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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)

PMR & GCA

Janet Pope

Professor of Medicine

Division of Rheumatology

University of Western Ontario

objectives
Objectives
  • 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
slide4
Case
  • 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
slide5
Case
  • 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

Diabetes

Other medical problems

slide7
Case
  • 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?
slide9
Case
  • CBC, ESR (CRP maybe)
  • TSH
  • Glucose
  • Creatinine, liver enzymes, (maybe: lytes, CK)
  • Maybe RF, ANA
slide10
Labs
  • WBC 6.4
  • Hbg 103
  • Plt 489
  • ESR 73
  • CRP 65
  • RF and ANA were both negative
  • Normal serum protein electrophoresis
slide12
PMR
  • 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
slide17
Case
  • 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
slide23
How often is PMR of sudden onset?
  • What proportion of PMR develops GCA?
  • What would you warn her about?
slide24
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?
treatment
Treatment
  • 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

Inflammation

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?
slide54
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

slide56
GCA
  • Jaw claudication
  • Scalp tenderness
  • Anemia
  • Fever
  • Weight loss
  • Visual change
  • Swollen temporal arteries
  • Pulseless temporal arteries
  • Tender temporal arteries
  • A positive biopsy
slide57
GCA
  • 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%
conclusions
Conclusions
  • 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
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