Guideline sources The following summarized guidelines for the evaluation and management of polymyalgia rheumatica (PMR) are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023), the European League Against Rheumatism (EULAR 2022), the Italian Society of Rheumatology (ISR 2020), and the European League Against Rheumatism (EULAR/ACR 2015). 1 2 3 4 5 6 7 7 7 Definition PMR is a chronic, systemic inflammatory disorder associated with synovitis, tenosynovitis, and bursitis of proximal large joints. 5 Epidemiology The exact cause of PMR remains unknown. Genetic polymorphisms in intercellular adhesion molecules and interleukins have been associated with an increased risk. Epidemiological studies have suggested that infectious triggers (Mycoplasma pneumoniae, parvovirus B19, Chlamydia pneumoniae) may have a role in disease pathogenesis. 7 Pathophysiology https://web.pathway.md/diseases/recYBnaWiR1R0zakk 1/8 6/29/23, 11:19 PM Polymyalgia rheumatica Pathway The incidence of PMR in the United States is 63.9 cases per 100,000 person-years. 6 Disease course PMR is associated with predominant inflammation of periarticular structures (bursitis, tenosynovitis), although synovitis has also been reported. Hallmark clinical features include pain and stiffness involving the neck or torso, shoulders or proximal regions of the arms, and hips or proximal aspects of the thighs. Although myalgias are prominent symptoms in patients with PMR, muscle inflammation is not typically seen. 7 Prognosis and risk of recurrence Symptoms of PMR typically respond rapidly to low-dose prednisone or prednisolone (20 mg or less). Patients with PMR have reduced quality of life at time of diagnosis, which improves with treatment. Overall survival is similar to that of the general population. 7 Calculator Pathway Classification criteria for polymy Polymyalgia rheumatica Investigation and management Guidelines 1. Screening and diagnosis Differential diagnosis: adopt a safe and specific approach to exclude relevant mimicking or associated conditions, such as non-inflammatory, inflammatory (giant cell arteritis or rheumatoid arthritis), drug-induced, endocrine, infectious and neoplastic, to ascertain the PMR case definition in the presence of sufficient clinical findings. B 2. Classification and risk stratification Risk factors: insufficient evidence to define the role of risk factors for relapse/prolonged therapy in patients with PMR. I 3. Diagnostic investigations Clinical evaluation: assess for and exclude relevant mimicking conditions (non-inflammatory, inflammatory, drug-induced, endocrine, infective, and neoplastic) as part of the initial evaluation of patients with PMR. Laboratory tests: As per ISR 2020 guidelines: Obtain the following tests in patients with PMR before initiating treatment and to exclude mimicking conditions and establish a baseline for monitoring of therapy: https://web.pathway.md/diseases/recYBnaWiR1R0zakk 2/8 6/29/23, 11:19 PM Polymyalgia rheumatica Pathway CBC CRP and/or ESR glucose creatinine LFTs RF ACPA bone profile (including calcium, ALP) urinalysis. B Consider obtaining the following additional tests in patients with PMR: protein electrophoresis TSH CK. C As per EULAR 2015 guidelines, obtain the following tests as part of the initial evaluation of patients with PMR, before the initiation of treatment: CBC CRP and/or ESR glucose creatinine LFTs RF ACPA bone profile urinalysis. Diagnostic imaging: Consider obtaining CXR, abdominal ultrasound, and bone densitometry in patients with PMR at the discretion of the physician. C Consider obtaining FDG positron emission tomography/CT in a specialist setting, recognizing that insufficient evidence to define its clinical impact on the management of patients with PMR. C Evaluation for comorbidities: As per ISR 2020 guidelines, consider determining comorbidities in patients with PMR, particularly hypertension, diabetes, glucose intolerance, CVD, dyslipidemia, peptic ulcer, osteoporosis (and particularly recent fractures), presence of cataract or (risk factors for) glaucoma, presence of chronic or recurrent infections. C As per EULAR 2015 guidelines, screen for comorbidities in patients with PMR, in particular: diabetes or glucose intolerance, hypertension, CVD, dyslipidemia, peptic ulcer disease, osteoporosis, recent fractures, presence of cataract or risk factors for glaucoma, presence of chronic or recurrent