Polmyalgia Rheumatica and GCA

Question 1

What is Polymyalgia Rheumatica?

Answer 1

It is a syndrome with pain or stiffness, usually in the neck, shoulders, upper arms, and hips, but which may occur all over the body. The pain can be very sudden, or can occur gradually over a period.

There is a close relationship wth GCA (most common of the systemic vasculitides characterised by involvement of the large vessels).

• 20% of PMR have GCA
• 50% GCA have PMR

Question 3

What is the aetiology of PMR?

Answer 3

Unknown

Question 4

Who is most likely to be affected by PMR?

Answer 4

• Usually >70, rare in <50
• F>M - 2:1

Question 5

How does someone with PMR Present?

Answer 5

• SUDDEN onset of shoulder +/- pelvic girdle STIFFNESS
  
  • Bilateral aching and tenderness
  • Morning stiffnes
• Synovitis/Tenosynovitis
• Arthralgia
• Carpal tunnel syndrome
• Systemic features - WL, Fatigue, Fever, anorexia
• Depression

Question 6

What is the differential diagnosis for someone who presents clinically with symptoms associated with PMR?

Answer 6

• Rhematoid Arthritis - recent onset
• Primary muscle disease
• Osteoarthritis - particularly cervical spondylosis, shoulder OA
• Malignancy - e.g Multiple myeloma, lung cancer
• Hypo/hyperthyroidism
• Bilateral shoulder capsulitis/Subacromial impingement
• Fibromyalgia
• Inflmmatory joint disease - Myalgic onset

Question 7

What tests would you do when presented with symptoms associated with PMR?

Answer 7

No specific diagnostic test

Risk factors present - Age > 50, female

Bloods

• FBC - look for signs of myelodysplastic disease
• CRP - Increased
• ESR - Increased (> 50)
• ALP - Increased (in 30% of cases)
• CK - normal - helps distinguish from myopathies/myositis (e.g. DMD, BMD)

Dramatic steroid response

Question 8

How would you manage someone with PMR?

Answer 8

• Prednisolone - 15mg/day - 18-24 months
  
  • Gastric and bone protection due to prolonged use

Question 9

What is Giant Cell Arteritis?

Answer 9

An inflammatory disease of blood vessels most commonly involving large and medium arteries of the head, predominantly the branches of the external carotid artery.

Question 10

What is the pathophysiology of GCA?

Answer 10

The pathological mechanism seems to start when dendritic cells in the vessel wall recruit T cells and macrophages to form granulomatous infiltrates.

Giant-cell indicates the union of several distinct cells to form a granuloma, therefore this is a granulomatous large vessel vasculitis.

Question 11

What type of individual does GCA occur in?

Answer 11

• Elderly
  
  • If <55 - consider Takayasu’s arteritis
• Those with PMR

Question 12

How does GCA present?

Answer 12

• Temporal headache
• Scalp tenderness
• Jaw Claudication - can also get tongue claudication
• Sudden painless blindness - amaurosis fugax (occlusion of the opthalmic artery)
• Dyspnoea
• Morning stiffness
• Unequal/weak temporal pulses

Question 13

How would you diagnose GCA?

Answer 13

Bloods

• ESR + CRP - increased
• Platelets - increased
• ALP - increased
• Hb - decreased

Diagnosis

• Temporal Artery Biopsy - within 7 days of starting steroids

Question 14

How would you treat someone with suspected GCA?

Answer 14

• High Dose Steroids - Prednisolone 60mg/day - immediately
  
  • Gastric and bone protection in prolonged use
• Low dose aspirin

Question 15

What is a potential complication of GCA?

Answer 15

Sudden painless irreversible mono/bilateral blindness

Question 16

What visual distrubances can those with Giant cell arteritis experience?

Answer 16

• Scintillating scotoma
• Amaurosis fugax
• Diplopia