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Norwegian society of rheumatology recommendations on diagnosis and treatment of patients with Polymyalgia Rheumatica: a narrative review

BMC Rheumatology volume 8, Article number: 58 (2024) Cite this article

Abstract

Background

To provide evidence-based, up-to-date recommendations for physicians in primary and specialist healthcare setting in diagnosing and treating patients with polymyalgia rheumatica (PMR).

Methods

The PMR working group conducted a narrative review of the available evidence in the field and wrote the draft guidelines. These guidelines were discussed and revised according to the standard operating procedures within the Norwegian Society of Rheumatology. The European Alliance of Associations for Rheumatology (EULAR) and American College of Rheumatology (ACR) recommendations for the management of PMR, the British Society for Rheumatology (BSR) guidelines for the management for PMR, the treat-to-target recommendations in giant cell arteritis and PMR and the 2023 recommendations for early referral of individuals with suspected polymyalgia rheumatica were used in particular for purpose of harmonization.

Results

A total of 10 recommendations have been formulated covering initial diagnostic investigations, comorbidity assessment, imaging, specialist referral criteria, treatment involving glucocorticoids and steroid-sparing agents and follow-up care.

Conclusion

Norwegian recommendations for diagnostics and treatment to improve management and outcome in patients with PMR were developed.

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Introduction

Background

Polymyalgia rheumatica (PMR) is a common inflammatory rheumatic disease characterized by subacute onset of proximal muscle pain and stiffness in the neck, shoulders, pelvic girdle and elevated acute phase reactants [1]. Occasionally, the onset is more gradual and can include nonspecific symptoms such as fatigue, anorexia, weight loss, or a low-grade fever [2, 3]. Morning stiffness is usually pronounced, and daily activities may be impaired. PMR occurs almost exclusively in people older than 50 years, with a peak between 70 and 79 years of age, and women are approximately 2 to 3 times more likely to be affected than men [4]. The highest incidence of PMR has been reported in Nordic countries and among North-American people of Scandinavian descent [5,6,7]. PMR may present isolated or in association with Giant Cell Arteritis (GCA), a large vessel vasculitis (LVV) usually affecting the aorta and its main branches. Around one quarter of PMR patients present with subclinical vasculitis without specific vasculitic symptoms (subclinical GCA), and the patients in this disease subset may be at increased risk of relapses and GCA-related ischemic- and vascular complications [8,9,10].

There is no gold standard for diagnosing PMR and the classification criteria has been defined for use in research [11]. In practice, the diagnosis is based on clinical judgment supported by consistent signs and symptoms of PMR, a rapid response to moderate glucocorticoid (GC) doses and exclusion of mimicking conditions. Recent studies indicate that implementing PMR fast-track clinics may decrease time from symptom onset to diagnosis, and reduce the number of hospital contacts and hospitalization days prior to PMR diagnosis [12,13,14].

The mainstay of PMR-treatment is moderately dosed GCs with gradual tapering for relief of symptoms. Relapses are common, with an estimated 43% of patients experiencing at least one relapse within a year after commencing GC, and 51% of patients still require GCs after 2 years of treatment [15]. PMR has been associated with a higher burden of comorbid diseases and GC-related adverse events [16, 17]. However, PMR per se is not associated with organ damage or increased mortality [7, 18,19,20].

Objectives of the guideline

The guideline is intended for physicians in primary or specialist health care involved in diagnosing and treating patients older than 50 years of age suspected to have PMR. The guideline aims to provide evidence-based, up-to-date recommendations and harmonize the diagnostic and treatment procedures for PMR in the Norwegian Healthcare System.

