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Polymyalgia Rheumatica (PMR) Giant-Cell Arteritis (GCA) which is also called: Temporal Arteritis (TA). PMR & GCA. Janet Pope Professor of Medicine Division of Rheumatology University of Western Ontario. Objectives.

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Pmr gca

Polymyalgia Rheumatica (PMR)

Giant-Cell Arteritis (GCA) which is also called:

Temporal Arteritis (TA)


Janet Pope

Professor of Medicine

Division of Rheumatology

University of Western Ontario


  • Define polymyalgia rheumatica (PMR) & giant cell arteritis or temporal arteritis (GCA or TA).

  • Describe the underlying pathophysiology of PMR and GCS.

  • Discuss risk factors, clinical features and treatment.

  • Discuss the prognosis of PMR & GCA

Polymyalgia rheumatica
Polymyalgia Rheumatica

  • Poly = many

  • Myalgia = sore muscles

  • Rheumatica = something to do with rheumatism


  • 70 year old woman, previously healthy

  • She has an active lifestyle and is taking only a multivitamin

  • January – she began to notice pain and stiffness in her shoulders

  • She was told it was just “old age” by her family doctor and a friend told her it was probably “rheumatism” caused by the cold weather

  • By February the pain and stiffness had become worse


  • The pain and stiffness was around her hips and low back and into her shoulders and neck

  • She complained of fatigue which was new

  • She was very stiff in the morning taking hours if not all day to get going

  • She had lost her appetite and lost 4Kg

  • What else do you want to know or ask?

Ask pmr patient about
Ask PMR patient about

  • Temporal arteritis symptoms

    • HA, scalp tenderness, visual problems, jaw claudication, tongue pain, weight loss, fever

      Fracture history


      Other medical problems


  • She was worried that she had cancer or something dreadful

  • In March she went back to see you

Case 1 p e
Case 1 – P/E

  • Afebrile, HR 88, BP 160/70, in NAD

  • General exam – normal

  • MSK exam normal ROM of joints,

    • no swelling, pain on ROM of hips and shoulders

  • What investigations would you do?


  • CBC, ESR (CRP maybe)

  • TSH

  • Glucose

  • Creatinine, liver enzymes, (maybe: lytes, CK)

  • Maybe RF, ANA


  • WBC 6.4

  • Hbg 103

  • Plt 489

  • ESR 73

  • CRP 65

  • RF and ANA were both negative

  • Normal serum protein electrophoresis


  • Older woman (> 50, often far older)

    • Increases with age

    • It does not occur in the young

  • Pain & stiffness in the hips and shoulders

  • Profound morning stiffness

  • Insidious onset (half are sudden onset)

  • Associated Fatigue

  • Weight Loss in less than half

  • Inflammatory Markers (ESR, CRP, could also have anemia, thrombocytosis)

Polymyalgia rheumatica2
Polymyalgia Rheumatica

  • There is NOTHING wrong with the muscles

  • Proximal achiness is from the joints

  • We see inflammation of joints and peri-articular structures such as bursae

  • PMR is a history of shoulder and hip girdle symmetrical stiffness, no findings usually on exam and a high ESR

How would you treat her
How would you treat her?

  • Write the prescription

Polymyalgia rheumatica3
Polymyalgia Rheumatica

  • It is Exquisitively Sensitive to Corticosteroids like prednisone


  • 15 or 20 mg of prednisone per day

  • Report back in a couple of days

  • There should be nearly 100% improvement in 72 hours or less

  • In 12 hours she felt almost completely normal and couldn’t believe it

  • How long do we usually need to treat it?

Duration of treatment for pmr
Duration of Treatment for PMR

  • Usual treatment (median) is 2 years

  • Decrease prednisone to lowest dose that controls the symptoms

  • Do we need to follow the ESR?

  • What other treatment may you want to add?

