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polymyalgia rheumatica (PMR)

Etiology: 1) unknown 2) frequently associated with osteoarthritis 3) may be associated with temporal arteritis 4) may be paraneoplastic syndrome [13] Epidemiology: 1) more frequent in women 2) more frequent in northern Europeans, rare in Asians Pathology: - large vessel vasculitis [5] Clinical manifestations: 1) onset of symptoms after 50 years of age 2) onset of symptoms may be insidious or abrupt 3) bilateral shoulder pain, neck pain & hip pain a) prolonged morning stiffness b) pain on range of motion in shoulders c) pain may be in associated muscles rather than in joints d) no crepitus with movement of shoulders 4) myalgias with disuse atrophy 5) no muscle tenderness [8]; + muscle tenderness [5] 6) true weakness is not present 7) joint effusions in knees (frequent) 8) proximal symptoms predominate but distal joints may also be involved [20] 9) generalized symptoms a) mild weight loss b) depression c) malaise d) fatigue e) anorexia f) low-grade fever 10) often attributed to 'getting old' by patients Laboratory: 1) complete blood count (CBC): - anemia of chronic inflammation may be present - MCV marginally low or low normal [5] 2) erythrocyte sedimentation rate (ESR) may be elevated - do not use ESR to adjust glucocorticoid dosage [5] 3) serum creatine kinase must be normal 4) rheumatoid factor (RF) is negative 5) antinuclear antibody (ANA) is negative 6) complement levels are normal 7) serum iron may be very low (10 ug/dL) out of proportion to moderately low TIBC (200 ug/dL) [5] 8) serum ferritin may be high 9) increased serum AST, serum ALT (example of 54 & 58 U/L respectively) [7] 10) muscle biopsies & joint aspirations are normal & should be avoided Special laboratory: - temporal artery biopsy to rule out giant cell arteritis (see giant cell arteritis) Radiology: - bone scans may be abnormal, but are not helpful - MRI to detect subacromial bursitis & subdeltoid bursitis if diagnosis is uncertain - CT angiography of neck & thorax if a) giant cell arteritis suspected, but bilateral temporal artery biopsy negative b) unexplained fever without symptoms of temporal arteritis [5] Complications: - excess risk for cardiovascular disease (HR=2.6) - excess risk similar for cardiac, cerebral & peripheral vascular events [12] Differential diagnosis: 1) giant cell arteritis* - presence of generalized symptoms - temporal tenderness, jaw claudication, low grade fever - failure to respond to low-dose (15 mg) of prednisone 2) fibromyalgia 3) myalgia of viral infection 4) rheumatoid arthritis 5) osteoarthritis - crepitus on movement of shoulders 6) bursitis 7) tendonitis 8) polymyositis/dermatomyositis: - proximal muscle weakness not due to pain - morning stiffness not a feature 9) malignancy - may be paraneoplastic syndrome [13] - increased serum AST & serum ALT suggest polymyalgia rheumatica or giant cell arteritis 10) depression 11) amyloidosis * constitutional symptoms (intermittent fever, weight loss) increases likelihood of giant cell arteritis, even in the absence of increased ESR, serum CRP, or classic giant cell arteritis symptoms, such as headache & jaw claudication [7] Management: 1) glucocorticoids (treatment of choice) a) prednisone 40-80 mg QD or 1 mg/kg/day divided BID-QID for suspected giant cell arteritis (headache, temporal tenderness, jaw claudication, failure to respond to low-dose (15 mg) of prednisone) b) prednisone 15 mg QD for 3-5 days, 10 mg QD for 1-2 weeks, 5 mg QD for 12 months, then taper 1 mg/month c) prednisone 10-20 mg QD for 4-6 weeks [7] - taper by 1 mg each month d) for recurrences, resume previous dose or 5 mg/day, whichever is greater - restart at 12.5-25 mg [22] e) clinical improvement generally within a few days f) do not use ESR to adjust glucocorticoid dosage [5] g) treatment failures usually secondary to: 1] incorrect diagnosis 2] underlying or co-existing condition - consider giant cell arteritis if failure to respond to 1st week of therapy [7] 2) methotrexate 10 mg QD plus folinic acid 7.5 mg QD may facilitate reduction in prednisone dose [5,6] 3) tocilizumab (8 mg/kg) every 4 weeks may facilitate reduction in prednisone dose [19] 4) sarilumab sustains remission & reduces total glucocorticoid use in relapsing polymyalgia rheumatica during glucocorticoid taper 5) non-steroidal anti-inflammatory agents (NSAIDs) a) rarely effective [1] b) may facilitate reduction in prednisone dose [2] 6) follow-up after 4-6 weeks then every 4-6 months. Visits every 1-2 months as prednisone is tapered from 5 mg. 7) watch for symptoms of temporal arteritis - in the absence of symptoms, temporal artery biopsy is not indicated 8) relapses common, duration of treatment typically 1-3 years [5]