Methods

The guideline was developed according to the standard operating procedures within the Norwegian Society of Rheumatology (NSR). These procedures aim to ensure equitable and evidence-based investigation, treatment and follow-up of people with rheumatic disease in Norway. Each procedure involves the formation of a working group comprising representatives from at least 3 out of 4 healthcare regions in Norway. The working groups develop guidelines for the selected rheumatic disease by including guidelines of relevant international societies and performing a narrative review of the existing evidence. The review process for the NSR procedures is not mandated to follow a systematic approach, since the work is conducted entirely on a voluntary basis with no associated funding. The procedures are then proposed, discussed, revised, and accepted either by consensus or majority vote within the working group. This is followed by a hearing conducted by the NSR, where all registered rheumatology departments (n = 18) and privately practicing rheumatologists in Norway are invited to provide feedback on the draft guidelines.

The current recommendations were developed by the Norwegian PMR working group (consisting of the authors of this guideline). The evidence on diagnostics and treatment of PMR published from January 1st, 2015, to March 31st, 2024, was reviewed and included in this work. PubMed and a combination of search terms Polymyalgia Rheumatica and treatment and/or diagnosis were used for the literature search. The 2015 European League Against Rheumatism (EULAR) and American College of Rheumatology (ACR) recommendations for the management of PMR, the British Society for Rheumatology (BSR) guidelines for the management for PMR, the treat-to-target recommendations in GCA and PMR and the 2023 recommendations for early referral of individuals with suspected polymyalgia rheumatica were used in particular for purpose of harmonization [21,22,23,24,25].

The present guideline is the foundation upon which clinical practice should be based. Guidelines, unlike some types of policies, are not mandatory. Individual patient circumstances may influence clinical decisions, and clinicians should work alongside patients to make care-based shared decisions. Thus, failure to adhere to this guideline should not necessarily be considered negligent. The guideline should not be used to limit access to other diagnostic or treatment options.

Results

The working group formulated a total of 10 recommendations based on the finding of the literature review. Following this, feedback was provided by 10 of 18 registered rheumatology departments and one private practicing rheumatologist through the national hearing by the NSR. The feedback was thoroughly reviewed, leading to a final revision, which were then officially endorsed by the NSR. Compared to the 2015 EULAR/ACR recommendations for the management of PMR [21], the following major additions has been incorporated to the current guideline: Recommendations for imaging, including ultrasound (US) and fluorodeoxyglucose positron-emission tomography combined with computed tomography (FDG-PET/CT), the use of IL-6 receptor inhibitors (IL-6Ri), and screening for fracture risk. A summary of the NSR`s recommendations for management of polymyalgia rheumatica is provided in Table 1. A suggested algorithm for the diagnosis and treatment of PMR is provided in Fig. 1.

Diagnosis

Recommendation 1

In the presence of symptoms and signs suggestive of PMR, a thorough clinical evaluation is essential to exclude relevant mimicking inflammatory and non-inflammatory conditions (Table 2). To exclude mimicking conditions but also to serve as a baseline for the monitoring of subsequent therapy we recommend the following initial laboratory workup for every patient before GC-therapy: Erythrocyte sedimentation rate (ESR), c-reactive protein (CRP), complete blood count, creatinine, alanine transaminase (ALAT), alkaline phosphatase (ALP), calcium, fasting blood glucose, Hb1Ac, rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibodies (ACPA) [21, 25]. Additional investigations to consider include but are not limited to creatine kinase (CK), thyroid stimulating hormone (TSH) and protein electrophoresis.

PMR may be the initial manifestation of GCA or may develop later in the disease course of PMR [26]. Therefore, patients should be in particular evaluated for cranial and ischemic symptoms and signs on physical examination of vascular involvement of GCA (Table 2), during both at diagnosis and follow-up, as GCA requires urgent initiation of high-dose GCs.

Recommendation 2

The diagnosis of PMR may be supported by diagnostic imaging (Table 3) and/or validated against current classification criteria (Table 4). However diagnostic imaging is not obligatory for diagnosis and many patients with PMR will lack relevant findings on imaging. The extent of imaging in such cases will depend on the need to exclude mimicking conditions as the symptoms and laboratory findings of PMR are inherently non-specific.

