Polymyalgia rheumatica overview: Difference between revisions

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__NOTOC__
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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}; Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]; {{Rim}}
{{Polymyalgia rheumatica}}
{{Polymyalgia rheumatica}}
{{CMG}}; {{AE}} {{CZ}}; [[User:Ayesha A. Khan|Ayesha A. Khan, MD]][mailto:Ayesha.khan@stvincentcharity.com] Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]; {{Rim}}; {{AEL}}


==Overview==
==Overview==
[[Image:Bodydone.GIF|thumb|200px|In polmyalgia rheumatica (PMR), pain is usually located in the [[shoulders]] and [[hips]].]]
Polymyalgia rheumatica (PMR) is a chronic inflammatory disease that involves the [[articular]] and periarticular parts of the cervical region, [[shoulder girdle]] and [[pelvic girdle]]. PMR affects subjects over the age of 50 years and it is characterized by [[pain]] and stiffness in the [[neck]], [[Shoulder|shoulders]], upper arms, [[Hip (anatomy)|hip]], and [[Thigh|thighs]]. Although [[myalgia]] is one of the symptoms of PMR, there is no [[inflammation]] of the [[muscle]]s; instead, PMR is a disease of the [[joint]] that causes [[synovitis]]. The diagnosis of PMR relies on the clinical findings and laboratory evidence of systemic [[inflammation]]. PMR is associated with [[giant cell arteritis]]. The cause of PMR is unknown but it has been suggested that both [[Genetics|genetic]] and environmental factors are implicated. The mainstay of treatment of PMR is [[steroid]] therapy.


Polymyalgia rheumatica (PMR) is a chronic inflammatory disease that involves the articular and periarticular parts of the cervical region, [[shoulder girdle]] and [[pelvic girdle]].  PMR affects subjects over the age of 50 years and it is characterized by pain and stiffness in the neck, shoulders, upper arms, hip and thighs. Although [[myalgia]] is one of the symptoms of PMR, there is no inflammation of the [[muscle]]s; instead, PMR is a disease of the joint that causes [[synovitis]].  The diagnosis of PMR relies on the clinical findings and laboratory evidence of systemic [[inflammation]].  PMR is associated with [[giant cell arteritis]].  The cause of PMR is unknown but it has been suggested that both genetic and environmental factors are implicated.<ref name="pmid23051717">{{cite journal| author=Kermani TA, Warrington KJ| title=Polymyalgia rheumatica. | journal=Lancet | year= 2013 | volume= 381 | issue= 9860 | pages= 63-72 | pmid=23051717 | doi=10.1016/S0140-6736(12)60680-1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23051717  }} </ref>  The mainstay of treatment of PMR is [[steroid]] therapy.<ref name="pmid23051717">{{cite journal| author=Kermani TA, Warrington KJ| title=Polymyalgia rheumatica. | journal=Lancet | year= 2013 | volume= 381 | issue= 9860 | pages= 63-72 | pmid=23051717 | doi=10.1016/S0140-6736(12)60680-1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23051717  }} </ref>
==Historical Perspective==
Polymyalgia rheumatica was first described in 1888 by Bruce William as "senile rheumatic gout". The disease was referred to as "polymyalgia rheumatica" by Stuart Barber in 1957 in his article entitled "myalgic syndrome with constitutional effects; polymyalgia rheumatica".


==Historical Perspective==
==Classification==
Polymyalgia rheumatica was first described in 1888 by Bruce William as "senile rheumatic gout".<ref name="pmid20752457">{{cite journal| author=Bruce W| title=Senile Rheumatic Gout. | journal=Br Med J | year= 1888 | volume= 2 | issue= 1450 | pages= 811-3 | pmid=20752457 | doi= | pmc=PMC2198572 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20752457  }} </ref>  The disease was referred to as "polymyalgia rheumatica" by Stuart Barber in 1957 in his article entitled "mylagic syndrome with constitutional effects; polymyalgia rheumatica".<ref name="pmid13445065">{{cite journal| author=BARBER HS| title=Myalgic syndrome with constitutional effects; polymyalgia rheumatica. | journal=Ann Rheum Dis | year= 1957 | volume= 16 | issue= 2 | pages= 230-7 | pmid=13445065 | doi= | pmc=PMC1006948 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13445065  }} </ref>
There is no established system for the classification of polymyalgia rheumatica.


