QuestionSBA
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Polymyositis is a type of idiopathic inflammatory myopathy caused by autoimmune damage to the muscle. This typically presents as bilateral, proximal muscle weakness. Polymyositis is a key differential for polymyalgia rheumatica, however as this patient has normal muscle power on examination, a diagnosis of polymyalgia rheumatica is more likely
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Polymyositis
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Polymyositis is a type of idiopathic inflammatory myopathy caused by autoimmune damage to the muscle. This typically presents with bilateral, proximal muscle weakness with associated skin rashes (including heliotrope rash, Gouttron's papules and mechanic's hands). Dermatomyositis is a key differential for polymyalgia rheumatica, however as this patient has normal muscle power on examination, which makes a diagnosis of polymyalgia rheumatica more likely. They also do not have any skin disease, making a diagnosis of dermatomyositis less likely
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Dermatomyositis
QuestionChoice
Polymyalgia rheumatica is an inflammatory condition that causes inflammatory pain, stiffness and systemic symptoms, typically affecting those >50 years old. The pain and stiffness typically last more than 30 minutes, is relieved by exercise, and affects the shoulder and hip girdles. These patients often report muscle weakness and other systemic symptoms, including weight loss, fever, malaise and fatigue. Typical examination findings include normal power (as in this case), which differentiates it from other key differentials such as myositis. This patent should have blood tests done, including ESR and CRP. Treatment of polymyalgia rheumatica is commended after diagnosis, typically with oral steroids (e.g. prednisolone). This is in contrast to a patient presenting with features of giant cell arteritis, who would be started on steroids before the diagnosis is confirmed
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Polymyalgia rheumatica
QuestionChoice
Giant cell arteritis is an inflammatory large vessel vasculitis affecting the temporal arteries. Typical symptoms include temporal headache, jaw claudication (pain upon repeated movement of the jaw, e.g. chewing), scalp tenderness (e.g. while brushing hair) and ocular complications (e.g. blindness, amaurosis fugax). This typically affects patients >50 years old and can be associated with polymyalgia rheumatica. As this patient has not had any of these symptoms, it is more likely this is simply a diagnosis of polymyalgia rheumatica. It is important to safety net this patient for these symptoms, as they should be started on prednisolone at presentation of suspected giant cell arteritis, instead of once the diagnosis has been confirmed in polymyalgia rheumatica
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Giant cell arteritis
QuestionChoice
It is worth considering functional disorders, factitious disorder and malingering in any patient presenting with examination findings out of keeping with their history. However, normal power is a well-documented finding in polymyalgia rheumatica and given this patient's demographics, history and typical examination findings; polymyalgia rheumatica is the most likely diagnosis
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Factitious disorder
QuestionComment
No mention of muscle pain in the question... "The most characteristic symptoms include shoulder and hip girdle stiffness AND pain"
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-myalgia, pain is literally in the name ugh I hated this question
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does anyone know why in PMR they experience weakness but their power is normal on examination ???
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The weakness experienced by individuals with PMR is not due to muscle damage or neurological problems but is rather a consequence of the inflammation and pain associated with the condition. The inflammation affects the muscles and surrounding tissues, leading to pain, stiffness, and limited range of motion. These symptoms can make it difficult for individuals to exert their full strength, resulting in a subjective feeling of weakness.

During a physical examination, muscle strength is assessed by asking the patient to perform specific movements or resist against force. While the patient may perceive weakness due to pain and stiffness, the actual muscle strength may be intact. This can be attributed to the fact that PMR primarily affects the large muscles around the shoulders and hips, which are responsible for movements like lifting or pushing. In contrast, the small muscles responsible for fine motor skills may be less affected, leading to normal strength on examination.
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Embolism Transplant
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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 65-year-old woman presents to the GP complaining of fatigue and weakness over the last four months. She says she feels stiff, especially around her shoulders and hips. On examination, all movements are 5/5 power using the Medical Research Council (MRC) muscle scale. She does not report any headaches and has no skin rashes.

What is the most likely diagnosis?
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