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Polymyalgia Rheumatica

  • Author: Ehab R Saad, MD, MA, FACP, FASN; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Sep 23, 2015
 

Background

Polymyalgia rheumatica (PMR) is a relatively common chronic inflammatory condition of unknown etiology that affects elderly individuals. It is characterized by proximal myalgia of the hip and shoulder girdles with accompanying morning stiffness that lasts for more than 1 hour. Approximately 15% of patients with PMR develop giant cell arteritis (GCA), and 40-50% of patients with GCA have associated PMR. Despite the similarities of age at onset and some of the clinical manifestations, the relationship between GCA and PMR is not yet clearly established.[1]

PMR is a clinical diagnosis based on the complex of presenting symptoms and exclusion of the other potential diseases (see Clinical and Workup.) Corticosteroids are considered the treatment of choice, and a rapid response to low-dose corticosteroids is considered pathognomonic (see Treatment). Patients have an excellent prognosis, although exacerbations may occur if steroids are tapered too rapidly, and relapse is common (see Prognosis).

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Pathophysiology

The cause of PMR is unknown. PMR is closely linked to giant cell arteritis (GCA, temporal arteritis), although it is controversial whether GCA and PMR are two separate diseases or part of the same spectrum of disease. One hypothesis is that in a genetically predisposed patient, an environmental factor, possibly a virus, causes monocyte activation, which helps determine the production of cytokines that induce manifestations characteristic of PMR and GCA. However, although several infectious agents have been investigated as possible triggers, results are inconclusive.[2]

Immunogenetic studies support a polygenic basis for GCA and PMR. Occurrence in siblings and increased prevalence in those of Northern European heritage suggest a genetic role in the pathophysiology of the disease. Although most studies confirm an association between HLA-DRB1*04 alleles and GCA, the strength of this association with PMR varies between different populations. Interleukin (IL)–1 and tumor necrosis factor (TNF)–alpha gene polymorphisms have weak association with GCA and PMR. In Spain, an IL-6 polymorphism was associated with the expression of PMR symptoms in GCA patients. Additionally, in this Spanish population, the RANTES polymorphism was associated with PMR and not GCA.[3]

Pathologically, GCA and PMR are similar, with the exception of the absence of significant vascular involvement in pure PMR. Synovitis, bursitis, and tenosynovitis around the joints, especially the shoulders, hips, knees, metacarpal phalangeal joints, and wrists, are seen in PMR. Inflammation is thought to start within the synovium and bursae, with recognition of an unknown antigen by dendritic cells or macrophages.[4]

Systemic macrophage and T-cell activation are characteristic of both GCA and PMR. Patients often have an elevated IL-6 level which is likely responsible for the systemic inflammatory response in both GCA and PMR. Most studies in PMR show that a decrease in the level of circulating IL-6 correlates with remission of clinical symptoms. Data on other circulating cytokines (eg, IL-1, IL-2, TNF-alpha, IL-10) are too scant to draw any conclusions. However, recent studies do show that interferon-gamma (IFN-γ) is expressed in nearly 70% of temporal artery biopsy samples from patients with GCA but is not detected in patients with isolated PMR, suggesting IFN-γ may be crucial to the development of GCA.[2, 3, 5, 6]

Although PMR causes severe pain and stiffness in the proximal muscle groups, no evidence of disease is present on muscle biopsy. Muscle strength and electromyographic findings are normal. Instead, the inflammation is at the level of the synovium and bursae, with MRI studies revealing periarticular inflammation as well as bursitis in the bursae associated with both the shoulder and hip girdles.[7, 8]

Some evidence suggests the presence of cell-mediated injury to the elastic lamina in the blood vessels in the affected muscle groups. A prospective study of 35 patients with isolated PMR noted vascular [18 F] fluorodeoxyglucose positron emission tomography (FDG-PET) imaging at diagnosis in 31% of patients, predominately at the subclavian arteritis, but at a much lower intensity than in GCA patients. Increased FDG uptake in the shoulders was seen in 95% of the patients, in the hips in 89%, and in the spinous processes of the cervical and lumbar vertebrae (correlating with interspinous bursitis) of 51% of the patients with isolated PMR.[9]

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Etiology

The exact cause (or causes) of PMR is unknown. The disease is more common among northern Europeans, which may indicate a genetic predisposition. Other risk factors for PMR are an age of 50 years or older and the presence of GCA.

