View source for Polymyalgia rheumatica

{{Short description|Inflammatory disease featuring pain and stiffness}}
{{other uses|PMR (disambiguation)}}
{{Use mdy dates|date=December 2023}}
{{Infobox medical condition (new)
| name = Polymyalgia rheumatica
| synonyms = 
| image = Polymyalgia rheumatica man.svg
| image_size = 200px
| caption = In polymyalgia rheumatica, pain is usually located in the [[shoulders]] and [[Hip (anatomy)|hips]].
| pronounce = 
| field = [[Rheumatology]]
| symptoms = Shoulder, neck and hip pain<ref name="NIAMS"/>
| complications = 
| onset = Age greater than 50
| duration = 
| types = 
| causes = 
| risks = 
| diagnosis = Elevated inflammatory markers, [[C-reactive protein|CRP]] and [[erythrocyte sedimentation rate|ESR]]
| differential = [[Myositis]], [[giant cell arteritis]]
| prevention = 
| treatment = 
| medication = Corticosteroids
| prognosis = 
| frequency = 
| deaths = 
}}

<!-- Definition and symptoms -->
'''Polymyalgia rheumatica''' ('''PMR''') is a systemic inflammatory disease characterized by pain or stiffness, usually in the [[neck]], [[shoulders]], upper arms, and [[Hip (anatomy)|hips]], but which may occur all over the body. Almost all cases occur in people age 50 or older. Pain and stiffness of PMR is worst in the morning and improves throughout the day, but these symptoms frequently persist to some extent throughout the day and into the evening.<ref name=":1">{{Cite journal |last=Espígol-Frigolé |first=Georgina |last2=Dejaco |first2=Christian |last3=Mackie |first3=Sarah L |last4=Salvarani |first4=Carlo |last5=Matteson |first5=Eric L |last6=Cid |first6=Maria C |date=October 2023 |title=Polymyalgia rheumatica |url=https://linkinghub.elsevier.com/retrieve/pii/S0140673623013107 |journal=The Lancet |language=en |volume=402 |issue=10411 |pages=1459–1472 |doi=10.1016/S0140-6736(23)01310-7|url-access=subscription }}</ref>

<!-- Cause and diagnosis -->
People who have polymyalgia rheumatica may also have [[temporal arteritis]] ([[giant cell arteritis]]), an inflammation of blood vessels in the face which can cause blindness if not treated quickly.<ref>{{cite journal|last1=Schmidt|first1=J|last2=Warrington|first2=KJ |name-list-style=vanc|title=Polymyalgia rheumatica and giant cell arteritis in older patients: diagnosis and pharmacological management|journal=Drugs & Aging|date=August 1, 2011|volume=28|issue=8|pages=651–66|pmid=21812500|doi=10.2165/11592500-000000000-00000|s2cid=44787949}}</ref> The pain and stiffness can result in a lowered quality of life, and can lead to depression.<ref name="NIAMS">{{cite web |date=April 11, 2017 |title=Polymyalgia Rheumatica |url=https://www.niams.nih.gov/health-topics/polymyalgia-rheumatica |access-date=February 10, 2021 |website=National Institute of Arthritis and Musculoskeletal and Skin Diseases |language=en}}</ref> The exact cause of PMR, including whether or not it may be an [[autoimmune disease]], is unclear.<ref name=":2" /> Persons of [[Northern European]] descent are at greater risk.<ref name="Cimmino">{{cite journal |last1=Cimmino |first1=Marco A. |name-list-style=vanc |date=1997 |title=Genetic and environmental factors in polymyalgia rheumatica |url= |journal=Annals of the Rheumatic Diseases |volume=56 |issue=10 |pages=576–577 |doi=10.1136/ard.56.10.576 |pmc=1752263 |pmid=9389216}}</ref> There is no definitive laboratory test, but [[C-reactive protein]] (CRP) and [[erythrocyte sedimentation rate]] (ESR) can be useful as non-specific markers of systemic inflammation.<ref name=":1" />

