QuestionSBA
QuestionChoice
Pain in fibromyalgia is generally more diffuse than the pain experienced in polymyalgia rheumatica. In addition, there is not usually the characteristic shoulder stiffness
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Fibromyalgia
QuestionChoice
Osteoarthritis is not an inflammatory condition. The associated stiffness does not follow this classic "worse in the morning for more than 1 hour" pattern - there still tends to be morning stiffness, but this resolves more quickly, and the pain is often described as being worse after periods of physical activity. Additionally, this patient has had a relatively acute onset of these symptoms, whereas symptoms related to OA tend to be more insidious in onset over many months - years
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Osteoarthritis (OA)
QuestionChoice
Adrenal insufficiency is associated with long term steroid use. PMR is treated with steroids, and therefore, these patients are at high risk of developing insufficiency during periods of intercurrent illness. However, at this point, the patient is yet to be diagnosed and commenced on any treatment
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Adrenal insufficiency
QuestionChoice
PMR most commonly occurs in women over 50. The most characteristic symptoms include shoulder and hip girdle **stiffness and pain** - both of which can affect patients ability to do their daily activities - and which classically inhibit a patient from standing from a seated position. Other common features of the condition which are mentioned in this stem include fatigue and low-grade fever. PMR is a systemic inflammatory condition - the associated stiffness is worse in the morning (lasting for 1 hour or more) - as is the case for most inflammatory rheumatological conditions
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Polymyalgia rheumatica (PMR)
QuestionChoice
GCA is an _associated condition_ often co-occurring in patients with polymyalgia. It presents with unilateral symptoms of jaw claudication, headache and scalp tenderness which can progress to visual loss if not identified and treated
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Giant cell arteritis (GCA)
QuestionComment
but she has muscle pain how is that pmr?
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Muscle pain and stiffness are possible presentations, the key differentiator between myositis and PMR is functional weakness
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Amaurosis Fugaxlegomenon
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Acute Serotonin
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# Summary

Polymyalgia rheumatica is a systemic inflammatory condition characterised by pain and stiffness of the neck, shoulders and pelvis. It is primarily a disease of older age, and particularly affects people of Northern European descent. Systemic symptoms include weight loss, low-grade fevers, fatigue and anorexia. Diagnosis is essentially clinical and is supported by responsiveness of steroid treatment, however patients should all have baseline bloods and a urine dipstick to rule out differentials. Most patients can be managed in primary care with oral prednisolone, however those with atypical features or a poor response to steroids should be referred to rheumatology for further assessment. 

# Definition

Polymyalgia rheumatica (PMR) is a chronic inflammatory condition which presents with stiffness and pain of the proximal large joints. 

# Epidemiology

- PMR has a prevalence of approximately 1% in the UK
- Incidence increases with age, peaking in those aged 70-80 years old
- PMR is very rare in the under 50s
- Women make up the majority of cases
- Incidence is significantly higher in patients with Northern European ancestry 
- Approximately 15-20% of patients with PMR develop giant cell arteritis (see separate topic), and PMR affects up to 60% of patients with giant cell arteritis

# Signs and Symptoms

- The key symptom is bilateral shoulder, neck and pelvic girdle pain 
- Joints feel stiff, especially after rest or sleep, and movement worsens pain
- Pain may radiate to the elbows or knees
- Systemic symptoms are common, including:
- Low-grade fevers
- Loss of appetite 
- Weight loss
- Malaise
- Depression
- On examination, muscle power is usually preserved (unless there is disuse atrophy from prolonged symptoms)
- Active range of motion may be limited by pain and stiffness
- Some patients may have associated swelling and pain in peripheral joints (e.g. wrists and ankles) - this is usually asymmetrical 
- Hands and feet may be swollen with pitting oedema
- Carpal tunnel syndrome may be present

# Differential Diagnosis

- **Myositis** is often painless but associated with prominent muscle weakness on examination; skin changes are seen in dermatomyositis and CK will be significantly increased
- **Rheumatoid arthritis** causes joint deformities, primarily affecting the small joints of the hands and feet rather than the larger proximal joints affected in PMR
- **Malignancy** can cause similar systemic features of weight loss and low-grade fevers; an apical lung cancer may cause shoulder pain (Pancoast tumour) and bone pain is seen in myeloma
- **Osteoarthritis** also causes joint pain and stiffness, more likely to involve hands, knees, hips and spine
- **Frozen shoulder** may sometimes be bilateral and causes pain and stiffness with limited range of motion
- **Chronic infections** such as tuberculosis or infective endocarditis cause similar systemic features and may present non-specifically
- **Hypothyroidism** also causes fatigue and myalgia; other symptoms include weight gain and constipation
- **Osteomalacia** causes bone pain and proximal muscle weakness due to vitamin D deficiency

# Investigations 

PMR is fundamentally a clinical diagnosis which is supported by resolution of symptoms with steroid treatment.