  1. A.

    We recommend US examination of the shoulder and hip regions as the preferred first-line imaging modality. In addition, supplementary vascular US should be performed if there is suspicion of coexisting GCA.

  2. B.

    FDG-PET/CT should be restricted to specialty care setting, where it is increasingly utilized to rule out or confirm suspicions of LVV or other serious differential diagnoses, such as infections or malignancies, in patients with clinically suspected PMR yet negative musculoskeletal- or vascular US.

  3. C.

    Additional imaging, such as Magnetic Resonance Imaging, chest X-ray, US of the abdomen, and CT-scan, may be considered at the physician’s discretion to rule out the alternative diagnosis [27].

The choice of imaging modality may depend on the level of care (primary/secondary care setting), the complexity of the disease presentation, and/or the course of the disease.

Recommendation 3

We recommend specialist referral in patients with atypical features (e.g., age < 50 years, peripheral inflammatory arthritis, constitutional symptoms, low inflammatory parameters), high risk of or already established GC-related adverse reactions, GC-refractory disease, repeated relapses, and/or prolonged treatment [21, 25].

As symptoms of PMR can be inherently nonspecific, it is often underdiagnosed, misdiagnosed, or diagnosed late, which may result in delay in appropriate treatment [28]. Given the diagnostic challenges, collaboration and coordination between general practitioners, rheumatologists and internists and other relevant specialists must be optimized. PMR/GCA-fast track circuits may be important measures to secure prompt and accurate diagnostic work-up for these patients [29, 30].

Treatment

Recommendation 4

Before the start of GC therapy, existing comorbidities and co-medications should be assessed. These include hypertension, cardiovascular diseases, hyperlipidaemia, diabetes mellitus, impaired glucose tolerance, osteoporosis and previous fractures, peptic ulcers, cataract, glaucoma, risk of infections, and treatment with non-steroidal anti-inflammatory drugs [21].

There is conflicting evidence on whether female sex, ESR > 40 mm/hr, and peripheral arthritis may be risk factors for relapses and/or longer treatment duration [15, 21].

Recommendation 5

Once the diagnosis of PMR has been made, therapy with GCs should be initiated with treatment target to induce remission of clinical symptoms. One should strive to minimize treatment related adverse events by using the lowest effective individualized dose and duration of GC therapy taking into consideration the patient’s perspective and preferences.

  1. A.

    For most PMR patients, we recommend an initial dose of oral prednisolone (or equivalent) of 15 mg/day.

  2. B.

    An initial dose exceeding 15 mg/day and up to 25 mg/day may be considered for patients judged to have increased risk of relapse and low-grade or absence of complicating comorbidities or other risk factors for developing GC-related adverse effects [21].

  3. C.

    An initial dose < 15 mg/day may be preferred in patients at increased risk of developing GC-related adverse effects.

  4. D.

    Initial dose ≤ 7.5 mg/day or ≥ 30 mg/day is not recommended.

  5. E.

    Divided daily doses may be considered in specific circumstances, such as prominent symptoms at night when GCs are reduced to lower doses [21].

The optimal GC-starting dose remains to be decisively established. Observational studies have found an association between a higher initial GC dose and the risk of prolonged treatment [31,32,33,34]. However, this association may be influenced by confounding by indication, where the initial severity of the disease impacted the treatment decisions, potentially affection treatment duration. A prospective observational study involving 39 patients with PMR found that those randomly assigned an initial prednisolone dose of 10 mg daily had higher relapse rate compared to those assigned a dose of 20 mg/day [35].

Recommendation 6

Once remission is achieved, it should be maintained with the minimal effective GC-dose and individualized tapering according to disease activity, laboratory markers, and adverse events (Table 5). Prednisolone should initially be tapered to 10 mg/day (or equivalent) within 4-8 weeks [21]. Once remission is achieved on this schedule, we recommend continued prednisolone tapering by 1.25 mg every 4th week until discontinuation, provided that remission is sustained. In many cases there is a need for GC-treatment for at least one year before discontinuation is tolerated.