==Pathophysiology==
==Pathophysiology==
PMR is a chronic inflammatory disease of the articular and periarticular structures of the cervical region, shoulder girdle and hip girdle. The underlying pathophysiology of PMR remains unknown.<ref name="pmid23051717">{{cite journal| author=Kermani TA, Warrington KJ| title=Polymyalgia rheumatica. | journal=Lancet | year= 2013 | volume= 381 | issue= 9860 | pages= 63-72 | pmid=23051717 | doi=10.1016/S0140-6736(12)60680-1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23051717  }} </ref>  It has been hypothesized that genetic and environmental factors are implicated, particularly due to the seasonal and geographical differences in the prevalence of this disease.<ref name="pmid16414971">{{cite journal| author=Smeeth L, Cook C, Hall AJ| title=Incidence of diagnosed polymyalgia rheumatica and temporal arteritis in the United Kingdom, 1990-2001. | journal=Ann Rheum Dis | year= 2006 | volume= 65 | issue= 8 | pages= 1093-8 | pmid=16414971 | doi=10.1136/ard.2005.046912 | pmc=PMC1798240 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16414971  }} </ref><ref name="pmid19026700">{{cite journal| author=Alvarez-Rodriguez L, Carrasco-Marin E, Lopez-Hoyos M, Mata C, Fernandez-Prieto L, Ruiz-Soto M et al.| title=Interleukin-1RN gene polymorphisms in elderly patients with rheumatic inflammatory chronic conditions: Association of IL-1RN*2/2 genotype with polymyalgia rheumatica. | journal=Hum Immunol | year= 2009 | volume= 70 | issue= 1 | pages= 49-54 | pmid=19026700 | doi=10.1016/j.humimm.2008.10.011 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19026700  }} </ref><ref name="pmid2383076">{{cite journal| author=Cimmino MA, Caporali R, Montecucco CM, Rovida S, Baratelli E, Broggini M| title=A seasonal pattern in the onset of polymyalgia rheumatica. | journal=Ann Rheum Dis | year= 1990 | volume= 49 | issue= 7 | pages= 521-3 | pmid=2383076 | doi= | pmc=PMC1004141 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2383076  }} </ref>  It has also been hypothesized that PMR is associated with infections such as [[parainfluenza virus type 1]],<ref name="pmid9972970">{{cite journal| author=Duhaut P, Bosshard S, Calvet A, Pinede L, Demolombe-Rague S, Dumontet C et al.| title=Giant cell arteritis, polymyalgia rheumatica, and viral hypotheses: a multicenter, prospective case-control study. Groupe de Recherche sur l'Artérite à Cellules Géantes. | journal=J Rheumatol | year= 1999 | volume= 26 | issue= 2 | pages= 361-9 | pmid=9972970 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9972970  }} </ref> [[mycoplasma pneumoniae]], [[chlamydia pneumoniae]], and [[parvovirus B19]].<ref name="pmid8838518">{{cite journal| author=Elling P, Olsson AT, Elling H| title=Synchronous variations of the incidence of temporal arteritis and polymyalgia rheumatica in different regions of Denmark; association with epidemics of Mycoplasma pneumoniae infection. | journal=J Rheumatol | year= 1996 | volume= 23 | issue= 1 | pages= 112-9 | pmid=8838518 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8838518  }} </ref>  In addition, histological examinations of synovial biopsies of affected individuals reveal mild synovitis with predominance of [[CD4 T cells]] and [[macrophages]].<ref name="pmid8670331">{{cite journal| author=Meliconi R, Pulsatelli L, Uguccioni M, Salvarani C, Macchioni P, Melchiorri C et al.| title=Leukocyte infiltration in synovial tissue from the shoulder of patients with polymyalgia rheumatica. Quantitative analysis and influence of corticosteroid treatment. | journal=Arthritis Rheum | year= 1996 | volume= 39 | issue= 7 | pages= 1199-207 | pmid=8670331 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8670331  }} </ref>  Although [[myalgia]] is a symptom of PMR, there is no [[inflammation]] of the [[muscle]]s.
Polymyalgia rheumatica (PMR) is a chronic [[Inflammation|inflammatory]] disease of the [[articular]] and periarticular structures of the [[cervical]] region, [[Pectoral girdle|shoulder girdle]] and [[Hip (anatomy)|hip girdle]]. The underlying pathophysiology of PMR remains unknown. It has been hypothesized that genetic and environmental factors are implicated, particularly due to the seasonal and geographical differences in the prevalence of this disease. It has also been hypothesized that PMR is associated with [[Infection|infections]] such as [[parainfluenza virus type 1]], [[mycoplasma pneumoniae]], [[chlamydia pneumoniae]], and [[parvovirus B19]]. In addition, histological examinations of [[synovial]] [[Biopsy|biopsies]] of affected individuals reveal mild [[synovitis]] with predominance of [[CD4 T cells]] and [[macrophages]]. Although [[myalgia]] is a symptom of PMR, there is no [[inflammation]] of the [[Muscle|muscles]].The [[senescence]] of the [[immune systems]] as demonstrated by the loss of the [[CD28]] on [[CD4+ T]] [[senescent cells]] may be responsible for aberrant [[immune]] responses in [[PMR]]. [[Adaptive]] [[immune]] alterations also occurs in [[PMR]] mainly represented by the activation of [[Th17 cells]], mainly driven by the increased [[IL-6]] levels. An altered distribution and phenotype of B cells also occurs in [[PMR]] even in the absence of a clear [[autoimmune]] response. Local activation  of [[myeloid]] and [[endothelial cells]] has been also demonstrated in the non-inflamed arteries and inflamed synovial tissues of PMR patients.
 
