Polymyalgia rheumatica typically manifests as inflammatory pain in the shoulder and / or pelvic girdles in a patient over 50 yrs of age t ( PMR ) is the most common inflammatory rheumatic disease in the elderly and is one of the biggest indications for long term steroid therapy
Pathophysiology- Poorly understood Polygenic inflammatory disorders ( PMR & GCA ) with several genetic & environmental factors likely influencing patient susceptibility & disease severity Closely linked to GCA ( Giant cell arteritis ) About one half of patients affected by GCA also meet diagnostic criteria of PMR – however only about 10 % of patients with PMR also have GCAso it is thought that mechanisms which contribute to GCA may be involved as genetically predisposed individual – an environment factor possibly virusPathologically GCA and PMR- similar Main difference – absence of significant vascular involvement in PMR PMR- no evidence of disease in muscle biopsy A mild synovitis with macrophages and CD4 + T cell infiltration in the glenohumeral jt ( systemic macrophages and T-cell activation are seen in both GCA and PMR ) Increased pro-inflammatory cytokines and melatonin
Epidemiology-Highest in individuals with Northern European descent – more in Scandinavian people than other white population Women twice more likely to be diagnosed Increases with age in both sexes until age 80 Seldom diagnosed in people younger than 50 yrs of age – median age of diagnosis is 72 yrs UK – annual incidence is 84 per 100,000 No increase in mortality associated with PMR
Complications long term steroid therapy- Osteoporosis Weight gain Skin bruising Raised BP Impaired blood glucose Depression Cardiac failure Susceptibility to infections Cataracts Glaucoma Cushing’s syndrome
Presentation –Bilateral shoulder pain and / or pelvic girdle pain Morning stiffness > 45 min Abrupt onset Age > 50 yrs Duration > 2 weeks Acute phase response – raised ESR / CRP Rapid response to prednisolone
ESR is sensitive for PMR but not specific- frequently greater than 40 & can exceed 100 Mildly elevated in 7-20 % and can be occasionally normal- a rapid response to steroids can suggest PMR in such cases
Low grade fever Anorexia Weight loss Malaise fatigue and depression Difficulty rising from bed in morning Difficulty getting up from toilet or out of a chair Synovitis of proximal joints and periarticular structures Peripheral arthritis ( in 25 % patients ) Muscle stiffness after prolonged inactivity
Investigations –FBC – mild normocytic normochromic anaemia is common ESR / CRP / Plasma viscosity U&E LFT – AlkPo4 may be mildly elevated Calcium Protein electrophoresis & Bence Jones Protein -Normal TFT Creatinine kinase – is normal Rheumatoid factor ( Anti- CCP if available ) CXR if significant systemic symptoms Urine dipstick MRI- bursitis joint effusions
ESR > 40 is considered significant ESR < 40 can be present in 7-20 % of patients Patients with low ESR have typically lower frequency of systemic symptoms ESR is sensitive for PMR but not specific CRP is also typically elevated
Differential- Cervical & lumbar spondylosis Osteoarthritis Frozen shoulder RC disorders Thyroid disorders Parathyroid disease leading to hypercalcaemia Diabetes Viral illness Chronic osteomyelitis Tuberculosis Infective endocarditis Septic arthritis Rh arthritis Polymyositis SLE Spondyloarthropathy Multiple myeloma Acute leukaemia Lymphoma Solid tumors -Lung m renal , prostate GCA or temporal arthritis Drug induced – eg statins Osteomalacia Fibromyalgia Chronic fatigue syndrome
Referral age < 60 chronic onset lack of shoulder movements no inflammatory stiffness red flags ♦ prominent systemic features ♦ weight loss ♦ night pain ♦ neurological signs peripheral arthritis Suspected conn tissue / muscle dis normal or very high CRP Incomplete or non- specific response to steroids Ill sustained response to steroids Unable to reduce steroids at reasonable intervals without causing relapse Steroids use beyond 2 yrs Contraindication to steroid therapy
LINKS AND RESOURCES
PATIENT INFORMATION
Charity PMR GCA http://www.pmrgca.co.uk/content/home-page
American College of Rheumatology information for patients https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Polymyalgia-Rheumatica
Versus Arthritis webpage on PMR https://www.versusarthritis.org/about-arthritis/conditions/polymyalgia-rheumatica-pmr/
Versus arthritis booklet on PMR ( 20 pages ) https://www.versusarthritis.org/media/1322/polymyalgia-rheumatica-information-booklet.pdf