However, given the wide variety of differentials, basic investigations should be undertaken in all patients as follows:

- **Urine dipstick** may be positive for blood due to myoglobin released in myositis
- **ESR** and/or **CRP** are usually moderately raised in PMR
- **FBC** - raised white cells may indicate infection, anaemia may be seen in malignancy or due to chronic disease
- **U&Es** may show renal impairment in myeloma or infection
- **LFTs** may be deranged e.g. due to metastatic cancer or a high ALP in osteomalacia
- **Bone profile** may show high calcium in myeloma, or low calcium in osteomalacia
- **Creatine kinase** will be raised in myositis 
- **Thyroid function tests** for hypo or hyperthyroidism
- **Protein electrophoresis** to screen for myeloma; **urine Bence Jones protein** should be considered
- **Rheumatoid factor** as a screen for rheumatoid arthritis, **ANA** and **anti-CCP** antibodies should also be sent if there is clinical suspicion
- **HbA1c** prior to starting steroids due to their impact on blood glucose control
- **Chest X-ray** may be indicated if there is suspicion of tuberculosis or lung cancer

# Management

- Patients with core symptoms of PMR, no atypical features and no suspicion of differentials after initial investigations should be started on steroids in primary care
- 15mg of oral prednisolone once a day is the usual regimen
- Patients should be reassessed after a week to assess treatment response, and ESR/CRP should be rechecked at 3-4 weeks
- Consider weaning steroids at 3 weeks - this should be done slowly with close monitoring 
- Usually patients require steroids for 1-2 years
- Safety-net patients for the risk of giant cell arteritis and advise them to seek urgent medical attention if symptoms develop

Important management considerations when starting long-term steroids include:

- Give patients a steroid card
- Advise never to stop steroids suddenly due to the risk of adrenal insufficiency
- If they are unable to take tablets (e.g. due to vomiting) they need to seek urgent medical advice
- Explain the risk of immunosuppression and check vaccination status - advise seeking urgent medical advice in patients not immunised if they are exposed to chickenpox, shingles or measles
- Consider starting bone protection with bisphosphonates +/- vitamin D and calcium supplementation; this should be continued for the duration of steroid treatment before reassessing fragility fracture risk
- Assess the risk of peptic ulceration and consider starting a proton pump inhibitor for gastric protection
- Monitor blood pressure and glucose (as hypertension and hyperglycaemia are common side effects)

The following patients should be referred to rheumatology:

- Atypical clinical features:
- Aged under 60
- No shoulder/pelvic girdle pain
- No stiffness lasting > 45 minutes on waking/after rest
- Chronic onset of symptoms
- Red flag signs or symptoms e.g. weight loss or night pain
- Atypical inflammatory markers i.e. normal or very high (ESR > 100 mm/hour)
- Limited or no response to steroid treatment
- Steroids required for over 2 years
- Unable to wean steroids without relapses
- Significant adverse effects (or high risk of these) with steroids
- Suspected giant cell arteritis (ideally requires same day assessment with same day ophthalmology assessment also if there is visual loss)

Secondary care management options for PMR include methotrexate and azathioprine, and physiotherapy may also be of benefit.

# Prognosis

- Generally prognosis is good, with most patients rapidly responding to steroids
- However relapse is common when steroids are weaned
- Usually patients require 1-2 years of steroids 
- Some may need many years or even lifelong steroid treatment

# NICE Guidelines

[NICE CKS - Polymyalgia Rheumatica](https://cks.nice.org.uk/topics/polymyalgia-rheumatica/)

# References

[Patient UK - Polymyalgia Rheumatica](https://patient.info/doctor/polymyalgia-rheumatica-pro)

[British Society for Rheumatology - Treatment of polymyalgia rheumatica](https://academic.oup.com/rheumap/article/8/1/rkae002/7606885)
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A 59-year-old woman presents to the GP with two weeks of stiffness in her shoulders and hips which she describes as worse in the morning. Initially, she recalls thinking she had the flu, as she had myalgia, a low-grade fever and significant fatigue. However, these symptoms have not improved, and she is now anxious that there may be something more sinister going on. The stiffness and muscle pain is so severe she can no longer stand from sitting independently, or open the top cupboards in her kitchen.

What is the most likely diagnosis?
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