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Polymyalgia Rheumatica
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Polymyalgia rheumatica (PMR)\: in
hips).
Population\: a
Aetiology\: immune-mediated, possibly autoimmune, linked to genetic predisposition and neurohormonal changes in older
adults; a
Risk factors\: age >50 years, prior history of giant cell arteritis (GCA), female, family history.
Symptoms\: acute/sub-acute onset, pain, sti
hour); constitutional symptoms (fever, weight loss, night sweats, malaise, fatigue, anorexia).
GCA symptoms\: painful/tender scalp, new temporal headaches, jaw claudication, visual changes (blurring/loss); GCA can
lead to permanent vision loss if untreated.
Examination\: bilateral proximal muscle tenderness, limited range of motion due to pain, normal muscle strength, tender
scalp (GCA), thickened/diminished temporal artery pulse (GCA).
Di
osteoarthritis, hypothyroidism.
Investigations\:
FBC\: normocytic anaemia, thrombocytosis.
In
Exclusion tests\: LFTs, renal function, immunoglobulins, RF, anti-CCP, TFTs, calcium, alkaline phosphatase.
Imaging\: ultrasound (joint e
GCA investigations\: temporal artery biopsy (gold standard), ultrasound, MRA.
Diagnosis\: clinical diagnosis based on age >50, proximal muscle pain/sti
steroids.
Management\:
Glucocorticoids\: prednisolone, tapered over weeks to months based on symptoms and in
Steroid side e
vitamin D).
Glucocorticoid sparing therapy\: DMARDs (methotrexate), biologics (tocilizumab).
Complications\: relapsing/remitting course, glucocorticoid-related complications, cardiovascular mortality (equivalent to
general population if systemic in
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A comprehensive topic overview
Introduction
Polymyalgia rheumatica (PMR) is an in
muscles, speci
1
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PMR can exist in isolation but often co-exists with other rheumatological disorders. PMR commonly a
females over the age of 50 years with the peak age of onset being 70-80 years.
2
Aetiology
The exact aetiology of PMR is not fully understood. It is recognised as an immune-mediated in
some autoimmune and familial associations, but the link is not well de
Additionally, because the age of onset is older adults, it is believed that those with a genetic predisposition may develop
PMR as their immune system ages along with changes to neurohormonal regulation.
3
While the term “polymyalgia” suggests PMR has a muscle-related aetiology, PMR tends to primarily a
articular structures such as tendons, bursa and synovium. 4
There is often no pathology seen in a muscle biopsy.
Risk factors
Risk factors for polymyalgia rheumatica include\:
Age over 50 years
Prior history of giant cell arteritis (GCA)
Female
Family history
Clinical features
History
PMR tends to present acutely or sub-acutely over days to weeks.
Patients describe pain, sti
their activities of daily living.
The distal muscles of the upper and lower limbs are rarely a
lasting over an hour.
5
Typical symptoms of PMR include\:
Shoulders\: patients describe a reduced range of motion and di
Hips\: patients have di
Constitutional symptoms\: low-grade fevers, weight loss, night sweats, malaise, fatigue and anorexia
The symptoms of PMR overlap with many systemic infections or malignancies so red
weight loss, night sweats, fevers, and personal or family history of malignancy should prompt consideration for sinister
pathologies.
PMR has a strong association with giant cell arteritis (GCA) leading most clinicians to consider them as disorders on the
same spectrum of disease. 6
Patients who present with PMR should be screened for GCA symptoms.
Giant cell arteritis (GCA)
Giant cell arteritis (GCA) is a vasculitis that a
arteritis, where there is in
7
Typical symptoms of GCS include\:
Painful and tender scalp\: often while combing or rubbing shampoo into their hair
Headaches\: new temporal headaches
Jaw claudication\: pain in the jaw while eating/ chewing
Visual changes\: blurring or loss of vision
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For more information, see the Geeky Medics guide to GCA (temporal arteritis).
GCA needs to be treated as a medical emergency as it can result in permanent vision loss if left untreated.
8
It is important to note that GCA can also a
cases, this may present as arm pain, but in more severe cases it could result in an aortic aneurysm.
9
Clinical examination
Typical
10
Bilateral proximal muscles may be di
Active and passive range of motion is limited by pain
Muscle strength is normal or limited by pain in the proximal muscles
The scalp may be tender to touch suggestive of GCA
An examination of the temporal artery may demonstrate a thickened temporal artery with diminished or lack of palpable
pulse
The neurological examination is usually normal.
