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

.

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

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

• Irritable bowel syndrome• Chronic fatigue syndrome• Migraine headache syndrome• Sleep disorder• Altered cognitive function• TM problems

Differential diagnosis

• 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)

• 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 ARTERITISPresentation

• 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 3-4/522) 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

• Don’t forget long term risks of steroid treatment especially if used for 12-24+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

• 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.