What are the symptoms and signs of polymyalgia rheumatica and how is it managed?

Symptoms and Signs of Polymyalgia Rheumatica

Polymyalgia rheumatica presents with bilateral shoulder and hip girdle pain accompanied by severe morning stiffness lasting more than 45 minutes in patients over 60 years old, with elevated inflammatory markers (ESR >40 mm/hr or elevated CRP) being present in over 90% of cases. 1, 2

Clinical Presentation

Cardinal Symptoms

• Bilateral shoulder pain is the hallmark feature, often the most prominent symptom 1, 3
• Hip girdle and pelvic pain occurs bilaterally, frequently accompanying shoulder involvement 3, 4
• Neck pain is common and part of the typical distribution 2, 5
• Morning stiffness lasting more than 45 minutes is characteristic and often severe 1, 4
• Acute or subacute onset with symptoms developing over days to weeks 2

Key Diagnostic Features

• Age ≥60 years is a critical criterion; younger patients warrant specialist referral for atypical presentation 1
• Elevated ESR (>40 mm/1st hour) or elevated CRP supports diagnosis in over 90% of patients 1, 2
• Dramatic response to glucocorticoids within 2 weeks is characteristic and helps confirm diagnosis 4, 6

Associated Conditions

• Giant cell arteritis (GCA) occurs in approximately 20% of PMR cases, and up to two-thirds of GCA patients have PMR symptoms 2, 3
• Monitor for visual symptoms, headache, and jaw claudication that suggest GCA 2

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Management of Polymyalgia Rheumatica

Initial Treatment Strategy

Start prednisone at 12.5-25 mg daily as first-line therapy, with the specific dose individualized based on relapse risk and comorbidity profile. 1, 7

Dose Selection Algorithm

• Use 20-25 mg/day for patients with high relapse risk (female sex, ESR >40 mm/hr, peripheral inflammatory arthritis) and low risk of adverse events 1, 7
• Use 12.5-15 mg/day for patients with relevant comorbidities (diabetes, osteoporosis, glaucoma, hypertension, cardiovascular disease, peptic ulcer) and risk factors for glucocorticoid-related side effects 1, 7

Why Glucocorticoids Over NSAIDs

The European League Against Rheumatism strongly recommends glucocorticoids rather than NSAIDs because NSAIDs showed a trend toward higher cardiovascular events (HR=1.64) and hypertension (RR=1.72), despite lower vertebral fracture rates 6, 7

Glucocorticoid Tapering Protocol

Initial Tapering Phase (First 4-8 Weeks)

• Reduce prednisone to 10 mg/day within 4-8 weeks of starting therapy 7, 1
• Clinical improvement should be noted within 2 weeks; almost complete response expected by 4 weeks 6

Maintenance Tapering Phase

• Once remission is achieved, decrease prednisone by 1 mg every 4 weeks (or use alternate-day schedules like 10/7.5 mg every other day) until discontinuation 7, 1, 8
• Continue tapering only if remission is maintained 6

Special Consideration for Low-Dose Range

• For nighttime pain when tapering below 5 mg/day, consider splitting the daily dose 6, 8
• However, persistent breakthrough symptoms should prompt reconsideration of the diagnosis 6

Management of Relapses

For relapse, increase prednisone to the pre-relapse dose that effectively controlled symptoms. 8, 6

Relapse Treatment Protocol

• Return to the previous effective dose immediately 8
• Gradually reduce over 4-8 weeks to the dose at which relapse occurred 8, 6
• Then taper more slowly at 1 mg per month (slower than initial tapering) 8, 1

Initial Non-Response Management

• If insufficient improvement within 2 weeks, increase oral dose up to 25 mg prednisone equivalent 6

Glucocorticoid-Sparing Therapy

Consider adding methotrexate 7.5-10 mg weekly for patients with frequent relapses, prolonged therapy requirements, or significant glucocorticoid-related adverse effects. 1, 6

Specific Indications for Methotrexate

• High risk of relapse/prolonged therapy: female sex, high ESR (>40 mm/hr), peripheral inflammatory arthritis 6, 7
• Risk factors for GC adverse events: relevant comorbidities (diabetes, osteoporosis, glaucoma, cardiovascular disease) or concomitant medications that increase GC toxicity 6, 7
• Multiple or frequent relapses during tapering 1, 8
• Inadequate response to glucocorticoids alone 6

Biologics

The European League Against Rheumatism strongly recommends against TNFα blocking agents due to no evidence of benefit but considerable risk of harm and high cost 6

Tocilizumab and secukinumab show promise in recent studies but lack formal guideline recommendations pending completion of ongoing trials 6, 9

Monitoring and Follow-Up Schedule

Schedule visits every 4-8 weeks during the first year, every 8-12 weeks in the second year, and as needed for relapses or dose adjustments. 7, 1, 8

At Each Visit, Assess:

• Clinical symptoms: pain severity, morning stiffness duration, functional status 1, 8
• Inflammatory markers: ESR and CRP to assess disease activity 8, 2
• Glucocorticoid-related adverse effects: weight gain, hyperglycemia, hypertension, bone loss, mood changes, infections 1, 9
• Risk factors for relapse: female sex, high ESR, peripheral arthritis 1, 7

Adjunctive Measures

Bone Protection

Initiate bone protection at treatment start because prednisone causes significant bone loss 1

Exercise and Rehabilitation

Recommend individually tailored exercise programs aimed at maintaining muscle mass and function, and reducing fall risk 7, 1

The European League Against Rheumatism conditionally recommends this despite lack of direct evidence, as exercise likely supports preservation of function and reduces glucocorticoid-related adverse events 6

Patient Education

• Provide education about disease impact, treatment expectations, and comorbidity management 7, 1
• Create an individualized management plan with shared decision-making between patient and physician 7

When to Refer to Specialist

Consider specialist referral for: 7

• Atypical presentations: age <60 years, peripheral inflammatory arthritis, systemic symptoms, low inflammatory markers 7
• High risk of therapy-related side effects 7
• PMR refractory to glucocorticoid therapy 7

Treatment Duration

Treatment duration typically ranges from 6 months to 3 years, with many patients requiring 2-3 years of therapy 2, 4

Some patients may require longer treatment, and no specific recommendation exists for optimal duration 6