==Causes==
There is no specific cause for polymyalgia rheumatica. However, there are possible theories about the causes which include [[Inflammation|inflammatory]] joint lining attack, [[Virus|viral infections]], and [[Biological inheritance|genetic inheritance]] of [[HLA-DR4]].


==Differential Diagnosis==
==Differentiating Polymyalgia Rheumatica from other Diseases==
PMR must be differentiated from other conditions such as late onset [[rheumatoid arthritis]], [[polymyositis]], [[dermatomyositis]], [[fibromyalgia]], and [[remitting seronegative symmetrical synovitis with pitting edema]].
PMR must be differentiated from other conditions such as late onset [[rheumatoid arthritis]], [[polymyositis]], [[dermatomyositis]], [[fibromyalgia]], and [[remitting seronegative symmetrical synovitis with pitting edema]].


==Epidemiology and Demographics==
==Epidemiology and Demographics==
PMR affects mostly subjects who are more than 50 years of age.<ref name="pmid12180732">{{cite journal| author=Doran MF, Crowson CS, O'Fallon WM, Hunder GG, Gabriel SE| title=Trends in the incidence of polymyalgia rheumatica over a 30 year period in Olmsted County, Minnesota, USA. | journal=J Rheumatol | year= 2002 | volume= 29 | issue= 8 | pages= 1694-7 | pmid=12180732 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12180732  }} </ref>  The prevalence of PMR is highest among subjects from Scandanavian countries and those from northern European origin.<ref name="pmid19917168">{{cite journal| author=Pamuk ON, Dönmez S, Karahan B, Pamuk GE, Cakir N| title=Giant cell arteritis and polymyalgia rheumatica in northwestern Turkey: Clinical features and epidemiological data. | journal=Clin Exp Rheumatol | year= 2009 | volume= 27 | issue= 5 | pages= 830-3 | pmid=19917168 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19917168  }} </ref>
Polymyalgia rheumatica (PMR) affects mostly subjects who are more than 50 years of age. The prevalence of PMR is highest among subjects from Scandinavian countries and those from northern European origin.


==Risk Factors==
==Risk Factors==
Age, female sex, Scandanavian and northern Europe origin are risk factors for polymyalgia rheumatica. [[Smoking]], sun exposure, [[infection]]s, nulliparity have also been considered risk factors.<ref name="pmid10948749">{{cite journal |author=Cimmino MA, Zaccaria A |title=Epidemiology of polymyalgia rheumatica |journal=Clin. Exp. Rheumatol. |volume=18 |issue=4 Suppl 20 |pages=S9–11 |year=2000 |pmid=10948749 |doi= |url=}}</ref>
Age, female sex, Scandinavia and northern Europe origin are risk factors for polymyalgia rheumatica. Other risk factors include [[smoking]], [[sun exposure]], [[Infection|infections]], and nulliparity.
 
== Screening ==
There is insufficient evidence to recommend routine screening for polymyalgia rheumatica.