Pmr initial treatment
PMR: Initial Treatment

  • Prednisone 15-20 mg per day

  • The patient must be virtually back to normal of you don’t have the correct diagnosis

  • Slowly wean down the steroids (example after a few weeks) and keep decreasing as symptoms are controlled

  • Often after 10 mg, patients need to reduce more slowly (ex 1mg every month or two)

Pmr steroid weaning
PMR: Steroid Weaning

  • Prednisone 15 mg x 1 month

  • Prednisone 12.5 mg x 1 month

  • Prednisone 10 mg x 1 month

  • Below 10 mg I reduce by 1 mg per month

  • TOTAL: 16 – 18 months

Prevention of corticosteroid induced op
Prevention of corticosteroid induced OP

  • Steroids can “thin” the bones and anyone you expect to take >7.5 mg of prednisone for >3 months should be protected

  • Calcium: 1500 mg calcium per day

  • Vitamin D: 1000 to 3000 IU per day

  • Bisphosphonate

  • Likely order a baseline BMD if one has not been done over the last couple of years

Prognosis of pmr
Prognosis of PMR

  • Average duration of corticosteroid use is about 2 years

  • A sub-group of patients will require low-dose prednisone for much longer

  • Occasionally Rheumatoid arthritis can present very much like PMR

  • If we can’t reduce prednisone or if patient has DM or severe OP, then Mtx can be used as a steroid sparing drug

  • How often is PMR of sudden onset?

    • 50%

  • What proportion of PMR develops GCA?

    • 10%

  • What would you warn her about?

    • Visual changes – decreased colour vision, missing part of visual field, TIA

    • Get seen ASAP if this occurs

    • Side effects of prednisone

Case 2
Case 2

  • 77 year old woman

  • HTN on HCT

  • Borderline hyperlipidemia, treated by diet

  • Hysterectomy at age 45 for metromenorrhagia

Case 21
Case 2

  • She noticed the onset of right sided headaches, new and constant like a vice grip over her entire head

  • These were associated with pain over the temple and it actually hurt to brush her hair

Case 22
Case 2

  • She also was quite fatigued and had noticed low grade fevers

  • No visual symptoms (i.e. loss of vision) or no symptoms of stroke (cerebrovascular accident)

  • No PMR symptoms

Case 23
Case 2

  • What is the most likely diagnosis?

  • How do you treat immediately?

  • What investigations would you do?

Case 24
Case 2

  • Prednisone 50 to 60 mg per day

  • Started on low dose ASA

  • Sent immediately to the lab

  • To return to the office the next day for preliminary results

  • What would you order?

Case 2 labs
Case 2: labs

  • Complete Blood Count (CBC)

  • Erythrocyte Sedimentation Rate (ESR)

  • C-Reactive Protein (CRP)

  • Glucose, creatinine, AST, ALT

Case 2 labs1
Case 2: labs

  • Hbg 99

  • WBC normal

  • Platelets normal

  • ESR 130 mm/hr

  • CRP 120

The next day
The Next Day

  • The patient is feeling a bit better

  • She is referred urgently to a rheumatologist (you phone and ask for apt ASAP) or general medicine

  • What else should be done?


  • High dose steroids until ESR normalizes

  • Ischemic pain may take a long time to improve

  • There is a risk of visual loss or stroke so taper is usually according to the inflammatory markers

  • ASA especially is visual changes

  • Prevention of ulcer (PPI) with ASA

  • Rx or prevention of OP with bisphoshonate

  • Steroid sparing drugs

    • There are data with Mtx to reduce overall amount of prednisone needed

  • Treatment is often for 1.5 to 3 years but in some it may last forever

  • 50% of GCA have PMR, but10% of PMR have GCA

Temporal giant cell arteritis
Temporal (Giant-Cell) Arteritis

  • Chronic granulomatous vasculitis affecting large arteries in older people

  • Most are >60 years of age (average 72)

  • Inflammation of the walls of large arteries

    • Cranial arteritis (most common): Temporal, occipital, ophthalmic

    • Subclavian, iliac/femoral

    • Aorta

Temporal giant cell arteritis1
Temporal (Giant-Cell) Arteritis

  • Physical Examination

    • Very tender over temples (common)

    • Swollen, rope like temporal artery (rare)

    • Optic disc swelling due to ischemia

Vision loss1
Vision Loss

  • Transient repeated episodes of diminished vision are usually reversible.

  • Sudden loss of vision is an ominous sign and is almost always permanent.

  • If loss of vision in one eye, patient has a high risk of losing vision in other eye

  • EMERGENCY: give iv 100mg solumedrol and make sure pt is taking ASA

Temporal giant cell arteritis3
Temporal (Giant-Cell) Arteritis

  • Investigations

    • Complete Blood Count (CBC)

      • Normochromic, normocytic anemia

      • Reactive thrombocytosis

      • WBC is usually normal

    • Erythrocyte Sedimentation Rate (ESR)

      • Significantly elevated

    • C-Reactive Protein (CRP)

      • Significantly elevated

Temporal artery biopsy2
Temporal Artery Biopsy


Multi-Nucleated Giant Cell

Gca pathology
GCA pathology

  • There is a ring of granulomanous inflammation centered around the elastic lamellae within and bounding the media.