Di
Di
In
Infectious conditions\: Lyme disease, infective endocarditis
Malignancy\: multiple myeloma
Other\: osteoarthritis, hypothyroidism
Investigations
Laboratory investigations
Relevant laboratory investigations include\:
Full blood count (FBC)\: normocytic anaemia or thrombocytosis may be seen in ongoing in
In
PMR. CRP is sensitive for acute disease activity and is followed up to assess response to treatment.
11
Because the symptoms of PMR can be generalised and non-speci
other conditions. These tests include LFTs, renal function tests, immunoglobulins, rheumatoid factor (RF), anti-CCP, thyroid
function tests, calcium and alkaline phosphatase.
Imaging
Relevant imaging investigations include\:
12,15
Ultrasound (US)\: commonly shows joint e
in
Magnetic resonance imaging (MRI)\: MRI is a more sensitive test to assess for synovitis and bursitis (not routinely
performed)
Fluorodeoxyglucose F18- positron emission tomography (FDU PET)\: new imaging modality used to see increased
uptake in the bursa and synovium of the shoulders, ischial tuberosity and greater trochanters consistent with the pain of
PMR. FDU PET is also useful for assessing vasculitis (not routinely performed).
Investigations for giant cell arteritis (GCA)
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Patients with symptoms of GCA will require further investigation. The current gold standard con
is a temporal artery biopsy (TAB).
13
GCA is known to have skip lesions, so a negative result on a biopsy doesn’t exclude GCA. Prompt treatment with
steroids may also give false-negative results on TAB.
Ongoing research supports minimally invasive diagnostic modalities including ultrasound and magnetic resonance
angiogram (MRA) to better assess the entire length of the a
the assessment of other large vessels like the aorta and its branches implicated in GCA.
Diagnosis
There is no single test for the diagnosis of PMR. It is a clinical diagnosis based on the following features\:
Age of onset over 50 years
The classical presentation of symptoms with proximal muscle sti
A blood test showing elevated in
Improvement of symptoms with the initiation of steroid therapy
Management
Medical management
Glucocorticoids (e.g. prednisolone) are the mainstay of treatment for PMR.
Glucocorticoids should be tapered (a reducing course) over a period of weeks to months as symptoms resolve and
in
Glucocorticoid tapering
NICE suggest the following regime for tapering the dose of prednisolone when treating PMR\:
14
Continue prednisolone 15 mg each day until symptoms are fully controlled (usually 3 weeks)
Reduce the dose to 12.5 mg each day for 3 weeks
Reduce the dose to 10 mg each day for 4–6 weeks
Reduce the dose by 1 mg every 4–8 weeks until treatment is stopped
Glucocorticoids tapers are important, as a rapid withdrawal of steroids after long-term use can result in an
Addisonian crisis. The rate of taper di
symptoms.
Steroid side e
Glucocorticoids have numerous side e
Diabetes\: glucocorticoids cause hyperglycaemia and can precipitate a hyperglycaemic hyperosmolar state (HHS) in
patients with co-morbid diabetes
Gastro-oesophageal re
addition of a proton-pump inhibitor (PPI) may be indicated
Osteoporosis\: given that elderly women have lower bone density and are the main demographic of PMR,
glucocorticoids can further increase fracture risk. This group may bene
antagonist), calcium and vitamin D supplements.
Glucocorticoid sparing therapy
Glucocorticoid sparing drugs are used in PMR and GCA to limit the adverse e
may include disease-modifying anti-rheumatic agents (DMARDs) or biologics.
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Methotrexate is an anti-folate DMARD that can be used to manage PMR symptoms as part of a glucocorticoid taper.
Tocilizumab is a licensed IL-6 inhibitor that reduces the acute in
Complications
PMR can have a relapsing and remitting course, with asymptomatic periods and acute
Most long-term complications of PMR are linked to glucocorticoid therapy. There has been a concern regarding increased
cardiovascular mortality, but a recent (2015) meta-analysis suggests that with control of systemic in
cardiovascular mortality is equivalent to the general population.