==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==
PMR affects the quality of life of the patients. The pain and stiffness in the proximal joints might lead to sleep disturbance as well as inability to do regular daily activities such as getting dressed and getting out of a chair. The symptoms begin rapidly and last for weeks. Once the [[steroid]] treatment is initiated, the symptoms resolve rapidly within few days. In fact, the rapid resolution of symptoms with the [[steroid]] therapy reinforces the diagnosis of PMR. The [[steroid]] treatment can be associated with complications such as [[weight gain]] and bone fracture.<ref name="pmid23051717">{{cite journal| author=Kermani TA, Warrington KJ| title=Polymyalgia rheumatica. | journal=Lancet | year= 2013 | volume= 381 | issue= 9860 | pages= 63-72 | pmid=23051717 | doi=10.1016/S0140-6736(12)60680-1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23051717  }} </ref> Approximately 40 to 50% of subjects experience relapse of the symptoms. PMR is associated with [[giant cell arteritis]].
Polymyalgia rheumatica (PMR) affects the quality of life of the patients. The [[pain]] and stiffness in the proximal [[joints]] might lead to sleep disturbance as well as an inability to do regular daily activities such as getting dressed and getting out of a chair. The symptoms begin rapidly and last for weeks. Once the [[steroid]] treatment is initiated, the symptoms resolve rapidly within few days. In fact, the rapid resolution of symptoms with the [[steroid]] therapy reinforces the diagnosis of PMR. The [[steroid]] treatment can be associated with complications such as [[weight gain]] and [[bone fracture]]. Approximately 40 to 50% of subjects experience a relapse of the symptoms. PMR is associated with [[giant cell arteritis]].


==Diagnosis==
==Diagnosis==
===Diagnostic Criteria===
===Diagnostic Study of Choice===
The diagnosis of PMR is mostly clinical and it is supported with specific findings on laboratory tests and ultrasound of the affected [[joints]]. The European League Against Rheumatism/American College of Rheumatology collaborative initiative developed a provisional classification criteria for PMR. The following criteria are required for the diagnosis of PMR: age more than 50 years, bilateral [[shoulder pain]], and elevated [[C-reactive protein]] (CRP) and/or [[erythrocyte sedimentation rate]] (ESR).<ref name="pmid22388996">{{cite journal| author=Dasgupta B, Cimmino MA, Maradit-Kremers H, Schmidt WA, Schirmer M, Salvarani C et al.| title=2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative. | journal=Ann Rheum Dis | year= 2012 | volume= 71 | issue= 4 | pages= 484-92 | pmid=22388996 | doi=10.1136/annrheumdis-2011-200329 | pmc=PMC3298664 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22388996  }} </ref>
The diagnosis of polymyalgia rheumatica (PMR) is mostly clinical and it is supported with specific findings on laboratory tests and [[ultrasound]] of the affected [[joints]]. The European League Against Rheumatism/American College of Rheumatology collaborative initiative developed a provisional classification criteria for PMR. The following criteria are required for the diagnosis of PMR: age more than 50 years, bilateral [[shoulder pain]], and elevated [[C-reactive protein]] (CRP) and/or [[erythrocyte sedimentation rate]] (ESR).