The most important risk factor for relapse in isolated PMR is the rate of gradual reduction of GCs [36]. The risk of relapse increases with a tapering regimen of prednisolone > 1 mg/month and when the prednisolone dose is less than 7.5 mg/day [36]. Therefore, during follow-up, a more prolonged tapering regimen than above may be needed, particularly when reducing to lower doses.

Recommendation 7

In cases of relapse, we recommend increasing the GC dose to the pre-relapse dose, followed by gradual tapering (within 4–8 weeks) to the dose at which the relapse occurred [21].

Relapse and GC-refractory disease should prompt re-evaluation of the diagnosis.

In patients with PMR symptoms and normal ESR/CRP, testing for GC-induced adrenal gland insufficiency with basal morning cortisol should be considered [37, 38].

Recommendation 8

Methotrexate (MTX) 10–20 mg once a week, in addition to GCs, should be considered early in patients with comorbidities that may be exacerbated by GC therapy. Additionally, MTX may be considered in patients with relapsing disease or GC-related adverse events [39]. Consider IL-6Ri for patients who cannot tolerate or do suffer a relapse while on MTX.

The MTX-dosage should be reduced in individuals of advanced age or those with impaired kidney function.

Leflunomide 10–20 mg daily could be used as an alternative to MTX, but the scientific evidence is poor and based only on case series [40,41,42].

TNF inhibitors have not demonstrated significant benefit in patients with PMR and are not recommended [43, 44].

Tocilizumab (TCZ), an IL-6Ri, has been documented to have effect on isolated PMR in combination with GCs [45,46,47]. However, further studies are warranted to evaluate the efficacy and assess the balance of potential benefits and harms. In a phase 3 study (SAPHYR), the IL-6Ri sarilumab demonstrated higher rate of sustained remission, lower rate of relapses and lower GC-exposure at 52 weeks compared to the placebo group [48]. Based on the results of this study, the FDA has approved sarilumab for patients with PMR who have inadequate response to GCs or cannot tolerate GC taper. Currently, neither sarilumab nor TCZ are approved for reimbursement following use in PMR by the Norwegian Health Authorities. Therefore, IL-6Ri should be reserved for patients who cannot tolerate or do suffer a relapse while on MTX.

Tofacitinib, a janus kinase inhibitor, was recently shown to have promising efficacy and good safety profile in PMR [49, 50]. Currently there is not sufficient evidence to include guidance on the use of tofacitinib in the national recommendations.

Recommendation 9

It is crucial to ensure that everyone diagnosed with PMR is aware of the link between PMR and GCA. If any symptoms of GCA arise, individuals should promptly seek medical attention [25].

In cases of PMR evolving into or co-existing with GCA, we recommend that treatment should be adjusted according to the guideline for the management of GCA [51].

Monitoring / follow-up

Recommendation 10

Patients should be followed closely after starting GC treatment. We recommend a follow-up visit at 2–4 weeks and 3, 6, 9, and 12 months after commencing GCs as a minimum in newly diagnosed patients. Frequent follow-up visits adjusted to individual needs, are recommended in patients with relapsing disease. Continued follow-up with regular attempts to reduce or discontinue prednisolone are recommended in all patients with continued GC-treatment beyond 12 months.

Patients should be monitored for recurrence of their original PMR symptoms and symptoms and signs suggestive of GCA or other alternative diagnoses. ESR and CRP should be monitored, with additional tests as indicated individually. ALP may possibly predict risk of relapse in PMR according to one study [52]. US may be used to monitor disease activity and treatment response [53].

