About Polymyalgia Rheumatica
Polymyalgia rheumatica (PMR) is an inflammatory autoimmune articular and periarticular disease of the elderly.
- Onset: >50 yo
Mean age: 70 yo - Females affected 2-3x more often than males
- Symmetric, profound stiffness -> pain in shoulders and hip girdle that is predominant in the morning
- Improves with activity
- Subacute onset over ~2-3 weeks
- Systemic symptoms include malaise, fatigue, weight loss
- Physical exam:
- Shoulders can be tender to palpation
- ROM often limited due to stiffness >>> pain
- Strength may be limited by discomfort
Lab Workup
- Lab tests alone are not diagnostic of PMR
- ESR and CRP elevated
- Normal inflammatory markers should raise concern for an alternate diagnosis (like rotator cuff tendinopathy, hip osteoarthritis, fibromyalgia, etc.)
Imaging
Imaging is not indicated to establish a diagnosis of PMR. However, it may be useful to evaluate for mimics of PMR.
- X-rays: may be normal or show OA changes
- MRI/ultrasound: non-specific, may show subacromial bursitis and/or trochanteric bursitis
Response to Steroids
- Significant improvement with prednisone 20 mg/d
- With systemic steroids on board, if symptoms are worsening after 3-7 days or persistent after 7 days, this essentially excludes the diagnosis
- Other rheumatic diseases
- Giant cell arterits
- Rheumatoid arthritis
- Myositis
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Fibromyalgia
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Malignancy
- Infection
- Infectious arthropathy (TB, bacteria)
- Endocarditis
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Endocrinopathies:
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Hypothyroidism
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Diabetes associated myalgia, arthralgia
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Drug toxicities
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Statins
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- Osteoarthritis/mechanical musculoskeletal disease (with normal ESR/CRP):
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Rotator cuff tendinopathy
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Osteoarthritis of hips or shoulders
- Cervical radiculopathy/stenosis
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- Prompt response to prednisone (15-20 mg/d) is highly characteristic of PMR
- Symptom reduction should be evident ~72 hours, usually within 24 hours
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Initial dose of prednisone
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Start at 10-20 mg/d
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Taper by 2.5 mg every month
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When able to get to 10 mg/d: taper dose by 1 mg every month until off
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Ending with an every other day dosing before coming completely off of steroids can help decrease symptoms of relative adrenal insufficiency
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- Second line therapy: sarilumab
- Used if patient unable to wean or tolerate steroids; refer to rheumatology
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If sarilumab needed for refractory/recurrent PMR, when sarilumab is started, start prednisone at 15 mg/d with decrease of 1 mg every week until off (~14 week taper)
Flares
- Best identified by symptoms, ESR/CRP usually elevated
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An increase in ESR/CRP without symptoms is not a flare
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CRP can be falsely low with use of IL6 pathway antagonists like sarilumab
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If no symptoms but the inflammatory markers are persistently elevated or rise, screen for symptoms of GCA or signs of GCA-like thoracic aneurysm
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If CRP rises back up with constitutional symptoms, keep infection on differential diagnosis
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- Escalate prednisone back to previous dose for flare
- 75% of patients able to taper off steroids in 2 years
Monitor for Giant Cell Arteritis (GCA)
- 15% of patients with PMR can get GCA
- GCA symptoms:
- Unilateral headache
- Jaw claudication
- Scalp tenderness
- Vision loss
Monitor for Toxicities of Systemic Steroids
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Very important to screen for osteoporosis with DEXA scans
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Monitor for signs of steroid-induced diabetes (serum glucose, A1C, etc.)
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Infections can occur even at lower doses of steroids, but PJP ppx at these doses without parenchymal disease usually not warranted
- Alternative diagnosis should be strongly considered if no symptom relief with ≥10-20 mg/d of prednisone.
- Flares often occur if prednisone is tapered too rapidly.
- Every visit, inquire about giant cell arteritis (GCA) symptoms (vision loss, recurrent headaches, scalp tenderness, jaw claudication).
- If GCA suspected, patient should be started on high dose prednisone (1 mg/kg/d or more).
- Temporal artery biopsy is gold standard for GCA diagnosis – prednisone should not be delayed for biopsy.