<!-- Treatment -->
PMR is usually treated with [[corticosteroid]]s taken by mouth.<ref>{{cite journal|last1=Dejaco|first1=C|last2=Singh|first2=YP|name-list-style=vanc|title=2015 Recommendations for the management of polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative.|journal=Annals of the Rheumatic Diseases|date=October 2015|volume=74|issue=10|pages=1799–807|pmid=26359488|doi=10.1136/annrheumdis-2015-207492|doi-access=free|hdl=2445/115060|hdl-access=free}}</ref> Most people need to continue the corticosteroid treatment for two to three years.<ref name="Mayoclinic treatments 2010">{{cite web |title=Polymyalgia Rheumatica treatments and drugs |url=http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=treatments-and-drugs |date=December 4, 2010 |publisher=MayoClinic |access-date=January 19, 2012}}</ref> PMR sometimes goes away on its own in a year or two, but medications and [[self-care]] measures (e.g., eating the [[5 A Day|recommended amount of fruits and vegetables]]) can improve the rate of recovery.<ref>{{cite web|title=Polymyalgia Rheumatica definition |url=http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441 |date=May 17, 2008 |publisher=MayoClinic |access-date=January 19, 2012 |url-status=dead |archive-url=https://web.archive.org/web/20080623104752/http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441 |archive-date=June 23, 2008 }}</ref>

<!-- History, society, and culture -->
PMR was first established as a distinct disease in 1966 by a case report<ref>{{cite journal |vauthors=Davison S, Spiera H, Plotz CM | title = Polymyalgia rheumatica | journal = Arthritis and Rheumatism | volume = 9 | issue = 1 | pages = 18–23  | date = February 1966 | pmid = 4952416 | doi = 10.1002/art.1780090103 }}</ref> on 11 patients at [[Mount Sinai Hospital (Manhattan)|Mount Sinai Hospital]] in New York City.<ref>{{cite web |url=http://www.the-rheumatologist.org/details/article/4722531/Letters_More_on_the_History_of_Polymyalgia_Rheumatica_and_Giant_Cell_Arteritis.html |title=Letters: More on the History of Polymyalgia Rheumatica and Giant Cell Arteritis |last1=Plotz |first1=Charles |last2=Docken |first2=William |date=May 2013 |website=The Rheumatologist |publisher=ACR/ARHP |access-date=June 1, 2014 |archive-url=https://web.archive.org/web/20150212223014/http://www.the-rheumatologist.org/details/article/4722531/Letters_More_on_the_History_of_Polymyalgia_Rheumatica_and_Giant_Cell_Arteritis.html |archive-date=February 12, 2015 |url-status=dead }}</ref> It takes its name from the [[Greek language|Greek]] word Πολυμυαλγία ''polymyalgia'', which means "pain in many muscles".

==Signs and symptoms==
A wide range of symptoms can indicate if a person has polymyalgia rheumatica. The classic symptoms include:<ref name=":1" /><ref>{{cite web |url=https://www.nhs.uk/conditions/polymyalgia-rheumatica/| title=Polymyalgia rheumatica
|website=NHS | date=October 20, 2017
|access-date= July 23, 2021}}</ref>
* Pain and stiffness (moderate to severe) in the neck, shoulders, upper arms, thighs, and hips, which inhibits activity, especially in the morning, but which usually persists to some degree throughout the day. Pain can also occur in the groin area and in the buttocks. The pain can be limited to one of these areas as well. It is a disease of the "girdles" meaning shoulder girdle or pelvic girdle.
* [[Fatigue (medical)|Fatigue]] and lack of appetite (possibly leading to weight loss)
* [[Anemia]]
* An overall feeling of illness or flu-like symptoms.
* Low-grade (mild) fever<ref>{{cite web |title=Polymyalgia Rheumatica symptoms |url=http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=symptoms |date=December 4, 2010 |publisher=MayoClinic |access-date=January 19, 2012}}</ref> or abnormal temperature is sometimes present.
** High or spiking fevers may occur in patients who also have [[giant cell arteritis]].<ref name=":1" />
* Generalized weakness
** Note that this is ''generalized'' weakness, not ''muscle'' weakness. The presence of muscle weakness likely indicates a different diagnosis.<ref name=":1" />
* Inflammatory swelling and pain of wrists and/or knees (only ~25% of cases)
* Pitting edema (non-inflammatory swelling) of wrists, ankles, hands, and feet (only ~10% of cases)

