Polymyalgia and Temporal Arteritis.
Clinical syndromes which represent different ends
of a disease spectrum.
Affects elderly patients--is rare below 50.
Respond to steroids.
Classification of Symptoms.
Systemic--malaise, anorexia, fever, night sweats,
weight loss, depression.
Myalgic--proximal, symmetrical muscle pain and stiffness
in PMR.
Classification of arteritic symptoms
Pain, swelling, erythema over affected artery.
Partial occlusion resulting in claudication-like
symptoms.
Total occlusion resulting in ischaemia and necrosis
(can occur anywhere in body).
Polymyalgia Rheumatica
Stiffness in morning which may worsen again in the
evening.
May literally have to roll out of bed in morning.
Muscle strength generally preserved.
Temporal Arteritis.
Almost only seen in Caucasians.
Pain commonly around temporal artery, jaw and post
auricular artery.
Less common presentations may lead to pain in tongue,
throat and teeth.
Loss of pulsation of artery is a debatable sign.
Histology reveals giant cell arteritis in 80% of
cases.
Ophthalmic Features.
Sudden, painless deterioration of vision in one eye,
usually on waking.
Visual acuity varies from 6/6 to no light perception.
Fundus examination reveals optic disc oedema with
splinter retinal haemorrhages.
Starts as unilateral condition but may progress to
bilateral disease.
Other Manifestations of GCA.
Giant cell arteritis can affect all arteries.
One study revealed 8 out of 244 patients with new
stroke had GCA or PMR.
May lead to isolated cranial nerve palsies.
GCA can affect coronary arteries which may lead to
myocardial infarct.
Great vessels can be affected, there is an association
with aortic incompetence.
Diagnosis
PMR in particular is a diagnosis of exclusion.
History is critical. Systemic, local and visual symptoms
should be sought.
Examination should include full cardiovascular and
neurological review as well as eliciting local arteritic signs.
The examination in PMR tends to be normal.
Differential Diagnosis for PMR.
Neoplastic disease.
Cervical spondylosis.
Rheumatoid arthritis.
Connective tissue disease.
Myeloma and leukaemia.
Bone disease (osteomyelitis).
Hypothyroidism.
Miliary TB.
Differential Diagnosis of TA.
Dental conditions.
Trigeminal neuralgia.
Otological conditions.
Retinal vascular accident.
Other causes of opthalmoplegia.
Investigations
ESR/Plasma viscosity raised in 80% but very non-specific.
ALP marginally raised in 30-50%.
Other features include thrombocytosis, raised IgG
and a normocytic, normochromic anaemia.
Histology remains the gold standard.
Histology.
Biopsy of artery only justified if arteritis suspected.
A negative biopsy dose not exclude TA.
Giant cell arteritis is characterised by skip lesions.
An inch of artery should be removed, preferably from
a tender area.
Case history J.W. male d.o.b.3/10/34
Oct 91 doing building work at home
myalgia thighs & buttocks
morning stiffness to mid afternoon
difficulty walking
lethargy
Dec 91 progressively worse
shoulder girdle myalgia
unable to crouch/elevate arms
night sweats
no energy
no headaches
normal temporal arteries
no muscle tenderness
moderate restriction shoulders & hips
ESR 88
prednisolone 15 mgs/day
better in 48 hrs
ESR 14 within 2 weeks
Dec 92: well on prednisolone 5 mg/day
Jan 93: altered bowel habit rectal bleeding rectal
carcinoma resected
Nov 93: right knee effusion (ESR 7)
Aug 95: right shoulder synovitis prednisolone 1mg/day
July 96: stopped steroids. Well. ESR 9.
Case History Mrs. D.E. female d.o.b.7/7/27
Oct 96: pain stiffness right hand shoulder,both knees
morning stiffness shoulder & pelvic girdles
anorexia & weight loss 9 kg
Feb 96: severe headaches,flashing lights
pain in tongue & throat when eating
tender scalp & nodules on temples
anorexia & weight loss 15 kg
O/E nodules (R) temporal artery no temporal pulse
palpable
ESR90 , gamma GT 136
TA biopsy: active giant cell arteritis
prednisolone 40 mg/day.