===History and Symptoms===
===History and Symptoms===
PMR is typically characterized by symmetrical pain and morning stiffness in the proximal joints and limbs, including the [[neck]], the [[shoulder girdle]], the [[pelvic girdle]], the lower back, and the thighs. In some patients, there is involvement of the distal parts of the body such as peripheral [[synovitis]] or [[arthritis]]. Constitutional symptoms can also be present, and they include [[fever]], [[fatigue]], [[loss of appetite]], and [[weight loss]].<ref name="pmid22388996">{{cite journal| author=Dasgupta B, Cimmino MA, Maradit-Kremers H, Schmidt WA, Schirmer M, Salvarani C et al.| title=2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative. | journal=Ann Rheum Dis | year= 2012 | volume= 71 | issue= 4 | pages= 484-92 | pmid=22388996 | doi=10.1136/annrheumdis-2011-200329 | pmc=PMC3298664 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22388996  }} </ref>  There is an association between PMR and [[giant cell arteritis]] which can present with one or more of the following symptoms that include [[headaches]], scalp tenderness, [[jaw claudication]], [[fever]], or distorted vision.<ref name="pmid7598301">{{cite journal| author=Salvarani C, Gabriel SE, O'Fallon WM, Hunder GG| title=The incidence of giant cell arteritis in Olmsted County, Minnesota: apparent fluctuations in a cyclic pattern. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 3 | pages= 192-4 | pmid=7598301 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7598301  }} </ref><ref name="pmid7880191">{{cite journal| author=Salvarani C, Gabriel SE, O'Fallon WM, Hunder GG| title=Epidemiology of polymyalgia rheumatica in Olmsted County, Minnesota, 1970-1991. | journal=Arthritis Rheum | year= 1995 | volume= 38 | issue= 3 | pages= 369-73 | pmid=7880191 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7880191  }} </ref><ref name="pmid1629827">{{cite journal| author=Franzén P, Sutinen S, von Knorring J| title=Giant cell arteritis and polymyalgia rheumatica in a region of Finland: an epidemiologic, clinical and pathologic study, 1984-1988. | journal=J Rheumatol | year= 1992 | volume= 19 | issue= 2 | pages= 273-6 | pmid=1629827 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1629827  }} </ref>
Polymyalgia rheumatica (PMR) is typically characterized by symmetrical [[pain]] and morning stiffness in the proximal [[Joint|joints]] and [[Limb (anatomy)|limbs]], including the [[neck]], the [[shoulder girdle]], the [[pelvic girdle]], the [[lower back]], and the [[Thigh|thighs]]. In some patients, there is involvement of the distal parts of the body such as peripheral [[synovitis]] or [[arthritis]]. Constitutional symptoms can also be present, and they include [[fever]], [[fatigue]], [[loss of appetite]], and [[weight loss]]. There is an association between PMR and [[giant cell arteritis]] which can present with one or more of the following symptoms that include [[headaches]], scalp tenderness, [[jaw claudication]], [[fever]], or distorted vision.


==Physical Examination==
===Physical Examination===
Physical examination of patients with PMR reveals limitation of the active and passive range of motion of the affected joint. There is no true muscle weakness.<ref name="pmid18640460">{{cite journal| author=Salvarani C, Cantini F, Hunder GG| title=Polymyalgia rheumatica and giant-cell arteritis. | journal=Lancet | year= 2008 | volume= 372 | issue= 9634 | pages= 234-45 | pmid=18640460 | doi=10.1016/S0140-6736(08)61077-6 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18640460  }} </ref>
Physical examination of patients with polymyalgia rheumatica reveals limitation of the active and passive [[range of motion]] of the affected [[joint]]. There is no true [[muscle weakness]]. There are no changes in the [[Joint|joints]]. [[Ophthalmoscope|Ophthalmoscopic]] exams in patients with polymyalgia rheumatica associated with [[Temporal arteritis|giant cell arteritis]] might be abnormal.  


===Laboratory Findings===
===Laboratory Findings===
PMR is a clinical diagnosis that is supported by laboratory tests. Elevation in [[C-reactive protein]] (CRP) and/or [[erythrocyte sedimentation rate]] (ESR) is essential for the diagnosis of PMR.<ref name="pmid22388996">{{cite journal| author=Dasgupta B, Cimmino MA, Maradit-Kremers H, Schmidt WA, Schirmer M, Salvarani C et al.| title=2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative. | journal=Ann Rheum Dis | year= 2012 | volume= 71 | issue= 4 | pages= 484-92 | pmid=22388996 | doi=10.1136/annrheumdis-2011-200329 | pmc=PMC3298664 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22388996  }} </ref>
Polymyalgia rheumatica (PMR) is a clinical diagnosis that is supported by laboratory tests. Elevation of [[C-reactive protein]] (CRP) and/or [[erythrocyte sedimentation rate]] (ESR) is essential for the diagnosis of PMR.
 
=== Electrocardiogram ===
There are no EKG findings associated with polymyalgia rheumatica.
 
===X Ray===
There are no x ray findings associated with polymyalgia rheumatica.
 
=== Echocardiography and Ultrasound ===
There are no echocardiography findings associated with polymyalgia rheumatica. [[Ultrasound]] exam is important for the diagnosis of polymyalgia rheumatica (PMR). It can reveal evidence of [[bursitis]], [[synovitis]] or [[tenosynovitis]] in the affected areas.
 
===CT Scan===
There are no CT findings associated with polymyalgia rheumatica.


