1. Polymyalgia Rheumatica and Giant Cell Arteritis

2. Definition of Fibromyalga
“Chronic and widespread pain located at 11 or more of 18 tender points”-ACR definition
No specific diagnostic test and no cure.

3. Clinical manifestations
Chronic and persistent pain with varying manifestations
Diffuse musculoskeletal pain, stiffness and fatigue
Joints not swollen painful or red
Sensation such as tingling, numbness, burning, crawling are often described
Pain is aggravated by change in weather, stress, fatigue, lack of sleep et cetera et cetera

4. Epidemiology 2% of all US citizens affected: 5.5 million
Most common cause of generalized pain in women ages 20 to 50
Prevalence increases with age: 2% ages 20 to 35, with 8% at age 70
Females 10 times more common than males

5.                                    .
                                  
Source: “The Manual Tender Point Survey”, D. Sinclair, T. Starz, D. Turk Univ. of Pittsburgh, School of Medicine

6. Tender points Usually described with a number of kilos of pressure
In reality no real guidelines
11 of 18 tender points
Fraught with controversy

7. : Comorbid conditions Irritable bowel syndrome
Chronic fatigue syndrome
Migraine headache syndrome
Sleep disorder
Altered cognitive function
TM problems

8. Differential diagnosis
Polymyalgia rheumatica
Dermato or polymyositis
Hypothyroidism
Rheumatoid arthritis
Lyme disease
Toxic myopathies secondary to statin exposure or fibric acid

9. Definitions
Polymyalgia Rheumatica (PMR) – Inflammatory condition of unknown etiology.
Giant Cell Arteritis (GCA) – Vasculitis of medium or large arteries of unknown cause
They often occur together so are suspected to have the same underlying pathophysiology.

10. PMR
Diagnosis is difficult and uncertain as the symptoms are non-specific. As such incidence is hard to predict.
Shoulder and/or pelvic girdle pain with early morning stiffness forms the polymyalgic syndrome.
There is no diagnostic test and is in many ways a diagnosis of exclusion.

11. Bird Criteria (3 or more)
Bilateral shoulder pain or stiffness
Onset of illness less than 2 weeks
Initial ESR >40
Morning stiffness >1hr
Age 65 or older
Depression and/or weight loss
Bilateral tenderness in upper arms

12. PMR Take care not to miss other causes of the symptoms
PMR less likely to be the cause if: incomplete response to steroids, muscular weakness, prominent peripheral joint symptoms, lack of morning stiffness, unilateral symptoms.
ESR/CRP are usually raised, normochromic normocytic anaemia is common, LFT’s esp AlkP may be abnormal.

13. PMR It’s complications are debility due to pain. 20% will develop GCA
Response to steroid is rapid though some patients are hard to wean off steroids.

14. GCA 3 times more common in females Also known as temporal arteritis
Affects cranial branches of arteries from arch of aorta and branches of ophthalmic artery.
Associated with critical ischaemia : stroke blindness.
These can occur very early in the disease.

15. GCA
Rare if <50y old
Unilateral headache most often in temporal region.
Scalp tenderness classically noticed while brushing hair.
Pain on chewing – claudication of muscles of mastication
Fever, weight loss, fatigue, anorexia.

16. GCA
Double vision or amaurosis fugax (transient painless loss of monocular vision of variable duration)
Can have reduced visual acuity and/or visual field defects.
Fundoscopy – oedema/pallor of optic disc small retinal haemorrhages may also be seen

17. GCA
Include general CV examination – check BP both arms as aortic dissection can occur.
Check peripheral pulses and listen for carotid bruits.
The American College Of Rheumatology (ACR) is a helpful diagnostic criteria.

18. ACR Criteria for GCA (three or more needed)
Age 50 or greater
New type of headache
Clinically abnormal temporal artery (thickened, tender or nodular decreased pulsation)
ESR 50 or >
Abnormal artery biopsy – necrotizing arteritis with mononuclear infiltrate or granulomatous infiltrate usually with multinucleated giant cells!

19. GCA
Inflammatory markers are not always raised do not withhold treatment if strong clinical suspicion.
As PMR normochromic normocytic anaemia is common as are abnormal LFT’s
Use of temporal artery biopsy is debated - + confirms GCA but negative does not exclude due to skip lesions

20. GCA Large artery complications common
Aortic aneurysm or dissection occurs in 18%!!!
Patients with GCA have a higher risk of stroke/MI/CHF.
A variety of neurological complications can also ensue
Early adequate dose steroids prevent most complications.

21. GIANT CELL ARTERITIS Presentation
Headache
Scalp tenderness
Thickened temporal arteries
Jaw claudication
Acute visual loss
Weight loss, anorexia, fever, night sweats, malaise & depression

22. GIANT CELL ARTERITIS Temporal Artery Biopsy
Arteries have skip lesions
ultrasound/Doppler may help identify involved areas
If positive, confirms diagnosis – helpful in management of future disease
If negative, doesn’t exclude diagnosis, but need to think about an alternative diagnosis

23. Management of PMR and GCA
Follows pattern of induction, maintenance and reduction of treatment.
Most evidence for steroid dose is observational
Oral prednisolone is the most common treatment used.

24. Management of PMR and GCA
PMR no signs GCA
Prednisolone 10-20mg od (after baseline investigations)
70% improve in 1/52
Inflammatory markers improve in 3-4/52
2) GCA without visual disturbance
40-60mg prednisolone od
R/V at 48hrs sooner if visual disturbance

25. Management of PMR and GCA
3) GCA with visual disturbance - Medical emergency 60mg prednisolone urgent admission to ophthalmologists

26. Maintenance
PMR and GCA continue dose steroid for up to 4 weeks then can be decreased every 2-4weeks 5mg a time in GCA and 2.5mg in PMR.
When on 10mg decrease every 4-6weeks by 1mg
When on 7mg maintain at this for 12months before reducing further.
Steroids should only be stopped if proximal pain and morning stiffness have resolved.
Helpful to monitor CRP/ESR during above
Can relapse requiring step up in steroid again

27. Who and when to Refer?
GCA any with visual symptoms even if on treatment should be referred on that day to ophthalmology.
Other patients with GCA – routine referral to rheumatology or general medicine for shared care.
PMR can be managed in GP setting
Consider secondary care referral if doubt as to diagnosis, poor response to treatment or adverse effects with steroid treatment.

28. Considerations
Don’t forget long term risks of steroid treatment especially if used for months
Frequent BP and bloods should be offered
Osteoporos is important consideration
Encourage increased activity

29. Summary
PMR – pain+stiffness proximal muscles GCA vasculitis – headaches/scalp tenderness
GCA vasculitis – headaches/scalp tenderness
GCA can cause irreversible blindness
ESR + CRP are commonly raised in GCA +PMR
Dramatic response to corticosteroids in both
Relapse common during steroid reduction
Patients require long term steroids thus use osteoporosis prophylaxis as per local guidelines.