Related

temporal arteritis; cranial arteritis; giant cell arteritis; Horton's disease

General

autoimmune disease vasculitis

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 796-97
  2. Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 527
  3. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 120
  4. Guide to Physical Examination & History Taking, 6th edition, Bates B, JB Lippincott, Philadelphia, 1995, pg 86-87
  5. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  6. Journal Watch 24(22):168, 2004 Caporali R, Cimmino MA, Ferraccioli G, Gerli R, Klersy C, Salvarani C, Montecucco C; Systemic Vasculitis Study Group of the Italian Society for Rheumatology. Prednisone plus methotrexate for polymyalgia rheumatica: a randomized, double-blind, placebo-controlled trial. Ann Intern Med. 2004 Oct 5;141(7):493-500. PMID: 15466766
  7. Geriatric Review Syllabus, 7th edition Parada JT et al (eds) American Geriatrics Society, 2010 - Geriatric Review Syllabus, 8th edition (GRS8) Durso SC and Sullivan GN (eds) American Geriatrics Society, 2013 - Geriatric Review Syllabus, 11th edition (GRS11) Harper GM, Lyons WL, Potter JF (eds) American Geriatrics Society, 2022
  8. Geriatrics at your Fingertips, 13th edition, 2011 Reuben DB et al (eds) American Geriatric Society
  9. 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. PMID: 12140303 - Salvarani C, Cantini F, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. Lancet. 2008 Jul 19;372(9634):234-45 PMID: 18640460
  10. Gonzalez-Gay MA, Vazquez-Rodriguez TR, Lopez-Diaz MJ et al Epidemiology of giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum. 2009 Oct 15;61(10):1454-61 PMID: 19790127
  11. Hernandez-Rodriguez J, Cid MC, Lopez-Soto A Treatment of polymyalgia rheumatica: a systematic review. Arch Intern Med. 2009 Nov 9;169(20):1839-50. Review. PMID: 19901135
  12. Hancock AT et al. Risk of vascular events in patients with polymyalgia rheumatica. CMAJ 2014 Jul 28 PMID: 25070989 http://www.cmaj.ca/content/early/2014/07/28/cmaj.140266
  13. Muller S et al. Is cancer associated with polymyalgia rheumatica? A cohort study in the General Practice Research Database. Ann Rheum Dis 2014 Oct; 73:1769. PMID: 23842460 http://ard.bmj.com/content/73/10/1769
  14. Kermani TA, Warrington KJ Polymyalgia rheumatica. Lancet. 2013 Jan 5;381(9860):63-72 PMID: 23051717
  15. Buttgereit F, Dejaco C, Matteson EL, Dasgupta B. Polymyalgia Rheumatica and Giant Cell Arteritis: A Systematic Review. JAMA. 2016 Jun 14;315(22):2442-58. Review. PMID: 27299619
  16. Gonzalez-Gay MA, Pina T. Giant cell arteritis and polymyalgia rheumatica: an update. Curr Rheumatol Rep. 2015 Feb;17(2):6. Review. PMID: 25618572
  17. 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. PMID: 26359488 Free Article
  18. Mahmood SB, Nelson E, Padniewski J et al. Polymyalgia rheumatica: an updated review. Cleve Clin J Med. 2020;87(9):549-556 https://www.ccjm.org/content/87/9/549
  19. Devauchelle-Pensec V, Carvajal-Alegria G, Dernis E et al Effect of Tocilizumab on Disease Activity in Patients With Active Polymyalgia Rheumatica Receiving Glucocorticoid Therapy. A Randomized Clinical Trial. JAMA. 2022;328(11):1053-1062. Sept 20 PMID: 36125471 https://jamanetwork.com/journals/jama/fullarticle/2796378 - Antiochos B Tocilizumab as a Novel Therapy for Steroid-Dependent Polymyalgia Rheumatica. JAMA. 2022;328(11):1047-1048. Sept 20 PMID: 36125486 https://jamanetwork.com/journals/jama/fullarticle/2796398
  20. Mahmood SB, Nelson E, Padniewski J, Nasr R. Polymyalgia rheumatica: An updated review. Cleve Clin J Med. 2020 Aug 31;87(9):549-556. PMID: 32868305 Free article. Review.
  21. Spiera RF et al. Sarilumab for relapse of polymyalgia rheumatica during glucocorticoid taper. N Engl J Med 2023 Oct 5; 389:1263. PMID: 37792612 https://www.nejm.org/doi/10.1056/NEJMoa2303452