===MRI===
===MRI===
[[MRI]] is used for the assessment of [[bursitis]], [[synovitis]], and [[tenosynovitis]] among patients with PMR. [[MRI]] is more sensitive than [[ultrasonography]] for the evaluation of iliopsoas [[bursitis]] and hip [[synovitis]].<ref name="pmid16095113">{{cite journal |author=Cantini F, Niccoli L, Nannini C, ''et al.'' |title=Inflammatory changes of hip synovial structures in polymyalgia rheumatica |journal=Clin. Exp. Rheumatol. |volume=23 |issue=4 |pages=462–8 |year=2005 |pmid=16095113 |doi= |url=}}</ref>  A study has demonstrated that [[MRI]] of the shoulders facilitates the proper diagnosis in patients with the typical proximal symptoms of [[PMR]] with normal [[ESR]] values.<ref name="pmid11352249">{{cite journal| author=Cantini F, Salvarani C, Olivieri I, Niccoli L, Macchioni P, Boiardi L et al.| title=Inflamed shoulder structures in polymyalgia rheumatica with normal erythrocyte sedimentation rate. | journal=Arthritis Rheum | year= 2001 | volume= 44 | issue= 5 | pages= 1155-9 | pmid=11352249 | doi=10.1002/1529-0131(200105)44:5<1155::AID-ANR198>3.0.CO;2-N | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11352249  }} </ref>
[[MRI]] is used for the assessment of [[bursitis]], [[synovitis]], and [[tenosynovitis]] among patients with polymyalgia rheumatica (PMR). [[MRI]] is more sensitive than [[ultrasonography]] for the evaluation of iliopsoas [[bursitis]] and hip [[synovitis]]. A study has demonstrated that [[MRI]] of the shoulders facilitates the proper diagnosis in patients with the typical proximal symptoms of [[PMR]] with normal [[ESR]] values.


===Ultrasound===
===Other Imaging Findings===
Ultrasound exam is important for the diagnosis of PMR. It can reveal evidence of [[bursitis]], [[synovitis]] or [[tenosynovitis]] in the affected areas.<ref name="pmid22388996">{{cite journal| author=Dasgupta B, Cimmino MA, Maradit-Kremers H, Schmidt WA, Schirmer M, Salvarani C et al.| title=2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative. | journal=Ann Rheum Dis | year= 2012 | volume= 71 | issue= 4 | pages= 484-92 | pmid=22388996 | doi=10.1136/annrheumdis-2011-200329 | pmc=PMC3298664 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22388996  }} </ref>
There are no other imaging findings associated with polymyalgia rheumatica.


===Other Diagnostic Studies===
===Other Diagnostic Studies===
A [[muscle biopsy]] might be performed to differentiate PMR from other diseases. In addition, [[temporal artery]] biopsy is required when a subject with PMR have symptoms suggestive of [[giant cell arteritis]].
[[muscle biopsy]] might be performed to differentiate polymyalgia rheumatica (PMR) from other diseases. In addition, [[temporal artery]] biopsy is required when a subject with PMR have symptoms suggestive of [[giant cell arteritis]].
 
==Treatment==
 
===Medical Therapy===
The mainstay of treatment of polymyalgia rheumatica (PMR) is low dose [[glucocorticoids]], typically [[prednisone]] or [[prednisolone]]. The starting dose of the [[glucocorticoid]] treatment is 12.5-15 mg daily for 2 to 4 weeks after which the treatment should be slowly tapered. The average duration of the treatment with [[glucocorticoids]] is 1 to 2 years; nevertheless, longer [[corticosteroids]] regimens might be necessary among patients who experience relapse of the symptoms. Prophylaxis for [[osteoporosis]] with [[calcium]] and [[vitamin D]] should be started with the [[steroid]] therapy.


==Medical Therapy==
===Surgery===
The mainstay of treatment of PMR is low dose [[glucocorticoids]], typically [[prednisone]] or [[prednisolone]].  The starting dose of the [[glucocorticoid]] treatment is 15-20 mg daily for 2 to 4 weeks after which the treatment should be slowly tapered.  The average duration of the treatment with [[glucocorticoids]] is 1 to 2 years; nevertheless, longer [[corticosteroids]] regimens might be necessary among patients who experience relapse of the symptoms.  Prophylaxis for [[osteoporosis]] with [[calcium]] and [[vitamin D]] should be started with the [[steroid]] therapy.<ref name="pmid23051717">{{cite journal| author=Kermani TA, Warrington KJ| title=Polymyalgia rheumatica. | journal=Lancet | year= 2013 | volume= 381 | issue= 9860 | pages= 63-72 | pmid=23051717 | doi=10.1016/S0140-6736(12)60680-1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23051717  }} </ref>
Surgical intervention is not recommended for the management of polymyalgia rheumatica.