An autoimmune process may play a role in PMR development. PMR is associated with the HLA-DR4 haplotype. High level of IL-6 is associated with increased disease activity.

Many investigators believe that nonerosive synovitis and tenosynovitis are responsible for many symptoms of PMR.

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Epidemiology

In the United States, the average annual incidence of polymyalgia rheumatica (PMR) is 52.5 cases per 100,000 persons aged 50 years and older. The prevalence is approximately 0.5-0.7%.

Worldwide, the frequency varies by country. In Europe, the frequency decreases from north to south, with a high incidence in Scandinavia and a low incidence in Mediterranean countries. In Italy, for example, the incidence is 12.7 cases per 100,000 persons.

Whites are affected more than other ethnic groups. PMR is only occasionally reported in African-American persons. PMR is twice as common in females.

The incidence increases with advanced age. PMR rarely affects persons younger than 50 years. The median age at diagnosis is 72 years.[10]

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Contributor Information and Disclosures
Author

Ehab R Saad, MD, MA, FACP, FASN Associate Professor, Department of Medicine, Medical College of Wisconsin

Ehab R Saad, MD, MA, FACP, FASN is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Society of Nephrology, American Society of Transplantation, International Society for Peritoneal Dialysis, National Kidney Foundation

Disclosure: Nothing to disclose.

Coauthor(s)

Gloria Fioravanti, DO Clinical Assistant Professor, Program Director, Department of Internal Medicine, St Luke's Hospital of Bethlehem, Temple University School of Medicine

Gloria Fioravanti, DO is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Osteopathic Association

Disclosure: Nothing to disclose.

Allen Samuels, MD 

Allen Samuels, MD is a member of the following medical societies: American College of Rheumatology

Disclosure: Nothing to disclose.

Patricia J Papadopoulos, MD Staff Rheumatologist, MultiCare Rheumatology Specialists

Patricia J Papadopoulos, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American College of Rheumatology

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Acknowledgements

The views expressed in this article are those of the authors and do not reflect the official policy of the Department of the Army, Department of Defense, or the US Government. Additionally, this publication does not imply the Federal or Department of Defense endorsement of any product.

Past Contributors

Michael S Beeson, MD, MBA, FACEP Professor of Emergency Medicine, Northeastern Ohio Universities College of Medicine and Pharmacy; Attending Faculty, Akron General Medical Center

Michael S Beeson, MD, MBA, FACEP is a member of the following medical societies: American College of Emergency Physicians, Council of Emergency Medicine Residency Directors, National Association of EMS Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Gino A Farina, MD, FACEP, FAAEM Associate Professor of Clinical Emergency Medicine, Albert Einstein College of Medicine; Program Director, Department of Emergency Medicine, Long Island Jewish Medical Center

Gino A Farina, MD, FACEP, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Center for the Advancement of Women's Health and Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

Geofrey Nochimson, MD Consulting Staff, Department of Emergency Medicine, Sentara Careplex Hospital

Geofrey Nochimson, MD is a member of the following medical societies: American College of Emergency Physicians

Disclosure: Nothing to disclose.