=== Temporal arteritis ===
About 20% of people who are diagnosed with polymyalgia rheumatica also have [[temporal arteritis]] (also called ''giant cell arteritis''), and about 50% of people with temporal arteritis have polymyalgia rheumatica.<ref name=":1" /> Some symptoms of temporal arteritis include [[headaches]], scalp tenderness, [[jaw]] or facial soreness, [[distorted vision]], or aching in the limbs caused by decreased blood flow, and fatigue.<ref name="WebMD">{{cite web |date=November 18, 2007 |title=Polymyalgia Rheumatica and Temporal Arteritis |url=http://arthritis.webmd.com/polymyalgia-rheumatica-temporal-arteritis |access-date=June 10, 2008 |publisher=[[WebMD]] |vauthors=Gelfand JL}}</ref>

==Causes==
The pathophysiology of polymyalgia rheumatica is not well-understood. Evidence shows that there is likely a combined genetic and environmental pathophysiology behind the disease, but concrete identification of the causes, including whether or not polymyalgia rheumatica is an [[autoimmune disease]], remains elusive.<ref name=":2">{{Cite journal |last=Guggino |first=G. |last2=Ferrante |first2=A. |last3=Macaluso |first3=F. |last4=Triolo |first4=G. |last5=Ciccia |first5=F. |date=2018-03-27 |title=Pathogenesis of polymyalgia rheumatica |url=https://www.reumatismo.org/index.php/reuma/article/view/1048 |journal=Reumatismo |language=en |volume=70 |issue=1 |pages=10–17 |doi=10.4081/reumatismo.2018.1048 |issn=2240-2683|hdl=10447/399886 |hdl-access=free }}</ref> It is, at the very least, an immune-''mediated'' disease, with both [[Innate immune system|innate]] and [[adaptive immune system]] elements being known to play a role.<ref name=":1" /><ref name=":2" /> Infectious diseases have historically been hypothesized as a likely trigger for disease in genetically susceptible people. Individual studies have sometimes found correlations between specific pathogens and development of the disease, but broader analysis fails to find significant correlations. Proposed causative pathogens, none of which have been proven, include:<ref name=":2" />

* ''[[Mycoplasma pneumoniae]]''
* ''[[Chlamydia pneumoniae]]''
* [[Hepatitis B]] virus (HBV)
* [[Parvovirus B19]]
* [[Adenovirus infection|Adenovirus]] (ADV)
* [[Respiratory syncytial virus]] (RSV)

The immune cell involvement in polymyalgia rheumatica includes the activation of dendritic cells and monocytes/[[Macrophage|macrophages]], leading to inflammation in the synovium and bursae of the shoulder and hip girdles which is primarily mediated by the innate immune system. There is an altered balance between [[T helper 17 cell|Th17]] and [[Regulatory T cell|Treg]] cells, with increased [[Interleukin 6|IL-6]] levels driving Th17 cell activation. Disturbed [[B cell]] distribution and function are also observed, with a decrease in circulating B cells that recover after steroid treatment. Additionally, ''systemic'' activation of circulating [[Monocyte|monocytes]] is associated with increased IL-6 and [[Interleukin 1 beta|IL-1 beta]] production. Associations of uncertain significance with multiple types of [[Tumor necrosis factor|TNF]] have also been found.<ref name=":2" />

Despite the severe pain associated with the condition in multiple muscle groups, as well as the signs of systemic inflammation, muscle [[Biopsy|biopsies]] have found no signs of ''localized'' inflammation in muscle tissue in patients with PMR. [[Electromyography]] studies also typically turn up normal. The only locations known definitively to be inflamed in PMR are the [[Synovial membrane|synovial membranes]] and [[Synovial bursa|bursae]] of joints.<ref name=":2" />

Persons having the [[HLA-DR4]] type of [[human leucocyte antigen]] appear to have a higher risk of PMR.<ref name=agabegi2nd255>Page 255 in: {{cite book |author1=Elizabeth D Agabegi |author2=Agabegi, Steven S. |title=Step-Up to Medicine (Step-Up Series) |publisher=Lippincott Williams & Wilkins |location=Hagerstwon, MD |year=2008 |isbn=978-0-7817-7153-5 |url-access=registration |url=https://archive.org/details/stepuptomedicine0000agab }}</ref>