  • Disruption of internal elastic lamina

  • multinucleated giant cells

  • intimal thickening and fibrosis as well as the central acute thrombus.

  • nonspecific inflammatory infiltrate in the adventitia

  • Fibrinoid necrosis

Gca biopsy
GCA biopsy

  • If you treat prior to biopsy, what would be the chance of having a positive biopsy in one week,

  • How about in one month?

  • What does the classic biopsy show?

Gca biopsy1
GCA biopsy

  • If you treat prior to biopsy, what would be the chance of having a positive biopsy in one week, highly likely

  • How about in one month? Still can be positive but partially healed, try not to wait more than 2 weeks but do not with hold treatment if suspicion is high (40% + when Rx with pred for >1 month

  • What does the classic biopsy show?

  • Disruption of internal elastic lamina, inflammation, maybe giant cells

Gca initial treatment
GCA: Initial Treatment

  • Prednisone 50-60 mg per day (1mg/kg/d)

  • I start at 50-60 mg per day and hold on that dose until

    • The patient is feeling well

    • The inflammatory markers have normalized

  • I then begin to slowly wean down the steroids (usually after a month or two)

Gca steroid weaning
GCA: Steroid Weaning

  • Prednisone 50 mg x 1-2 month

  • Then reduce by 5 mg every 2 weeks until @ 20 mg (3 months)

  • Then reduce by 2.5 mg every 4 weeks until at 10 mg (4 months)

  • Then reduce by 1 mg every month (10 months)

  • TOTAL: 18+ months

Prognosis of gca
Prognosis of GCA

  • Average duration of corticosteroids is 2.4 years

  • A sub-group of patients who will have smoldering disease activity for much longer (7-10 years)

  • Thoracic aneurysms can appear up to 15 years after initial diagnosis

Prognosis of gca1
Prognosis of GCA

  • Most significant complications include:

    • Visual loss

    • Cerebrovascular accident (stroke)

  • Mortality is due to vascular complications relating to inflammation

  • What should you ask if you suspect GCA?

Case 2 gca
Case 2 - GCA

  • PMR, other neurologic symptoms, duration of visual change, location of HA, jaw claudication, weight loss, fever, scalp tenderness, illness prior

  • Other health issues: CAD, HTN, smoking, etc

Mortality in gca
Mortality in GCA

  • Is mortality increased?

  • What proportion of GCA has PMR?

  • Is there a role for a steroid sparing drug in the treatment?

  • What would you follow?

  • How long is the median treatment of PMR and or GCA?

  • Is mortality increased? yes

  • What proportion of GCA has PMR? 50%

  • Is there a role for a steroid sparing drug in the treatment? maybe

  • What would you follow? ESR

  • How long is the median treatment of PMR and or GCA?

    2+ years with a wide range

Seasonal variation of gca
Seasonal Variation of GCA

  • Chart review of GCA (bx proven and or met ACR criteria) between 1980-2004, N=206

  • Peaks in May – June in Jerusalem

  • 3 peaks over 20 yrs but no increase in incidence

  • 1.4 women to 1 man

  • 11.3 per 100,000 incidence over 25 years

  • Seasonal changes and ocassional peaks suggest an infectious or other environmental agent but none identified

Breuer #1927


  • Jaw claudication

  • Scalp tenderness

  • Anemia

  • Fever

  • Weight loss

  • Visual change

  • Swollen temporal arteries

  • Pulseless temporal arteries

  • Tender temporal arteries

  • A positive biopsy


  • Jaw claudication - uncommon

  • Scalp tenderness - common

  • Anemia - common

  • Fever – 25%

  • Weight loss – 25%

  • Visual change – less than 10%

  • Swollen temporal arteries – totally rare

  • Pulseless temporal arteries - uncommon

  • Tender temporal arteries - common

  • A positive biopsy – depends on bx size and time after treatment – 75%


  • PMR is common and patients should be virtually 100% better at most 72 hrs after starting prednisone (ex 15mg a day)

  • Usual treatment is for a couple of years

  • Use the lowest dose possible to control symptoms

  • Temporal arteritis is an emergency

  • Usually referral to a specialist after starting prednisone 50mg a day and ASA if diagnosis is strongly suspected