Robert E O'Connor, MD, MPH Professor and Chair, Department of Emergency Medicine, University of Virginia Health System

Robert E O'Connor, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American College of Physician Executives, American Heart Association, American Medical Association, Medical Society of Delaware, National Association of EMS Physicians, Society for Academic Emergency Medicine, and Wilderness Medical Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References
  1. Gonzalez-Gay MA, Barros S, Lopez-Diaz MJ, Garcia-Porrua C, Sanchez-Andrade A, Llorca J. Giant cell arteritis: disease patterns of clinical presentation in a series of 240 patients. Medicine (Baltimore). 2005 Sep. 84(5):269-76. [Medline].

  2. Gonzalez-Gay MA, Vazquez-Rodriguez TR, Lopez-Diaz MJ, Miranda-Filloy JA, Gonzalez-Juanatey C, Martin J, et al. Epidemiology of giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum. 2009 Oct 15. 61(10):1454-61. [Medline].

  3. Ghosh P, Borg FA, Dasgupta B. Current understanding and management of giant cell arteritis and polymyalgia rheumatica. Expert Rev Clin Immunol. 2010 Nov. 6(6):913-28. [Medline].

  4. Caylor TL, Perkins A. Recognition and management of polymyalgia rheumatica and giant cell arteritis. Am Fam Physician. 2013 Nov 15. 88(10):676-84. [Medline].

  5. Salvarani C, Cantini F, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. Lancet. 2008 Jul 19. 372(9634):234-45. [Medline].

  6. Martinez-Taboada VM, Alvarez L, RuizSoto M, Marin-Vidalled MJ, Lopez-Hoyos M. Giant cell arteritis and polymyalgia rheumatica: role of cytokines in the pathogenesis and implications for treatment. Cytokine. 2008 Nov. 44(2):207-20. [Medline].

  7. Gonzalez-Gay MA, Garcia-Porrua C, Miranda-Filloy JA, Martin J. Giant cell arteritis and polymyalgia rheumatica: pathophysiology and management. Drugs Aging. 2006. 23(8):627-49. [Medline].

  8. Wilke WS. The role of imaging in polymyalgia rheumatica/giant cell arteritis. Skeletal Radiol. 2008 Sep. 37(9):779-83. [Medline].

  9. Blockmans D. PET in vasculitis. Ann N Y Acad Sci. 2011 Jun. 1228:64-70. [Medline].

  10. Salvarani C, Cantini F, Boiardi L, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med. 2002 Jul 25. 347(4):261-71. [Medline].

  11. Healey LA. Long-term follow-up of polymyalgia rheumatica: evidence for synovitis. Semin Arthritis Rheum. 1984 May. 13(4):322-8. [Medline].

  12. Macchioni P, Boiardi L, Catanoso M, Pazzola G, Salvarani C. Performance of the new 2012 EULAR/ACR classification criteria for polymyalgia rheumatica: comparison with the previous criteria in a single-centre study. Ann Rheum Dis. 2013 Dec 2. [Medline].

  13. Dasgupta B, Cimmino MA, Maradit-Kremers H, Schmidt WA, Schirmer M, Salvarani C, et al. 2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative. Ann Rheum Dis. 2012 Apr. 71(4):484-92. [Medline].

  14. Salvarani C, Cantini F, Macchioni P, Olivieri I, Niccoli L, Padula A, et al. Distal musculoskeletal manifestations in polymyalgia rheumatica: a prospective followup study. Arthritis Rheum. 1998 Jul. 41(7):1221-6. [Medline].

  15. [Guideline] Dejaco C, Singh YP, Perel P, et al. 2015 Recommendations for the management of polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative. Ann Rheum Dis. 2015 Oct. 74 (10):1799-807. [Medline]. [Full Text].

  16. Cantini F, Salvarani C, Olivieri I, et al. Erythrocyte sedimentation rate and C-reactive protein in the evaluation of disease activity and severity in polymyalgia rheumatica: a prospective follow-up study. Semin Arthritis Rheum. 2000 Aug. 30(1):17-24. [Medline].

