GCA and PMR

Question 1

What is GCA?

Answer 1

Systemic vasculitis of the medium and large arteries

Question 2

Who is at higher risk of GCA?

Answer 2

Women over 50

Question 3

Main symptoms in GCA (5)

Answer 3

Severe unilateral headache typically around temple and forehead

Scalp tenderness my be noticed when brushing hair

Jaw claudication

Blurred or double vision

Irreversible painless complete sight loss can occur rapidly

Question 4

Associated symptoms in GCA

Answer 4

Fever

Muscle aches

Fatigue

Weight loss

Loss of appetite

Peripheral oedema

Question 5

How is GCA diagnosed?

Answer 5

Clinical presentation

Raised ESR: usually 50 mm/hour or more

Temporal artery biopsy findings (Multinucleated giant cells)

Question 6

Investigations in GCA

Answer 6

Full blood count may show a normocytic anaemia and thrombocytosis (raised platelets)

Liver function tests can show a raised alkaline phosphatase

C reactive protein is usually raised

Duplex ultrasound of the temporal artery shows the hypoechoic halo sign

Temporal artery biopsy

Question 7

Initial management of GCA

Answer 7

Start steroids immediately
40-60mg prednisolone per day

Aspirin 75mg daily decreases visual loss and strokes
PPi

Review the response to steroids within 48 hours

Question 8

Who do you refer GCA to?

Answer 8

Vascular surgeons for a temporal artery biopsy

Rheumatology

Opthalmology - emergency same day appointment if they develop visual symptoms

Question 9

Ongoing Management GCA

Answer 9

Continue high dose steroids (40-60mg) until the symptoms have resolved

Slowly wean off the steroids (can take years)

Question 10

Measures for patients on long term steroids

Answer 10

DON’T – Don’t stop taking steroids abruptly. There is a risk of adrenal crisis

S – Sick Day Rules

T – Treatment Card

O – Osteoporosis prevention with bisphosphonates and supplemental calcium and vitamin D

P – Proton pump inhibitor for gastric protection

Question 11

Early complications of GCA

Answer 11

Vision loss

Cerebrovascular accident (stroke)

Question 12

Late complications of GCA

Answer 12

Relapses of the condition are common

Steroid related side effects and complications

Cerebrovascular accident (stroke)

Aortitis leading to aortic aneurysm and aortic dissection

Question 13

What is PMR?

Answer 13

Inflammatory condition that causes pain and stiffness in the shoulders, pelvic girdle and neck

Strong association to giant cell arteritis

Question 14

Core features associated with PMR

Answer 14

Should be present for at least 2 weeks

Bilateral shoulder pain that may radiate to the elbow

Bilateral pelvic girdle pain

Worse with movement

Interferes with sleep

Stiffness for at least 45 minutes in the morning

Question 15

Associated symptoms with PMR

Answer 15

Systemic - weight loss, fatigue, low grade fever and low mood

Upper arm tenderness

Carpel tunnel syndrome

Pitting oedema

Question 16

Differentials to exclude in PMR (9)

Answer 16

OA

RA

SLE

Myositis (from conditions like polymyositis or medications like statins)

Cervical spondylosis

Adhesive capsulitis of both shoulders

Hyper or hypothyroidism

Osteomalacia

Fibromyalgia

Question 17

Investigations to exclude other diagnoses (9)

Answer 17

Urine dipstick

Full blood count
Urea and electrolytes
Liver function tests
Thyroid function test

Calcium
Raised in hyperparathyroidism or cancer
Low in osteomalacia

Serum protein electrophoresis for myeloma and other protein disorders

Myositis - Creatine kinase

RA - Rheumatoid Factor

Question 18

Additional investigations to consider in PMR

Answer 18

Anti-nuclear antibodies (ANA) for systemic lupus erythematosus

Anti-cyclic citrullinated peptide (anti-CCP) for rheumatoid arthritis

Urine Bence Jones protein for myeloma

Chest xray

Question 19

Treatment of PMR

Answer 19

Steroids

Initially 15mg/day

Assess at 1 week - Poor response = unlikely PMR
Assess 3-4 weeks after starting steroids. You would expect a 70% improvement in symptoms

Slow wean