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PMR & GCA

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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. - PowerPoint PPT Presentation
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PMR & GCA Janet Pope Professor of Medicine Division of Rheumatology University of Western Ontario Polymyalgia Rheumatica (PMR) Giant-Cell Arteritis (GCA) which is also called: Temporal Arteritis (TA)
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Page 1: PMR & GCA

PMR & GCA

Janet PopeProfessor of Medicine

Division of RheumatologyUniversity of Western Ontario

Polymyalgia Rheumatica (PMR)

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

Page 2: PMR & GCA

Objectives

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

2. Describe the underlying pathophysiology of PMR and GCS.

3. Discuss risk factors, clinical features and treatment.

4. Discuss the prognosis of PMR & GCA

Page 3: PMR & GCA

Polymyalgia Rheumatica

• Poly = many

• Myalgia = sore muscles

• Rheumatica = something to do with rheumatism

Page 4: PMR & GCA

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

Page 5: PMR & GCA

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?

Page 6: PMR & GCA

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

Page 7: PMR & GCA

Case

• She was worried that she had cancer or something dreadful

• In March she went back to see you

Page 8: PMR & GCA

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?

Page 9: PMR & GCA

Case

• CBC, ESR (CRP maybe)

• TSH

• Glucose

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

• Maybe RF, ANA

Page 10: PMR & GCA

Labs

• WBC 6.4• Hbg 103• Plt 489• ESR 73• CRP 65• RF and ANA were both negative• Normal serum protein electrophoresis

Page 11: PMR & GCA

What is the most likely diagnosis?

Page 12: PMR & GCA

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)

Page 13: PMR & GCA

Polymyalgia Rheumatica

Page 14: PMR & GCA

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

Page 15: PMR & GCA

How would you treat her?

• Write the prescription

Page 16: PMR & GCA

Polymyalgia Rheumatica

• It is Exquisitively Sensitive to Corticosteroids like prednisone

Page 17: PMR & GCA

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?

Page 18: PMR & GCA

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?

Page 19: PMR & GCA

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)

Page 20: PMR & GCA

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

Page 21: PMR & GCA

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

Page 22: PMR & GCA

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

Page 23: PMR & GCA

• How often is PMR of sudden onset?

• What proportion of PMR develops GCA?

• What would you warn her about?

Page 24: PMR & GCA

• 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

Page 25: PMR & GCA

Case 2

• 77 year old woman

• HTN on HCT

• Borderline hyperlipidemia, treated by diet

• Hysterectomy at age 45 for metromenorrhagia

Page 26: PMR & GCA

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

Page 27: PMR & GCA

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

Page 28: PMR & GCA

Case 2

• What is the most likely diagnosis?

• How do you treat immediately?

• What investigations would you do?

Page 29: PMR & GCA

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?

Page 30: PMR & GCA

Case 2: labs

• Complete Blood Count (CBC)• Erythrocyte Sedimentation Rate (ESR)• C-Reactive Protein (CRP)• Glucose, creatinine, AST, ALT

Page 31: PMR & GCA

Case 2: labs

• Hbg 99 • WBC normal• Platelets normal• ESR 130 mm/hr• CRP 120

Page 32: PMR & GCA

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?

Page 33: PMR & GCA

Temporal artery biopsy

Page 34: PMR & GCA

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

Page 35: PMR & GCA
Page 36: PMR & GCA

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

Page 37: PMR & GCA

Temporal (Giant-Cell) Arteritis

• Physical Examination– Very tender over temples (common)– Swollen, rope like temporal artery (rare)– Optic disc swelling due to ischemia

Page 38: PMR & GCA

Temporal (Giant-Cell) Arteritis

Page 39: PMR & GCA

Vision Loss

Page 40: PMR & GCA

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

Page 41: PMR & GCA

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

Page 42: PMR & GCA

Temporal Artery Biopsy

Page 43: PMR & GCA

Temporal Artery Biopsy

Inflammation

Multi-Nucleated Giant Cell

Page 44: PMR & GCA

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

Page 45: PMR & GCA

Disruption of internal elastic lamina

Page 46: PMR & GCA

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?

Page 47: PMR & GCA

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

Page 48: PMR & GCA

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)

Page 49: PMR & GCA

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

Page 50: PMR & 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

Page 51: PMR & GCA

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?

Page 52: PMR & 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

Page 53: PMR & 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?

Page 54: PMR & 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

Page 55: PMR & 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

Page 56: PMR & GCA

GCA

• Jaw claudication• Scalp tenderness• Anemia• Fever• Weight loss• Visual change• Swollen temporal arteries• Pulseless temporal arteries• Tender temporal arteries• A positive biopsy

Page 57: PMR & GCA

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%

Page 58: PMR & GCA

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

Page 59: PMR & GCA

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