infections, co-medication with NSAIDs or other relevant medications, risk factors for steroid-related side effects. https://web.pathway.md/diseases/recYBnaWiR1R0zakk 3/8 6/29/23, 11:19 PM Polymyalgia rheumatica Pathway Screening for chronic infections: screen for HBV infection in all patients eligible for treatment with conventional synthetic, biological, or targeted synthetic DMARDs, immunosuppressants, or corticosteroids (according to dose and duration). B Show 3 more 4. Medical management General principles: As per ISR 2020 guidelines: Treat patients with PMR aiming to provide the best care based on a shared decision by the patient and treating physician. B Provide an individualized management plan in patients with PMR. B As per EULAR 2015 guidelines, provide an individualized management plan in patients with PMR. Take into consideration patient perspective and preferences in the choice of initial corticosteroid dose and subsequent tapering schedules. Corticosteroids, indications: As per ISR 2020 guidelines, prefer corticosteroids over NSAIDs in patients with PMR. B Show 3 more As per ACR/EULAR 2015 guidelines, administer corticosteroids instead of NSAIDs for the treatment of patients with PMR, with the exception of possible short-term use of NSAIDs for other pain-related conditions. A Corticosteroids, dosing and duration: As per ISR 2020 guidelines, administer individualized daily dose of the initial corticosteroid therapy at the minimum effective dose within a range of 12.5-25 mg prednisone equivalent per day. B Show 5 more As per ACR/EULAR 2015 guidelines, consider using the minimum effective corticosteroid dose within a range of 12.5-25 mg prednisone (or equivalent) daily as the initial treatment for PMR. C Show 5 more Corticosteroids, tapering: As per ISR 2020 guidelines, individualize the dose tapering based on regular monitoring of the disease activity, laboratory markers and adverse events. B Show 3 more As per ACR/EULAR 2015 guidelines, follow an individualized corticosteroid tapering schedule, predicated to regular monitoring of patient disease activity, laboratory markers and adverse events. Consider following the following principles of corticosteroid dose tapering: Situation Guidance Taper dose to an oral dose of 10 mg/day prednisone equivalent within 4-8 weeks Initial tapering Treatment of relapse https://web.pathway.md/diseases/recYBnaWiR1R0zakk 4/8 6/29/23, 11:19 PM Polymyalgia rheumatica Pathway Increase oral prednisone to the pre- relapse dose and decrease it gradually to the dose at which the relapse occurred Taper daily oral prednisone by 1 mg every 4 weeks until discontinuation, as long as remission is maintained. A Tapering once remission is achieved following initial and relapse therapies Methotrexate: As per ISR 2020 guidelines: Consider administering methotrexate early in addition to corticosteroids particularly in patients with: high risk of relapse and/or prolonged therapy risk factors comorbidities and/or concomitant medications high risk of corticosteroid-related adverse events. B Consider administering methotrexate in patients with a relapse or experiencing corticosteroid-related adverse events. B As per EULAR 2015 guidelines: Consider introducing methotrexate early (7.5-10 mg/week), in addition to corticosteroids, in patients at high risk for relapse and in patients in whom corticosteroid-related adverse events are more likely to occur. C Consider initiating methotrexate in patients: with clinical relapse without significant response to corticosteroids with corticosteroid-related adverse effects. C Nonsteroidal anti-inflammatory drugs: Consider administering short-term NSAIDs and/or analgesics in patients with PMR experiencing pain related to other conditions. C Recognize the issue of co-medication with NSAIDs and other relevant medications in the management of patients with PMR. B Biological agents: As per ISR 2020 guidelines: Do not use TNF inhibitors for the treatment of patients with PMR. D Insufficient evidence to recommend biological agents, including IL-6 inhibitors for the treatment of patients with PMR. I As per EULAR 2015 guidelines, do not use TNF inhibitors for the treatment of patients with PMR. D 5. Nonpharmacologic interventions Exercise programs: https://web.pathway.md/diseases/recYBnaWiR1R0zakk 5/8 6/29/23, 11:19 PM Polymyalgia rheumatica Pathway As per ISR 2020 guidelines, consider offering individualized