15
Patients presenting with PMR or GCA should be counselled about GCA symptoms and advised to seek advice immediately
if symptoms arise. GCA can present at any time in the disease progression. Active in
following potentially signi
Blindness
Stroke
Aortitis leading to aortic aneurysm
References
Mackie, S. L., & Mallen, C. D. (2013). Polymyalgia rheumatica. B M J , 3 4 7 (Dec, 031).
Salvarani, C., Gabriel, S. E., Michael O'Fallon, W., & Hunder, G. G. (1995). Epidemiology of polymyalgia rheumatica in Olmsted
county, Minnesota, 1970–1991. A r t h r i t i s & R h e u m a t i s m , 3 8 (3), 369–373.
Weyand, C. M., & Goronzy, J. J. (2003). Giant-Cell Arteritis and Polymyalgia Rheumatica. A n n a l s o f I n t e r n a l M e d i c i n e , 1 3 9 (6),
505.
Ochi, J., Nozaki, T., Okada, M., Suyama, Y., Kishimoto, M., Akaike, G., Tasaki, A., Ohde, S., Saida, Y., & Yoshioka, H. (2015). MRI
Soubrier, M., Dubost, J.
-J., & Ristori, J.
-M. (2006). Polymyalgia rheumatica\: diagnosis and treatment. J o i n t B o n e S p i n e , 7 3 (6),
599–605.
Dejaco, C., Duftner, C., Buttgereit, F., Matteson, E. L., & Dasgupta, B. (2017). The spectrum of giant cell arteritis and
polymyalgia rheumatica\: revisiting the concept of the disease. R h e u m a t o l o g y , 5 6 (4), 506–515.
Barros, S., Lopez-Diaz, M. J., Garcia-Porrua, C., Sanchez-Andrade, A., & Llorca, J. (2005). Giant Cell Arteritis. M e d i c i n e , 8 4 (5),
269–276.
Chen, J. J., Leavitt, J. A., Fang, C., Crowson, C. S., Matteson, E. L., & Warrington, K. J. (2016). Evaluating the Incidence of
Arteritic Ischemic Optic Neuropathy and Other Causes of Vision Loss from Giant Cell Arteritis. O p h t h a l m o l o g y , 1 2 3 (9),
19992003.
Janssen, S. P., Comans, E. H., Voskuyl, A. E., Wisselink, W., & Smulders, Y. M. (2008). Giant cell arteritis\: Heterogeneity in
clinical presentation and imaging results. J o u r n a l o f V a s c u l a r S u r g e r y , 4 8 (4), 1025–1031.
van der Geest, K. S., Sandovici, M., Brouwer, E., & Mackie, S. L. (2020). Diagnostic Accuracy of Symptoms, Physical Signs, and
Laboratory Tests for Giant Cell Arteritis. J A M A I n t e r n a l M e d i c i n e , 1 8 0 (10), 1295–1304.
Cantini, F., Salvarani, C., Olivieri, I., Macchioni, L., Ranzi, A., Niccoli, L., Padula, A., & Boiardi, L. (2000). Erythrocyte
sedimentation rate and C-reactive protein in the evaluation of disease activity and severity in polymyalgia rheumatica\: A
prospective follow-up study. S e m i n a r s i n A r t h r i t i s a n d R h e u m a t i s m , 3 0 (1), 17–24.
Huwart, A., Garrigues, F., Jousse-Joulin, S., Marhadour, T., Guellec, D., Cornec, D., Gouillou, M., Saraux, A., & Devauchelle-
Pensec, V. (2018). Ultrasonography and magnetic resonance imaging changes in patients with polymyalgia rheumatica
treated by tocilizumab. A r t h r i t i s R e s e a r c h & T h e r a p y , 2 0 (1).
Sait, M. R., Lepore, M., Kwasnicki, R., Allington, J., Balasubramanian, R., Somasundaram, S. K., Vashisht, R., & Barkeji, M. (2017).
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artery biopsies? I n t e r n a t i o n a l J o u r n a l o f S u r g e r y O p e n , 9 , 19-23.
NICE Clinical Knowledge Summary. P o l y m y a l g i a r h e u m a t i c a . Available from\: [LINK]
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Ungprasert, P., Koster, M. J., & Warrington, K. J. (2015). Coronary artery disease in giant cell arteritis\: A systematic review and
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Reviewer
Dr Grainne Murphy
Consultant Rheumatologist
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Test yourself
Contents
Introduction
Aetiology
Risk factors
Clinical features
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