatica and giant cell arteritis share the same demographic characteristics, and although separate conditions, the two frequently occur together What age group is rarely affected by the two condiitons?

Answer 1

GCA and PMR rarely affect individuals below 50 years old

They are most common in those aged 60 and above

Question 2

What percentage of patients with PMR develop GCA? What percentage of patients with GCA have an associated PMR?

Answer 2

15% of patients with PMR develop GCA

40-50% of patients with GCA will have an associated PMR

Question 3

What are the presenting features of polymyalgia rheumatica? How do symptoms tend to improve?

Answer 3

Presenting features are age >50 (commonly >60)

Proximal myalgia of the hip and shoulder girdles

with accompanying morning stiffness that lasts for more than one hour

Symptoms tend to improve as the day goes on and with movement.

Question 4

There is no specific diagnostic test for PMR but it is almost always associated with what raised serum markers? What helps to distinguish this disease from myositis?

Answer 4

Raised CRP and ESR / plasma viscosity

Creatine kinase levels are normal helping to distinguish from poly / dermato myositis

Question 5

What is used as the treatment of polymyalgia rheumatica? Why can this be useful as a diagnostic tool?

Answer 5

Symptoms of PMR respond very dramatically to low dose steroids (15mg oral prednisolone) and this is sometimes useful as a diagnostic tool

If there is not a response within 1 week, consider alternative diagosis

Question 6

The does of steroids should be reduced slowly over what time period in PMR?

Answer 6

Gradually reduce the dose from the starting 15mg oral prednisolone over 18 months

By the end of this period the condition will have resolved in the majority of cases.

Question 7

What is the difference in onset between myositis and polymalgia rheumatica?

Answer 7

Myositis is insidious in onset and the patient slowly has myalgia and muscle weakness of the proximal upper and lower limb muscles

PMR is quite a subacute onset where there is a noticeable change over a couple of weeks if not less, in the shoulder and hip girdles

Question 8

GCA is the most common form of systemic vasculitis in adults It is of unkown aetiology What age group occur in? What size of vessels does it affect?

Answer 8

GCA, like PMR, mainly affects patients over the age of 60 (under 50 is rare)

It is a large vessel vasculitis

Question 9

What are the presenting symptoms of giant cell arteritis?

Answer 9

Presenting symptoms of GCA

Headache - temporal or occipital regions

Thickened temporal artery

Scalp tenderness - eg when combing hair

Jaw claudication

Visual disturbances

Constitutional symptoms -fever, maliase, fatigue

Question 10

What percentages of patients have visual symptoms over the course of the disease in GCA?

Answer 10

Around 50% of patients with GCA experience visual symptoms over the course of the disease

Question 11

What visual symptoms may be seen in GCA?

Answer 11

Visual symptoms seen in GCA include

* Unilateral sudden visual blurring or vision loss - usually painless or occasionally diplopia

* Alternatively a partial field defect may progress to complete blindness over a few days

Question 12

What is the medical condition in GCA involving loss of vision caused by damage to the optic nerve as a result of insufficient blood supply known as? What artery is affected here?

Answer 12

The medical condition is known as Arteritic Anterior Ischaemic Optic Neuropathy

It is due to the ophthalmic artery being inflamed preventing the central retinal artery from oxygenating the anterior portion of the optic nerve

Question 13

What may be seen on fundoscopy in a patient with GCA? - due to the arteritic anterior ischaemic optic neuropathy

Answer 13

Fundoscopic examination in a patient with ischaemia of the optic nerve may reveal pallor and oedema of the optic disc (optic disc pallor and swelling)

Question 14

What is the jaw claudication in giant cell arteritis dye to?

Answer 14

Jaw claudication is due to ischaemia of the maxillary artery (one of the two terminal branches of the ECA)

* Ishcaemia is due to inflammation of the artery and therefore when you try to chew, pain happens as no perfusion to the muscles

Question 15

What is the definitive test in temporal arteriits? Should treatment be delayed for this test to be carried out and why?

Answer 15

Definitive test in temporal arteritis is a temporal artery biopsy and should be carried out as soon as possible - within 2 weeks

Treatment should not be delayed due to the risk of blindness

Question 16

A positive temporal artery biopsy has 100% specificity but relatively low sensitivity (15-40%) for the diagnosis of GCA Why may patients with GCA have a negative test on a temporal artery biopsy? What area can be sampled should the biopsy come back negative?