==Diagnosis==
No specific test exists to diagnose polymyalgia rheumatica; many other diseases can cause inflammation and pain in muscles, but a few tests can help narrow down the cause of the pain. Limitation in shoulder motion or swelling of the joints in the wrists or hands, are noted by the doctor.<ref name="MedicineNet">{{cite web |author=Shiel Jr WC |date=March 13, 2008 |title=Polymyalgia Rheumatica (PMR) & Giant Cell Arteritis (Temporal Arteritis) |url=http://www.medicinenet.com/polymyalgia_rheumatica/article.htm |access-date=June 10, 2008 |publisher=MedicineNet}}</ref>

One [[blood test]] usually performed is the [[erythrocyte sedimentation rate]] (ESR) which measures how fast the patient's red blood cells settle in a test tube. The faster the red blood cells settle, the higher the ESR value (measured in mm/hour), which suggests that inflammation may be present. Many conditions can cause an elevated ESR, so this test alone is not proof that a person has polymyalgia rheumatica.<ref name="Mayoclinic diagnosis 2010">{{cite web |date=December 4, 2010 |title=Polymyalgia Rheumatica tests and diagnosis |url=http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=tests-and-diagnosis |access-date=January 19, 2012 |publisher=MayoClinic}}</ref><ref name="Mayoclinic diagnosis 2008">{{cite web|title=Polymyalgia Rheumatica tests and diagnosis |url=http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=tests-and-diagnosis |date=May 17, 2008 |publisher=MayoClinic |access-date=January 19, 2012 |url-status=dead |archive-url=https://web.archive.org/web/20080623203621/http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=tests-and-diagnosis |archive-date=June 23, 2008 }}</ref>

Another test that checks the level of [[C-reactive protein]] (CRP) in the blood may also be conducted. CRP is produced by the liver in response to an injury or infection, and people with polymyalgia rheumatica usually have high levels.<ref name="Mayoclinic diagnosis 2010"/><ref name="Mayoclinic diagnosis 2008"/> However, like the ESR, this test is also not very specific.<ref>{{Cite journal |last=Buttgereit |first=Frank |last2=Dejaco |first2=Christian |last3=Matteson |first3=Eric L. |last4=Dasgupta |first4=Bhaskar |date=2016-06-14 |title=Polymyalgia Rheumatica and Giant Cell Arteritis: A Systematic Review |url=http://jama.jamanetwork.com/article.aspx?doi=10.1001/jama.2016.5444 |journal=JAMA |language=en |volume=315 |issue=22 |pages=2442 |doi=10.1001/jama.2016.5444 |issn=0098-7484|url-access=subscription }}</ref>

Polymyalgia rheumatica is sometimes associated with [[temporal arteritis]], a condition requiring more aggressive therapy. To test for this additional disorder, a [[biopsy]] sample may be taken from the [[Superficial temporal artery|temporal artery]].<ref name="Mayoclinic diagnosis 2010"/>

==Treatment==
[[Prednisone]] is the drug of choice for PMR,<ref name=":0">{{cite journal |vauthors=Hernández-Rodríguez J, Cid MC, López-Soto A, Espigol-Frigolé G, Bosch X | title = Treatment of polymyalgia rheumatica: a systematic review | journal = Archives of Internal Medicine | volume = 169 | issue = 20 | pages = 1839–50 | date = Nov 2009 | pmid = 19901135 | doi = 10.1001/archinternmed.2009.352 | doi-access = free }}</ref> and treatment duration is frequently greater than one year.<ref name="MedicineNet"/> If the patient does not experience dramatic improvement after three days of 10–20&nbsp;mg oral prednisone per day, the diagnosis should be reconsidered.<ref>{{cite book | vauthors = McPhee SJ, Papadakis MA | year = 2010 | title = Current Medical Diagnosis and Treatment | page = [https://archive.org/details/isbn_9780071624442/page/767 767] | isbn = 978-0071624442 | url-access = registration | url = https://archive.org/details/isbn_9780071624442/page/767 }}</ref> Sometimes relief of symptoms occurs in only several hours.<ref name=":0" />

Nonsteroidal anti-inflammatory drugs (NSAIDs) such as [[ibuprofen]] are ineffective in the initial treatment of PMR,<ref name="ACR">{{cite web |author=Docken WP |title=Polymyalgia rheumatica | url = http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/polymyalgiarheumatica.asp | date=August 2009 |publisher=American College of Rheumatology |access-date=January 20, 2012}}</ref> but they may be used in conjunction with the maintenance dose of corticosteroid.<ref>{{cite web |website=MDGuidelines |title=Polymyalgia rheumatica |url=http://www.mdguidelines.com/polymyalgia-rheumatica |access-date=January 20, 2012}}</ref>

