Polymyalgia Rheumatica Treatment & Management
- Author: Ehab R Saad, MD, MA, FACP, FASN; Chief Editor: Herbert S Diamond, MD more...
Polymyalgia rheumatica (PMR) is a chronic, self-limited disorder. Therapy is based on empiric experiences because few randomized clinical trials are available to guide treatment decisions. The therapeutic goals are to control painful myalgia, to improve muscle stiffness, and to resolve constitutional features of the disease.
Corticosteroids (ie, prednisone) are considered the treatment of choice because they often cause complete or near-complete symptom resolution and reduction of the erythrocyte sedimentation rate (ESR) to normal. However, no definite evidence demonstrates that corticosteroids (or any other therapy) alter the natural history of PMR. Before the corticosteroid era, patients with PMR occasionally experienced spontaneous improvements, and musculoskeletal symptoms were treated with nonsteroidal anti-inflammatory drugs (NSAIDs). The low-dose corticosteroids used in PMR are almost certainly ineffective in the prevention of vasculitis progression.
Joint guidelines from the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) conditionally recommend starting corticosteroid therapy with 12.5-25 mg/day of prednisone or the equivalent. A systematic examination of the peer-reviewed literature, which included 30 studies, found that remission of PMR seemed to be achieved for most patients with a starting dose of prednisone at 15 mg/day. A slow tapering of the prednisone, less than 1 mg/month, was associated with fewer relapses. Once prednisone is tapered to 10 mg/day, a slow taper by 1 mg every 2 months until treatment discontinuation was associated with optimal control of disease activity.
Nevertheless, controversy remains regarding dose and duration of treatment. Dose depends on the patient's weight and severity of symptoms. Expect prompt relief of symptoms within 24-72 hours. Corticosteroid dose should be increased if symptoms are not well controlled within 1 week, and a diagnosis of giant cell arteritis may need to be pursued, especially if prednisone 20 mg/d does not control symptoms. In contrast to other rheumatic diseases, alternate-day administration of corticosteroids in PMR has largely been unsuccessful.
Tapering should be guided by clinical response to include decreased pain and stiffness, decreased morning stiffness, and decreased shoulder pain/limitation on clinical examination.[22, 23] Normalization of inflammatory markers is also helpful but should not set the guidelines for decreasing or stopping treatment. Patient-reported outcomes including global pain, hip pain, morning stiffness, physical function, mental function, as well as inflammatory markers have been reported as the best measures of disease activity and response to treatment.
If not contraindicated, NSAIDs may provide supplemental pain relief. However, per a study of 232 patients with PMR by Gabriel and colleagues, NSAIDs are associated with considerable drug-related morbidity and thus should be used with caution.
Corticosteroid-sparing agents are sometimes considered in patients with PMR to reduce corticosteroid-related adverse effects, especially in certain patient populations such as diabetic patients or in those who develop osteonecrosis.
Methotrexate has been investigated in three randomized studies in newly diagnosed PMR. One study used methotrexate at 7.5 mg/week plus 20 mg/day of prednisone and found no benefit in outcomes after 2 years of follow-up. Another study used oral and intramuscular methotrexate at a higher dose of 10 mg/week added to the prednisone regimen versus prednisone regimen alone. Overall, the patients receiving methotrexate 10 mg/week plus prednisone experienced corticosteroid-sparing effects compared with patients receiving prednisone alone.[26, 27]
Tumor necrosis factor alpha (TNF-α) inhibitors have also been investigated as corticosteroid-sparing agents in PMR. A randomized study with infliximab revealed no benefit. The only randomized trial using azathioprine (150 mg/d) during the maintenance phase of PMR showed a high frequency of adverse drug effects and a high number of patient withdrawals from the study, although a lower cumulative dose of corticosteroid at 52 weeks. At this time, the small number of completers and the high number of giant cell arteritis patients in the study make the study results difficult to interpret. EULAR/ACR guidelines strongly recommend against the use of TNF-α inhibitors in PMR.
Symptomatic palliation of pain with analgesic therapy alone with close monitoring may be preferable in patients with intolerable adverse effects from corticosteroids (eg, uncontrolled diabetes mellitus, severe symptomatic osteoporosis, psychosis).
Ophthalmologists, pathologists, and surgeons may be consulted on an as-needed basis should concern arise about the development of GCA.
Calcium and vitamin D supplementation should be initiated in all patients with PMR who are starting corticosteroid therapy.
Generally, activity restriction is unnecessary. Physical therapy is recommended for those with difficulty achieving good mobility despite adequate medical therapy.
Consultations and Long-Term Monitoring
Diagnosis and treatment involve the primary care physician and rheumatologist. In coordination with the primary care physician, the rheumatologist plays an important role in the diagnosis, treatment, and follow-up care. Involvement of primary care providers is imperative, to assist with the management of comorbidities such as prophylaxis for cardiovascular disease, glucocorticoid-induced hyperglycemia, and osteoporosis. Appropriate immunizations should be administered, ideally before corticosteroid therapy is initiated.
PMR is typically treated in an outpatient setting. Objective means of determining prognosis and decision concerning duration of treatment remain empiric and patients often need careful supervision.
Patients receiving steroids should have monthly follow-up, with regular monitoring of inflammatory markers. An isolated increase of ESR without symptoms during the course of treatment is not a valid reason to increase corticosteroid dose; however, a temporary delay in dosage reduction may be necessary. After steroid tapering, follow-up can be performed quarterly.
The risk of vertebral fractures is five times greater in women with polymyalgia rheumatica. A baseline bone mineral density study (eg, dual-energy x-ray absorptiometry [DEXA] scan) is recommended at the onset of treatment. As most patients require corticosteroids for at least 1-2 years, bisphosphonate therapy is recommended to prevent corticosteroid-induced osteoporosis.
Because relapses are more likely to occur during the initial 18 months of therapy and within 1 year of corticosteroid withdrawal, with a frequency of approximately 50%, all patients should be monitored for symptom of recurrence throughout corticosteroid tapering and for 12 months after cessation of therapy.
Relapses usually occur when the dose of prednisone is less than 5.0-7.5 mg/day or after therapy has been discontinued. If symptoms recur, the corticosteroid dose should be increased to the dose that previously controlled the symptoms. Recurrences of the disease more than 1 year after discontinuation of corticosteroid therapy has been reported.
Approximately 50-75% of patients can discontinue corticosteroid therapy after 2 years of treatment. However, some patients may require low doses of corticosteroids for several years.
Patients with PMR should be monitored regularly and carefully for symptoms and signs suggestive of GCA development.
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].
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].
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].
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].
Salvarani C, Cantini F, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. Lancet. 2008 Jul 19. 372(9634):234-45. [Medline].
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].
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].
Wilke WS. The role of imaging in polymyalgia rheumatica/giant cell arteritis. Skeletal Radiol. 2008 Sep. 37(9):779-83. [Medline].
Blockmans D. PET in vasculitis. Ann N Y Acad Sci. 2011 Jun. 1228:64-70. [Medline].
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].
Healey LA. Long-term follow-up of polymyalgia rheumatica: evidence for synovitis. Semin Arthritis Rheum. 1984 May. 13(4):322-8. [Medline].
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].
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].
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].
[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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].