Other management includes prophylaxis and monitoring for GC-related side effects including hypertension, glucose intolerance, and osteoporosis. Calcium- and vitamin D supplementation and screening for fracture risk by bone mineral density using dual-energy x-ray absorptiometry (DXA) should be performed for all patients treated with GCs ≥ 2.5 mg/day for > 3 months [54]. Anti-resorptive medications should be individualized based on patient characteristics and DXA-scan results.

Conclusions

Developed from a narrative review of available evidence and international consensus on PMR management, this guideline offers ten key recommendations for diagnosis and treatment. It ensures that physicians in both primary and specialist healthcare settings have the latest information to provide optimal patient care and ultimately improve outcomes for PMR patients.

Fig. 1
figure 1

Suggested algorithm for diagnosis and treatment of PMR*. *Algorithm adapted from 2015 EULAR/ACR recommendations for management of PMR [21] and Buttgereit et al. 2016 [55]. **A higher- or lower initial dose within the range of 12.5-25 mg/day may be considered depending on risk of relapses, adverse events, comorbidities and/or other risk factors for GC-related adverse effects

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Table 1 Summary of the Norwegian society of rheumatology`s recommendations for the management of polymyalgia rheumatica
Full size table
Table 2 PMR diagnosis and differentials*
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Table 3 Imaging modalities supporting the diagnosis of polymyalgia rheumatica
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Table 4 ACR/EULAR 2012 classification criteria for the PMR with or without using ultrasonography [11]
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Table 5 Prednisolone treatment in PMR
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Data availability

No datasets were generated or analysed during the current study.

Abbreviations

ACPA:

Anti-Cyclic Citrullinated Peptide Antibodies

ACR:

American College of Rheumatology

ALAT:

Alanine Aminotransferase

ALP:

Alkaline Phosphatase

BSR:

British Society for Rheumatology

CK:

Creatine Kinase

CRP:

C-Reactive Protein

DXA:

Dual-Energy X-Ray Absorptiometry

ESR:

Erythrocyte Sedimentation Rate

EULAR:

European Alliance of Associations for Rheumatology

FDG-PET/CT:

Fluorodeoxyglucose Positron-Emission Tomography Combined with Computed Tomography

GC:

Glucocorticoid

GCA:

Giant Cell Arteritis

IL-6Ri:

IL-6 Receptor Inhibitor

LVV:

Large Vessel Vasculitis

MTX:

Methotrexate

NSR:

Norwegian Society of Rheumatology

PMR:

Polymyalgia Rheumatica

RF:

Rheumatoid Factor

TCZ:

Tocilizumab

TSH:

Thyroid Stimulating Hormone

US:

Ultrasound

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Authors and Affiliations

  1. Department of Research, Hospital of Southern Norway, Kristiansand, Norway

    Stig Tengesdal & Geirmund Myklebust

  2. Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway

    Stig Tengesdal

  3. Division of Internal Medicine, Department of Infectious diseases, Akershus University Hospital, Lørenskog, Norway

    Andreas P Diamantopoulos

  4. Department of Rheumatology, Haugesund Hospital for Rheumatic Diseases, Haugesund, Norway

    Lene Kristin Brekke

  5. Department of Medicine, Hospital of Finnmark, Kirkenes, Norway

    Emilio Besada

  6. Institute of clinical medicine, UiT the arctic university of Norway, Tromsø, Norway

    Emilio Besada

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APD conceived the study concepts and design. ST performed the literature search and drafted the initial manuscript, with contributions from APD, LKB, EB and GM. All authors read and approved the final manuscript.

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Tengesdal, S., Diamantopoulos, A.P., Brekke, L.K. et al. Norwegian society of rheumatology recommendations on diagnosis and treatment of patients with Polymyalgia Rheumatica: a narrative review. BMC Rheumatol 8, 58 (2024). https://doiorg.publicaciones.saludcastillayleon.es/10.1186/s41927-024-00422-6

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BMC Rheumatology

ISSN: 2520-1026

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