==References==
===Primary Prevention===
{{reflist|2}}
There are no established measures for the primary prevention of polymyalgia rheumatica.
 
===Secondary Prevention===
There are no established measures for the secondary prevention of polymyalgia rheumatica.


==External links==
==External links==
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* [http://www.rheumatology.org/public/factsheets/pmr_new2.asp Patient Education - Polymyalgia Rheumatica] - American College of Rheumatology
* [http://www.rheumatology.org/public/factsheets/pmr_new2.asp Patient Education - Polymyalgia Rheumatica] - American College of Rheumatology


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Latest revision as of 23:47, 29 July 2020

Polymyalgia Rheumatica Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Polymyalgia rheumatica from other Diseases

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Natural History, Complications and Prognosis

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Diagnostic Study of Choice

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Ayesha A. Khan, MD[3] Ujjwal Rastogi, MBBS [4]; Rim Halaby, M.D. [5]; Ahmed Elsaiey, MBBCH [6]

Overview

Polymyalgia rheumatica (PMR) is a chronic inflammatory disease that involves the articular and periarticular parts of the cervical region, shoulder girdle and pelvic girdle. PMR affects subjects over the age of 50 years and it is characterized by pain and stiffness in the neck, shoulders, upper arms, hip, and thighs. Although myalgia is one of the symptoms of PMR, there is no inflammation of the muscles; instead, PMR is a disease of the joint that causes synovitis. The diagnosis of PMR relies on the clinical findings and laboratory evidence of systemic inflammation. PMR is associated with giant cell arteritis. The cause of PMR is unknown but it has been suggested that both genetic and environmental factors are implicated. The mainstay of treatment of PMR is steroid therapy.

Historical Perspective

Polymyalgia rheumatica was first described in 1888 by Bruce William as "senile rheumatic gout". The disease was referred to as "polymyalgia rheumatica" by Stuart Barber in 1957 in his article entitled "myalgic syndrome with constitutional effects; polymyalgia rheumatica".

Classification

There is no established system for the classification of polymyalgia rheumatica.

Pathophysiology

Polymyalgia rheumatica (PMR) is a chronic inflammatory disease of the articular and periarticular structures of the cervical region, shoulder girdle and hip girdle. The underlying pathophysiology of PMR remains unknown. It has been hypothesized that genetic and environmental factors are implicated, particularly due to the seasonal and geographical differences in the prevalence of this disease. It has also been hypothesized that PMR is associated with infections such as parainfluenza virus type 1mycoplasma pneumoniaechlamydia pneumoniae, and parvovirus B19. In addition, histological examinations of synovial biopsies of affected individuals reveal mild synovitis with predominance of CD4 T cells and macrophages. Although myalgia is a symptom of PMR, there is no inflammation of the muscles.The senescence of the immune systems as demonstrated by the loss of the CD28 on CD4+ T senescent cells may be responsible for aberrant immune responses in PMR. Adaptive immune alterations also occurs in PMR mainly represented by the activation of Th17 cells, mainly driven by the increased IL-6 levels. An altered distribution and phenotype of B cells also occurs in PMR even in the absence of a clear autoimmune response. Local activation of myeloid and endothelial cells has been also demonstrated in the non-inflamed arteries and inflamed synovial tissues of PMR patients.

Causes

There is no specific cause for polymyalgia rheumatica. However, there are possible theories about the causes which include inflammatory joint lining attack, viral infections, and genetic inheritance of HLA-DR4.

Differentiating Polymyalgia Rheumatica from other Diseases

PMR must be differentiated from other conditions such as late onset rheumatoid arthritis, polymyositis, dermatomyositis, fibromyalgia, and remitting seronegative symmetrical synovitis with pitting edema.

Epidemiology and Demographics

Polymyalgia rheumatica (PMR) affects mostly subjects who are more than 50 years of age. The prevalence of PMR is highest among subjects from Scandinavian countries and those from northern European origin.

Risk Factors

Age, female sex, Scandinavia and northern Europe origin are risk factors for polymyalgia rheumatica. Other risk factors include smokingsun exposureinfections, and nulliparity.

