Table 1 Summary of the Norwegian society of rheumatology`s recommendations for the management of polymyalgia rheumatica

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: ESR, CRP, complete blood count, creatinine, ALAT, ALP, calcium, fasting blood glucose, Hb1Ac, RF and ACPA. Additional investigations to consider include but are not limited to CK, TSH and protein electrophoresis.

2.

Diagnostic imaging is not obligatory for PMR-diagnosis. However, the diagnosis may be supported by diagnostic imaging and/or validated against current classification criteria.

 A. US examination of the shoulder and hip regions are the preferred first-line imaging modality, and supplementary vascular US is recommended if there is suspicion of coexisting GCA.

 B. In patients with suspected PMR yet negative musculoskeletal- or vascular ultrasound, FDG-PET/CT may, in specialty care, be considered if suspicion of LVV or other differential severe diagnoses such as infections or malignancies.

 C. Additional imaging, such as MRI of the shoulders or hips, chest X-ray, US of abdomen, and CT-scan, may be considered at the physician’s discretion to rule out alternative diagnosis.

3.

Specialist referral is recommended if atypical features (age < 50 years, peripheral inflammatory arthritis, constitutional symptoms, low ESR/CRP), high risk for or occurrence of GC-related adverse events, GC-refractory disease, repeated relapses and/or prolonged therapy.

4.

Before start of GC therapy, existing comorbidities and co-medications should be assessed.

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.

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

 B. A dose > 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.

 C. A lower dose may be preferred in patients at increased risk of developing GC-related adverse effects.

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

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

6.

Once remission is reached, 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. When remission is achieved, continue prednisolone tapering by 1.25 mg every 4th week until discontinuation, provided that remission is sustained.

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.

8.

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 and considered if relapsing disease or GC-related adverse events. Consider IL-6R inhibitor for patients who cannot tolerate or do suffer a relapse while on MTX.

9.

Everyone diagnosed with PMR should be informed about the link between PMR and GCA. If any symptoms of GCA arise, individuals should promptly seek medical attention.

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.

10.

Follow-up visits are recommended at 2–4 weeks and 3, 6, 9, and 12 months after commencing GCs as a minimum in newly diagnosed patients, and as indicated in case of relapse or as prednisolone is tapered and discontinued.