  17. van der Geest KS, Abdulahad WH, Rutgers A, Horst G, Bijzet J, Arends S, et al. Serum markers associated with disease activity in giant cell arteritis and polymyalgia rheumatica. Rheumatology (Oxford). 2015 Aug. 54 (8):1397-402. [Medline].

  18. Ochi J, Nozaki T, Okada M, Suyama Y, Kishimoto M, Akaike G, et al. MRI findings of the shoulder and hip joint in patients with polymyalgia rheumatica. Mod Rheumatol. 2015 Sep. 25 (5):761-7. [Medline].

  19. Matteson EL, Maradit-Kremers H, Cimmino MA, Schmidt WA, Schirmer M, Salvarani C, et al. Patient-reported Outcomes in Polymyalgia Rheumatica. J Rheumatol. 2012 Apr. 39(4):795-803. [Medline].

  20. Kermani TA, Warrington KJ. Lower extremity vasculitis in polymyalgia rheumatica and giant cell arteritis. Curr Opin Rheumatol. 2011 Jan. 23(1):38-42. [Medline]. [Full Text].

  21. Hernández-Rodríguez J, Cid MC, López-Soto A, Espigol-Frigolé G, Bosch X. Treatment of polymyalgia rheumatica: a systematic review. Arch Intern Med. 2009 Nov 9. 169(20):1839-50. [Medline].

  22. Dejaco C, Duftner C, Cimmino MA, et al. Definition of remission and relapse in polymyalgia rheumatica: data from a literature search compared with a Delphi-based expert consensus. Ann Rheum Dis. 2011 Mar. 70(3):447-53. [Medline]. [Full Text].

  23. Leeb BF, Bird HA, Nesher G, et al. EULAR response criteria for polymyalgia rheumatica: results of an initiative of the European Collaborating Polymyalgia Rheumatica Group (subcommittee of ESCISIT). Ann Rheum Dis. 2003 Dec. 62(12):1189-94. [Medline]. [Full Text].

  24. Gabriel SE, Sunku J, Salvarani C, O'Fallon WM, Hunder GG. Adverse outcomes of antiinflammatory therapy among patients with polymyalgia rheumatica. Arthritis Rheum. 1997 Oct. 40(10):1873-8. [Medline].

  25. van der Veen MJ, Dinant HJ, van Booma-Frankfort C, van Albada-Kuipers GA, Bijlsma JW. Can methotrexate be used as a steroid sparing agent in the treatment of polymyalgia rheumatica and giant cell arteritis?. Ann Rheum Dis. 1996 Apr. 55(4):218-23. [Medline]. [Full Text].

  26. Caporali R, Cimmino MA, Ferraccioli G, Gerli R, Klersy C, Salvarani C, et al. Prednisone plus methotrexate for polymyalgia rheumatica: a randomized, double-blind, placebo-controlled trial. Ann Intern Med. 2004 Oct 5. 141(7):493-500. [Medline].

  27. Ferraccioli G, Salaffi F, De Vita S, Casatta L, Bartoli E. Methotrexate in polymyalgia rheumatica: preliminary results of an open, randomized study. J Rheumatol. 1996 Apr. 23(4):624-8. [Medline].

  28. Salvarani C, Macchioni P, Manzini C, et al. Infliximab plus prednisone or placebo plus prednisone for the initial treatment of polymyalgia rheumatica: a randomized trial. Ann Intern Med. 2007 May 1. 146(9):631-9. [Medline].

  29. De Silva M, Hazleman BL. Azathioprine in giant cell arteritis/polymyalgia rheumatica: a double-blind study. Ann Rheum Dis. 1986 Feb. 45(2):136-8. [Medline]. [Full Text].

  30. González-Gay MA, García-Porrúa C, Vázquez-Caruncho M, Dababneh A, Hajeer A, Ollier WE. The spectrum of polymyalgia rheumatica in northwestern Spain: incidence and analysis of variables associated with relapse in a 10 year study. J Rheumatol. 1999 Jun. 26(6):1326-32. [Medline].

 
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