Resolution of symptoms in days
1996/7: unable to keep disease suppressed with less
than 15 mg prednisolone/day
methotrexate added
steady reduction of steroid thereafter
July 99: well. Stopped steroid. Continues methotrexate.
ESR19.
Polymyalgia Rheumatica and Giant Cell Arteritis
Steroid treatment
How much ?
How long ?
Complications
See attachment for 2 prescribing charts
Polymyalgia Rheumatica
Giant Cell Arteritis
Common concerns with treatment
Do not reduce steroids too quickly.
Apart from acute relapsing eye disease do not increase
steroid dose for about 1 week after an apparent flare. The flare may
be short lived.
There is no place for Deflazacort.
Intra-muscular steroids are currently under investigation.
Early bone loss and diabetes should be considered
Beyond 1 year, steroid-sparing agents should be used
if the steroid dose remains 7.5 - 10 mg +.
See attachment for benefits chart
Steroid induced Osteoporosis
See attachment for tables
Case History Mrs T 74 yrs
Jan 1993 - 3 yr history of temporal headaches
- Inconclusive TA biopsy (1992)
- Treated successfully with steroids (3 days)
Recurrence of temp. headaches, also aches in shoulder
girdles, Rt. buttock & thigh
O/E - Tender TA. ESR=70
On Prednisolone 30 mg for 3 days
Temporal Artery Biopsy Results
"Sections of temporal artery show focal duplication
and loss of elastic lamina but with no associated inflammation. Features
represent a healed stage of temporal arteritis."
NB: steroid therapy for 7 days
ESR vs Prednisolone see attachment for table
Osteoporosis Assessment
Menopause - 42 yrs
# R wrist - 50 yrs
# L patella - 63 yrs
Steroid therapy
c/o spinal pain - Thoracic wedge #
DXA scan - results
Osteoporosis Treatment
1993
Rocaltrol 250 nanograms b.d
Sandocal 1g o.d
Calcitonin 50 units 3 times weekly for 4 months
1996
Lowering BMD - Alendronate 10 mg o.d
1998
Improved BMD
Serial lumbar spine bone density see attachment
Subsequent course of condition
1993-94 Symptoms responded & did not recur but
ESR 40-90 mm/h
1994 Pred.5mg ESR40
1995 Pred.4mg ESR51
1997 Pred.3/2 mg ESR46
1998 Pred.2/1mg ESR46
1999 Pred.1mg ESR27
Giant Cell Arteritis and Polymyalgia Rheumatica -
Diagnostic difficulties
The Normal ESR
What is the ESR?
Rate of agglutination of red cells
determined by fibrinogen level
fibrinogen is an Acute Phase Reactant
main stimulus Interleukin-6
source of IL-6 in PMR/GCA?
IL-6 level correlates with disease activity
Is PMR/GCA a viable diagnosis with a normal ESR?
Retrospective analysis of cohorts of PMR cases:
1) Gonzalez et al : ESR<40 20% of 201 cases
more frequent in:
men; younger patients
less fever
no weight loss
no anaemia or electrophoretic abnormalities
2) Helfgott & Kieval : ESR<30
20% of 117 cases
men more frequent
higher Hb
much longer disease duration before treatment
Are these cases really PMR/GCA?
Retrospective studies
cases already included on clinical grounds
in ACR GCA criteria ESR excluded from classification
tree due to low specificity
best predictor is temporal artery tenderness or decreased
pulsation
Summary
PMR/GCA can be present in the absence of an elevated
ESR
generally indicates less severe disease
Giant Cell Arteritis and Polymyalgia Rheumatica-
diagnostic difficulties: the young patient
very rare under 50 years
often associated with malignancy
poor steroid response
no trials
Giant Cell Arteritis and Polymyalgia Rheumatica Diagnostic
difficulties: the normal temporal artery
variable percentage of biopsies in GCA are positive
?poor selection
previous steroid therapy
'some' PMR patients have positive biopsies
? positivity rate in clinically normal arteries
SUMMARY
do not over treat PMR 'in case' of GCA
consider prevention of steroid-induced osteoporosis
atypical cases of GCA/PMR occur rarely