PMR/Temporal arteritis

Question 1

Definition PMR

Answer 1

Inflammatory disorder that causes muscle pain and stiffness, especially in the shoulders and hips

The condition is related to another inflammatory condition called giant cell arteries (temporal arteritis)- inflammation of the lining of your arteries

Question 2

Aetiology PMR

Answer 2

The exact cause is unknown. 2 factors appeared to be involved in the development of the condition though:
– genetics- certain genes and gene varieties might increase your susceptibility
– an environmental exposure- new cases of poly myalgia rheumatic tend to come in cycles, possible developing seasonally. This suggests that an environmental tigger, such as virus’ might play a role. But no specific virus has been shown to cause PMR

Question 3

Epidemiology PMR

Answer 3

Most people are older than 65

Rarely affects people under the age of 50

More common in women

More common in caucasians

Question 4

Pathophysiology PMR

Question 5

Clinical presentation PMR

Answer 5

Symptoms occur in both sides of the body and might include:
– aches or pain in shoulders
– aches or pain in your neck, upper arms, buttocks, hips or thighs
– stiffness in affected areas, particularly in the morning or after being inactive for a time
– limited range of motion in affected areas
– pain or stiffness in wrists, elbows or knees
– pain worse with movement
– interferes with sleep
– stiffness in the morning, lasting longer than 30 minutes

1/3 of patients develop systemic features of:
– mild fever
– fatigue
– a general feeling of not being well (malaise)
– loss of appetite
– unintended weight loss
– depression
– pitting oedema

Question 6

Investigations PMR

Answer 6

Blood tests:
– raised inflammatory markers

Imaging tests:
– ultrasound is being used to differentiate PMR from other conditions that cause similar symptoms
– MRI can also identify other intraarticular causes of shoulder pain, such as joint changes

Additional investigations:
– ANA test- tests for systemic lupus erythematosus

– anti CCP- test for RA

Question 7

Treatment PMR

Answer 7

Streroids:
– both PMR and GCA respond very well to treatment with steroids
—- initially 15mg prednisolone
—– if there is a poor response in a week, then it is probably not PMR
—– within 3-4 weeks- there should be 70% improvement, inflammatory markers back to normal- this will allow a working diagnosis of PMR

Long term use of steroids:
– weight gain
– increased blood pressure
– loss of bone density
– diabetes
– cataracts

Physical therapy:
– most people who take steroids for PMR return to their previous levels of activity. However, if you’ve had a long stretch of limited activity, you might benefit from physical therapy

Question 8

Temporal arteritis Definition

Answer 8

An inflammatory granulomatous arteritis of large cerebral arteries which occurs in association with PMR.

Question 9

Aetiology TA

Question 10

Epidemiology TA

Answer 10

Elderly population over 50

Extremely rare under the age of 50

Higher likelihood of females getting temporal arteritis

50% of cases will have both PMR and TA

Question 11

Pathophysiology TA

Answer 11

Presence of giant cells in the artery.

Inflammation of all the vessel layers in the artery

Presence of CD4+ and CD8+ lymphocytes and also macrophages
– these contribute to the inflammatory process seen in GCA

– all these are usually found in the smooth muscle layer in GCA- causing smooth muscle hypertrophy

inflammation occurs in segments along the artery- occurs in skip lesions

When there is any damage to the endothelium, haemostasis occurs (thrombosis or fibrin mesh)

There is no fibrinoid necorsis that occurs, due to that mostly occurring within small blood vessels

Question 12

Clinical presentation TA

Answer 12

Headaches

Scalp tenderness (particularly over temple)

Jaw claudication when eating or using jaw
– if the facial artery is affected

Tongue claudication

Combing hair may be painful

Involvement of ophthalmic artery can cause blindness
– this is why early recognition and treatment is essential

Amaurosis fujax (vision loss)

Question 13

Investigations TA

Answer 13

Blood test:
– raised inflammatory markers

Ultrasound of temporal arteries

Temporal artery biopsy

Diagnosis should not slow down the treatment due to the risks it poses

Question 14

Treatment TA

Answer 14

Reacts well to steroids

Prednisolone 60mg a day for GCA or TA

Disease may relapse when the steroid treatment is stopped

Question 15

Relationship between GCA and TA

Answer 15

May be due to HLA-DRB1 allele

Frequently occur together

PMR in 40-60% of patients with GCA