Along with medical treatment, patients are encouraged to exercise and eat healthily, helping to maintain a strong immune system and build strong muscles and bones.<ref name="Mayoclinic lifestyle 2008">{{cite web|title=Polymyalgia Rheumatica lifestyle and home remedies |url=http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=lifestyle-and-home-remedies |date=May 17, 2008 |publisher=MayoClinic |access-date=January 19, 2012 |url-status=dead |archive-url=https://web.archive.org/web/20080623203610/http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=lifestyle-and-home-remedies |archive-date=June 23, 2008 }}</ref> A diet of fruits, vegetables, whole grains, and low-fat meat and dairy products, avoiding foods with high levels of refined sugars and salt is recommended.<ref name="Mayoclinic lifestyle 2010">{{cite web |title=Polymyalgia Rheumatica lifestyle and home remedies |url=http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=lifestyle-and-home-remedies |date=December 4, 2010 |publisher=MayoClinic |access-date=January 19, 2012}}</ref> Research in the UK has also suggested that people with polymyalgia rheumatica would benefit from a falls assessment when first diagnosed, and regular treatment reviews.<ref>{{Cite journal |date=June 21, 2022 |title=Polymyalgia rheumatica: treatment reviews are needed |url=https://evidence.nihr.ac.uk/alert/polymyalgia-rheumatica-treatment-reviews-and-falls-assessment/ |access-date=August 5, 2022 |website=NIHR Evidence |doi=10.3310/nihrevidence_51304 |s2cid=251774691 |language=en-GB|url-access=subscription }}</ref><ref>{{Cite journal |last1=Singh Sokal |first1=Balamrit |last2=Hilder |first2=Samantha L. |last3=Paskins |first3=Zoe |last4=Mallen |first4=Christian D |last5=Muller |first5=Sara |date=2021 |title=Fragility fractures and prescriptions of medications for osteoporosis in patients with polymyalgia rheumatica: results from the PMR Cohort Study |journal=Rheumatology Advances in Practice |volume=5 |issue=3 |pages=rkab094 |doi=10.1093/rap/rkab094|pmid=34988356 |pmc=8712242 }}</ref>

==Epidemiology==
No circumstances are certain as to which an individual will get polymyalgia rheumatica, but a few factors show a relationship with the disorder:
* Usually, PMR only affects adults over the age of 50.<ref name=":1" /><ref name="ACR"/>
* The average age of a person who has PMR is about 70 years old,<ref name="WebMD"/><ref name="Mayoclinic risk factors 2010">{{cite web |title=Polymyalgia Rheumatica risk factors |url=http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=risk-factors |date=December 4, 2010 |publisher=MayoClinic |access-date=January 19, 2012}}</ref> with the highest [[prevalence]] in those older than 70.<ref name=":1" />
* Women are three times as likely to get PMR as men.<ref name=":1" />
* People of Western and Northern European descent are more likely to get this disease.<ref name="WebMD"/> It is more likely to affect people of Northern European origin; Scandinavians are especially vulnerable.<ref name="Mayoclinic risk factors 2010"/>
* About 50% of people with temporal arteritis also have polymyalgia rheumatica.<ref name="WebMD"/>

== See also ==
* [[Remitting seronegative symmetrical synovitis with pitting edema]]
* [[Tocilizumab]]

== References ==
{{reflist}}

== External links ==
* [http://niams.nih.gov/Health_Info/Polymyalgia/default.asp Questions and Answers about Polymyalgia Rheumatica and Giant Cell Arteritis]&nbsp;- US National Institute of Arthritis and Musculoskeletal and Skin Diseases
* [https://www.symptomatrix-nl.eu/ Symptomatrix]
{{Medical resources
|  DiseasesDB     = 10331
|  ICD10          = {{ICD10|M|35|3|m|30}}
|  ICD9           = {{ICD9|725}}
|  ICDO           =
|  OMIM           =
|  MedlinePlus    = 000415
|  eMedicineSubj  = emerg
|  eMedicineTopic = 473
|  MeshID         = D011111
}}

{{Systemic connective tissue disorders}}
{{Soft tissue disorders}}

[[Category:Steroid-responsive inflammatory conditions]]
[[Category:Systemic connective tissue disorders]]