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What is Polymyalgia rheumatica
common systemic inflammatory disease that is one of the most common indications for long-term steroids. It is characterised by myalgia and muscles stiffness with preponderance to the neck, shoulder and pelvic girdle.
Who is affected by polymyalgia rheumatica
PMR is predominantly a disease of older adults and rarely presents before 50 years old. The peak prevalence is estimated between 70-80 years. Women are 2-3 times more likely to be affected than men.
Aetiology of PMR
there appears to be a pro-inflammatory response with elevated levels of IL-6, an increase in certain T-cell subsets and subclinical arterial inflammation in some patients.
Genetic and environmental factors for aetiology of PMR
Genetic: PMR, like GCA, has been associated with several human leucocyte antigen (HLA) alleles (e.g. HLA-DR4).
Environmental: the cyclical pattern of cases and peak incidence in winter months suggests an infectious trigger.
Predominant sites of inflammation for PMR
proximal articular and periarticular structures.
Pathophysiology of PMR
- predominant site of inflammation includes bursae and tendons. Bursae are fluid-filled sacs that counteract the friction associated with tendons.
- Despite the site of inflammation, patients still present with generalised muscle stiffness and pain, particularly in the shoulder and pelvic girdles.
Characteristic sites in the upper and lower extremities associated with PMR:
Shoulder girdle: subdeltoid/subacromial bursitis and biceps tenosynovitis.
Pelvic girdle: bursae around the greater trochanters and ischial processes. liopectineal and iliopsoas bursitis. Hamstring tendinitis and hip synovitis.
Symptoms of PMR
- Bilateral shoulder and/or hip girdle pain
- Stiffness and upper limb tenderness: particularly mornings
- Systemic features: low-grade fever, fatigue, weight loss
- Low mood
- Peripheral symptoms
Signs of PMR
- Reduced range of movement: shoulder, cervical spine, and hips
- Inability to abduct shoulders past 90º
- Synovitis and swelling
Motor exam:
GCA and PMR
10% of patients with PMR will develop GCA. Therefore, it is essential to assess for features of GCA including unilateral headache, visual changes, jaw claudication, temporal artery tenderness, scalp pain and constitutional symptoms.
Diagnosis of PMR
- Age: 50 years or older at disease onset
- Typical symptoms: bilateral, symmetrical shoulder and/or hip girdle pain associated with stiffness
- Duration: > 2 weeks and lasting > 45 minutes at a time
- Elevated inflammatory markers (ESR/CRP): supportive, but diagnosis can be made if normal
- Rapid resolution of symptoms with corticosteroids: patient-reported global improvement of 70% or more within a week
Atypical features
Younger age of onset
Significant weight loss
Night pain
Neurological findings
Absence of core symptoms
Normal, or markedly elevated, inflammatory markers
Chronic onset
Management of PMR
Start on oral prednisolone 15mg daily
After initiation - prednisolone should be reduced once symptoms are fully controlled - usually after a period of 3-4 weeks
Steroid - related complications
teroid-induced hyperglycaemia, mood changes, insomnia, gastrointestinal bleeding, immunosuppression, weight gain, cushingoid appearance, osteoporosis and adrenal cortex suppression.
Prognosis of PMR
Up to 45% of patients may not respond to steroids within the first 3-4 weeks of treatment and a more extended course of steroids may be needed.
Thankfully, there is no increased mortality associated with PMR, but relapse is common and patients may develop morbidity associated with side-effects from corticosteroids.
What is Pagets?
A chronic bone disorder that is characterised by focal areas of increased bone remodelling, resulting in overgrowth of poorly organised bone.
Also known as osteitis deformans.