A comprehensive patient information page by Vasculitis Foundation https://www.vasculitisfoundation.org/education/forms/polymyalgia-rheumatica/#disease-faq
Wish to print ? 2 page info from Arthritis Australia https://arthritissa.org.au/downloads/2015-05-11_223155_Polymyalgia-rheumatica.pdf
Arthritis foundation on PMR https://www.arthritis.org/diseases/polymyalgia-rheumatica
Vasculitis Org on PMR https://www.vasculitis.org.uk/about-vasculitis/polymyalgia-rheumatica
NHS inform on PMR https://www.nhsinform.scot/illnesses-and-conditions/muscle-bone-and-joints/conditions/polymyalgia-rheumatica
Patient steroid card http://www.nmouk.nhs.uk/downloads/Steroid-card.pdf
INFORMATION FOR HEALTH CARE PROFESSIONALS ON POLYMYALGIA RHEUMATICA
Recommendations for the Management of
Polymyalgia Rheumatica
A European League Against Rheumatism/American College of Rheumatology
Collaborative Initiative https://www.rheumatology.org/Portals/0/Files/2015%20PMR%20guidelines.pdf
British Society of Rheumatology -BSR and BHPR guidelines for the management of polymyalgia rheumatica https://academic.oup.com/rheumatology/article/49/1/186/1789113
A good plain language article from Prescriber on Diagnosis and management of PMR https://www.prescriber.co.uk/article/diagnosis-and-management-of-polymyalgia-rheumatica/
Latest Advances in the Diagnosis and Treatment of Polymyalgia Rheumatica New guidelines shed light on treatment for this common, autoimmune rheumatic disease. By Matthew J. Koster, MD and Kenneth J. Warrington, MD https://www.practicalpainmanagement.com/pain/myofascial/inflammatory-arthritis/latest-advances-diagnosis-treatment-polymyalgia-rheumatica
Prescribing for polymyalgia rheumatica – a well-written article by Australian Prescriber https://www.nps.org.au/australian-prescriber/articles/prescribing-for-polymyalgia-rheumatica
When should you issue a steroid treatment card from Shropshire CCG https://www.shropshireccg.nhs.uk/media/1344/steroid-card-advice-sccg.pdf
References
- Diagnosis and management of polymyalgia rheumatica Royal College of Physicians June 2010 https://www.rcplondon.ac.uk/guidelines-policy/diagnosis-and-management-polymyalgia-rheumatica
- Dejaco, C., Singh, Y.P., Perel, P., Hutchings, A., Camellino, D., Mackie, S., Abril, A., Bachta, A., Balint, P., Barraclough, K., Bianconi, L., Buttgereit, F., Carsons, S., Ching, D., Cid, M., Cimmino, M., Diamantopoulos, A., Docken, W., Duftner, C., Fashanu, B., Gilbert, K., Hildreth, P., Hollywood, J., Jayne, D., Lima, M., Maharaj, A., Mallen, C., Martinez‐Taboada, V., Maz, M., Merry, S., Miller, J., Mori, S., Neill, L., Nordborg, E., Nott, J., Padbury, H., Pease, C., Salvarani, C., Schirmer, M., Schmidt, W., Spiera, R., Tronnier, D., Wagner, A., Whitlock, M., Matteson, E.L. and Dasgupta, B. (2015), 2015 Recommendations for the Management of Polymyalgia Rheumatica: A European League Against Rheumatism/American College of Rheumatology Collaborative Initiative. Arthritis & Rheumatology, 67: 2569-2580. doi:10.1002/art.39333
- Oct Arthritis research UK : Polymyalgia rheumatica
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Polymyalgia rheumatica Published:July 31, 2017DOI:https://doi.org/10.1016/S0140-6736(17)31825-1 Prof Miguel A González-Gay, MD Eric L Matteson, MDSantos Castañeda, MD SEMINAR| VOLUME 390, ISSUE 10103, P1700-1712, OCTOBER 07, 2017
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Polymyalgia Rheumatica Ferri’s Clinical Advisor 2018
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Polymyalgia rheumatica in primary care: managing diagnostic uncertainty BMJ 2015; 351 doi: https://doi.org/10.1136/bmj.h5199 (Published 07 October 2015)
- BMJ Best Practice – Polymyalgia Rheumatica https://bestpractice.bmj.com/topics/en-gb/153
- Polymyalgia rheumaticaa -E Medicine Updated Sep 2016
- Helliwell, Toby et al. “Polymyalgia rheumatica: diagnosis, prescribing, and monitoring in general practice.” The British journal of general practice : the journal of the Royal College of General Practitioners vol. 63,610 (2013): e361-6. doi:10.3399/bjgp13X667231
- CKS NHS Polymyalgia rheumatica Aug 2013https://cks.nice.org.uk/polymyalgia-rheumatica
- Martin Soubrier, Jean-Jacques Dubost, Jen-Michel Ristori,
Polymyalgia rheumatica: diagnosis and treatment,
Joint Bone Spine,
Volume 73, Issue 6,
2006,
Pages 599-605,
ISSN 1297-319X,
https://doi.org/10.1016/j.jbspin.2006.09.005. - Understanding and managing polymyalgia rheumatica Medicine Today 2014 : 15 (9) : 47-51
- Stat Pearls PMR via https://www.ncbi.nlm.nih.gov/books/NBK537274/