Answer 16

Patients with GCA may have a negative biopsy test due to the presence of skip lesions - there is patchy involvement of the artery meaning some segments may come back normal

Occipital artery or contralateral temporal artery can also be sampled if the temporal artery biopsy comes back negative

Question 17

Why is temporal arteritis also known as giant cell arteritis?

Answer 17

The majority of cases affect the temporal artery however other arteries can (and can only) be affected

Typical biopsy findings include mononuclear infiltration or granulomatous inflammation using with the presence of giant cells

Question 18

How is giant cell arteritis treated? Over what time period is treatment usually tapered down?

Answer 18

The mainstay of therapy is corticosteroids,

* Usually prednisolone 40mg if no visual impairment

* Usually 60mg if visual symptoms.

Treatment with steroids should be started as soon as the diagnosis is suspected. Do not delay for biopsy, The prednisolone dose is gradually tapered over around 2 years. For the majority of patients the condition will resolve over this period.

Provide bone protection when giving long term corticosteroids - calcium, vitamin D, maybe biphosphonates

Question 19

What are the two main categories of large vessel vasculitis and what age groups are affected?

Answer 19

Giant cell arteritis - as discussed affects individuals >50

Takayasu arteritis - affects individuals <50

Question 20

Which race is more commonly affected by Takayasu’s arteritis? What are both GCA and TA characterised by on histology?

Answer 20

Takayasu’s arteritis predominantly affects East Asian countries ie Japan

Both conditions are characterised by granulomatous infiltration of the walls of the large vessels

Question 21

Early presenting features of both GCA and TA are non specific * Fever * Malaise * Night sweats * Weight loss * Arthralgia * Fatigue What is the main large vessel affected in TA? What can happen if the condition is left untreated?

Answer 21

The main large vessel affected is the aorta and its major branches

If left untreated, the granulomatous inflammation of these vessels leads to stenosis, thrombosis and aneursym formation and therefore symptoms depending on which arteries are involved - causes bruits, reduced pulses and potentially stroke

Question 22

What are some of the more specific presenting features of Takayasu’s arteritis?

Answer 22

Following the non -specific features patients can experience claudication symptoms in both the upper and lower limbs in TA

Also they may experience dizziness and visual blurring if the cerebral and ophthalmic vessels are affected

Question 23

What tests are done to diagnose takayasu arteritis? (similar to GCA)

Answer 23

ESR, PV and CRP and elevated.

Imaging such as MR angiography can detect thickened vessel walls and stenosis.

Question 24

What is the treatment of TA? What is given in resistance cases?

Answer 24

The mainstay of treatment is corticosteroids, starting at 40-60mg prednisolone and gradually reducing.

Steroid sparing agents such as methotrexate and azathioprine may be added in resistant cases .

Question 25

A 63 year old man presents with onset of pain and stiffness around his shoulders and hips. Ongoing for around 3 weeks. He now needs help to get out of bed in the morning. What else would you like to ask about? What examination would you perform? What tests would you like to do? What does he have?

Answer 25

Ask if there is any morning stiffness

Test muscle strength to rule out polymositis - confrontational testing (resist force on outstretched arms) and isotonic testing (sit to stand)

Test inflammatory markers - ESR, CRP, PV

Test his Creatine kinase - rule out polymyosiits, also measure anti-Jo1 and anti-SRP

He has polymyalgia rheumatica

Question 26

A 64 year old man has been noticing a heavy feeling in his arms for about 6 months. He is finding it increasingly tiring climbing the stairs. What else would you like to ask? What examination would you perform? What tests would be helpful? What does he have?

Answer 26

Any redness on body - dermatomyositis (V-shaped rash, shawl sign, gottron’s papules, heliotrope rash)

Perform isotonic testing and confrontational testing - looks for muscle weakness - should be positive

Test his Antibodies - Anti ANA, Anti Jo-1 and Anti SRP

Also measure creatine kinase - should be raised He has polymyositis based on the current history

Question 27

A 26 year old woman is referred with a 2 year history of worsening pain “all over”. She has difficulty performing simple tasks like house work due to fatigue. She finds sleep unrefreshing. What other questions would you ask? What examination findings would you expect? What tests would you do? What is the diagnoses?

Answer 27

Any bowel problems

Any depression / anxiety

Any headaches

Would find widespread muscle pain

Test her inflammatory markers - should all be negative

Fibromyalgia - manage with antidepressants and pain killers