Documenti di Didattica
Documenti di Professioni
Documenti di Cultura
Janet Pope
Professor of Medicine
Division of Rheumatology
University of Western Ontario
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
Polymyalgia Rheumatica
• Poly = many
• Myalgia = sore muscles
• Rheumatica = something to do with
rheumatism
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
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
• Temporal arteritis symptoms
– HA, scalp tenderness, visual problems, jaw
claudication, tongue pain, weight loss, fever
Fracture history
Diabetes
Other medical problems
Case
• She was worried that she had cancer or
something dreadful
• In March she went back to see you
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?
Case
• CBC, ESR (CRP maybe)
• TSH
• Glucose
• Creatinine, liver enzymes, (maybe: lytes,
CK)
• Maybe RF, ANA
Labs
• WBC 6.4
• Hbg 103
• Plt 489
• ESR 73
• CRP 65
• RF and ANA were both negative
• Normal serum protein electrophoresis
What is the most likely
diagnosis?
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 Rheumatica
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
Inflammation
Multi-Nucleated
Giant Cell
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
Disruption of internal elastic
lamina
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 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
• 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
• 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
• 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 GCA
• Most significant complications include:
– Visual loss
– Cerebrovascular accident (stroke)
• Mortality is due to vascular complications
relating to inflammation