Polymyalgia Rheumatica and
Giant Cell Arteritis
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.
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
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
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Source: “The Manual Tender Point Survey”, D. Sinclair, T. Starz, D. Turk Univ. of Pittsburgh,
Tender points
• Usually described with a number of kilos of
pressure
• In reality no real guidelines
• 11 of 18 tender points
• Fraught with controversy
: Comorbid conditions
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Irritable bowel syndrome
Chronic fatigue syndrome
Migraine headache syndrome
Sleep disorder
Altered cognitive function
TM problems
Differential diagnosis
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Polymyalgia rheumatica
Dermato or polymyositis
Hypothyroidism
Rheumatoid arthritis
Lyme disease
Toxic myopathies secondary to statin exposure
or fibric acid
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.
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.
Bird Criteria (3 or more)
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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
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.
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.
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.
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.
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
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.
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!
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
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.
GIANT CELL ARTERITIS
Presentation
• Headache
• Scalp tenderness
• Thickened temporal
arteries
• Jaw claudication
• Acute visual loss
• Weight loss, anorexia,
fever, night sweats,
malaise & depression
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
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.
Management of PMR and GCA
1) 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
Management of PMR and GCA
3) GCA with visual disturbance
- Medical emergency 60mg prednisolone urgent
admission to ophthalmologists
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
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.
Considerations
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Don’t forget long term risks of steroid
treatment especially if used for 1224+months
Frequent BP and bloods should be offered
Osteoporos is important consideration
Encourage increased activity
Summary
• PMR – pain+stiffness proximal muscles GCA
vasculitis – headaches/scalp tenderness
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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.