Screening

There is insufficient evidence to recommend routine screening for polymyalgia rheumatica.

Natural History, Complications and Prognosis

Polymyalgia rheumatica (PMR) affects the quality of life of the patients. The pain and stiffness in the proximal joints might lead to sleep disturbance as well as an inability to do regular daily activities such as getting dressed and getting out of a chair. The symptoms begin rapidly and last for weeks. Once the steroid treatment is initiated, the symptoms resolve rapidly within few days. In fact, the rapid resolution of symptoms with the steroid therapy reinforces the diagnosis of PMR. The steroid treatment can be associated with complications such as weight gain and bone fracture. Approximately 40 to 50% of subjects experience a relapse of the symptoms. PMR is associated with giant cell arteritis.

Diagnosis

Diagnostic Study of Choice

The diagnosis of polymyalgia rheumatica (PMR) is mostly clinical and it is supported with specific findings on laboratory tests and ultrasound of the affected joints. The European League Against Rheumatism/American College of Rheumatology collaborative initiative developed a provisional classification criteria for PMR. The following criteria are required for the diagnosis of PMR: age more than 50 years, bilateral shoulder pain, and elevated C-reactive protein (CRP) and/or erythrocyte sedimentation rate (ESR).

History and Symptoms

Polymyalgia rheumatica (PMR) is typically characterized by symmetrical pain and morning stiffness in the proximal joints and limbs, including the neck, the shoulder girdle, the pelvic girdle, the lower back, and the thighs. In some patients, there is involvement of the distal parts of the body such as peripheral synovitis or arthritis. Constitutional symptoms can also be present, and they include feverfatigueloss of appetite, and weight loss. There is an association between PMR and giant cell arteritis which can present with one or more of the following symptoms that include headaches, scalp tenderness, jaw claudicationfever, or distorted vision.

Physical Examination

Physical examination of patients with polymyalgia rheumatica reveals limitation of the active and passive range of motion of the affected joint. There is no true muscle weakness. There are no changes in the jointsOphthalmoscopic exams in patients with polymyalgia rheumatica associated with giant cell arteritis might be abnormal.

Laboratory Findings

Polymyalgia rheumatica (PMR) is a clinical diagnosis that is supported by laboratory tests. Elevation of C-reactive protein (CRP) and/or erythrocyte sedimentation rate (ESR) is essential for the diagnosis of PMR.

Electrocardiogram

There are no EKG findings associated with polymyalgia rheumatica.

X Ray

There are no x ray findings associated with polymyalgia rheumatica.

Echocardiography and Ultrasound

There are no echocardiography findings associated with polymyalgia rheumatica. Ultrasound exam is important for the diagnosis of polymyalgia rheumatica (PMR). It can reveal evidence of bursitis, synovitis or tenosynovitis in the affected areas.

CT Scan

There are no CT findings associated with polymyalgia rheumatica.

MRI

MRI is used for the assessment of bursitissynovitis, and tenosynovitis among patients with polymyalgia rheumatica (PMR). MRI is more sensitive than ultrasonography for the evaluation of iliopsoas bursitis and hip synovitis. A study has demonstrated that MRI of the shoulders facilitates the proper diagnosis in patients with the typical proximal symptoms of PMR with normal ESR values.

Other Imaging Findings

There are no other imaging findings associated with polymyalgia rheumatica.

Other Diagnostic Studies

muscle biopsy might be performed to differentiate polymyalgia rheumatica (PMR) from other diseases. In addition, temporal artery biopsy is required when a subject with PMR have symptoms suggestive of giant cell arteritis.

Treatment

Medical Therapy

The mainstay of treatment of polymyalgia rheumatica (PMR) is low dose glucocorticoids, typically prednisone or prednisolone. The starting dose of the glucocorticoid treatment is 12.5-15 mg daily for 2 to 4 weeks after which the treatment should be slowly tapered. The average duration of the treatment with glucocorticoids is 1 to 2 years; nevertheless, longer corticosteroids regimens might be necessary among patients who experience relapse of the symptoms. Prophylaxis for osteoporosis with calcium and vitamin D should be started with the steroid therapy.

Surgery

Surgical intervention is not recommended for the management of polymyalgia rheumatica.

Primary Prevention

There are no established measures for the primary prevention of polymyalgia rheumatica.

Secondary Prevention

There are no established measures for the secondary prevention of polymyalgia rheumatica.

External links