exercise programs in patients with PMR aiming to maintain muscular mass and function and reduce risk of falls, especially in older patients on long-term corticosteroids as well as in frail patients. C As per EULAR 2015 guidelines, consider offering individualized exercise programs in patients with PMR aiming to maintain the muscle mass and function, and reduce the risk of falls, especially in frail elderly patients on long-term corticosteroids. C Herbal products: do not use Chinese herbal preparations Yanghe and Biqi in patients with PMR. D 6. Patient education Patient education: provide patients with PMR access to education focusing on the impact and treatment of the disease (including disease and disease predictors) and advice on individually- tailored exercise programs. B 7. Preventative measures Routine immunizations: consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications. C Show 17 more Prophylaxis for Pneumocystis jirovecii pneumonia: consider administering prophylaxis against P. jirovecii pneumonia in patients initiating high-dose corticosteroids, especially in combination with immunosuppressants and depending on the risk-benefit ratio. C 8. Follow-up and surveillance Indications for referral: As per ISR 2020 guidelines, consider referring patients with PMR for specialist consultation, particularly in the following situations: atypical presentation such as peripheral inflammatory arthritis, systemic symptoms, low inflammatory markers, age of < 60 years experience of or high risk of therapy-related side effects refractory to corticosteroid therapy relapses/prolonged therapy. C As per EULAR 2015 guidelines, consider referring patients with PMR for specialist evaluation, particularly in the following situations: patients with atypical presentation (such as peripheral inflammatory arthritis, systemic symptoms, low inflammatory markers, age < 60 years) high risk or occurence of therapy-related side effects PMR refractory to corticosteroid therapy https://web.pathway.md/diseases/recYBnaWiR1R0zakk 6/8 6/29/23, 11:19 PM Polymyalgia rheumatica Pathway occurence of relapses or need for prolonged therapy. Follow-up: As per ISR 2020 guidelines, monitor patients with PMR treated in primary or secondary care for: risk factors and evidence for steroid-related side effects comorbidities other relevant medications evidence and risk factors for relapse/prolonged therapy. B Show 2 more As per EULAR 2015 guidelines: Monitor the following elements during follow-up of patients with PMR: risk factors and evidence for steroid-related side effects comorbidities other relevant medications evidence and risk factors for relapse/prolonged therapy. Schedule follow-up: Situation Guidance Every 4-8 weeks First year Every 8-12 weeks Second year As indicated. Relapse or as prednisone is tapered and discontinued Clinical findings Patient demographics Symptoms Age > 50 Anxiety Age > 70 Depression Female sex Diffuse aches Fatigue Past medical history Hip pain Hip stiffness Giant cell arteritis Joint pain Inflammation Loss of appetite Serological findings Morning stiffness Neck pain RF Shoulder pain anti-citrullinated cyclic peptide Shoulder stiffness Weight loss https://web.pathway.md/diseases/recYBnaWiR1R0zakk 7/8 6/29/23, 11:19 PM Polymyalgia rheumatica Pathway Lab findings Normal serum CK CRP ESR References 1. N Ughi, G D Sebastiani, R Gerli et al. The Italian Society of Rheumatology clinical practice guidelines for the management of polymyalgia rheumatica. Reumatismo. 2020 Apr 10;72 1 1 15. Open 2. 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. Open 3. George E Fragoulis, Elena Nikiphorou, Mrinalini Dey et al. 2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis. 2022 Nov 3;ard-2022 223335. Open 4. Anne R Bass, Eliza Chakravarty, Elie A Akl et al. 2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases. Arthritis Rheumatol. 2023 Jan 4. Online ahead of print. Open 5. Matteson EL, Dejaco C. Polymyalgia Rheumatica. Ann Intern Med. 2017 May 2;166 9 ITC65 ITC80. Open 6. Raheel S, Shbeeb I, Crowson CS et al. Epidemiology of Polymyalgia Rheumatica 2000 2014 and Examination of Incidence and Survival Trends Over 45 Years: A Population-Based Study. Arthritis Care Res Hoboken). 2017 Aug;69 8 1282 1285. Open 7. Kermani TA, Warrington KJ. Polymyalgia rheumatica. Lancet. 2013 Jan 5;381 9860 63 72. Open https://web.pathway.md/diseases/recYBnaWiR1R0zakk 8/8