Polymyalgia Rheumatica (PMR) Giant Cell Arteritis (GCA) Temporal Arteritis (TA)

Question 1

Polymyalgia Rheumatica (PMR) characteristics

Answer 1

Systemic inflammatory disorder
Symmetric, proximal muscle pain and stiffness
Symptom relief with prednisone

Question 2

Epidemiology

Answer 2

Mean age--73
Annual incidence--13 to 113 per 100,000
Caucasian >> African American,Hispanic
Women >> Men
Think of onset of symptoms over the age of 50

Question 3

Symptoms of PMR

Answer 3

95%--symmetric shoulder girdle pain
70%--neck and pelvic pain
Upper arm and thigh pain
Subacute
Morning >> Evening
Weight loss
Fever
Malaise
Difficulty standing
Difficulty grooming

Question 4

Physical Exam

Answer 4

Joint ROM –normal
Muscle strength –normal
Soft tissue swelling -knees, wrists, and MCP joints
Lower extremity edema
Carpel tunnel syndrome

Question 5

Diagnosis of PMR

Answer 5

No specific laboratory tests for PMR
***90%–ESR >40 mm/hr
CBC-leukocytosis, thrombocytosis, anemia.

Other lab abnormalities:
Alkaline phosphatase
C-reactive protein

Elevated CK is not seen in PMR- (because it is not muscle in nature)

No serological tests specific for the diagnosis of PMR
That is RA ANA etc

Question 6

Ddx for PMR

Answer 6

Rheumatic conditions
	myositis
	rheumatoid arthritis
	bursitis
	fibromyalgia
	osteoarthritis of shoulers
	impingement of shoulders
Drug toxicity (statins metabolic myopathy)
Hypothyroidism
Malignancies

Question 7

Treatment for PMR

Answer 7

Corticosteroids (CS) -treatment of choice
Prednisone 15 to 20 mg per day 10mg
Relief within days -if symptoms do not promptly improve -consider another diagnosis
Normalization of ESR/CRP will occur over weeks following symptom improvement

Question 8

Corticosteroid Treatment

Answer 8

Continue initial prednisone dose for 4 weeks

Taper to the lowest effective dose

Duration of therapy: 1-2 years

Relapses requiring increase in CS are common

Relapses should be based on clinical symptoms and not a rise in ESR/CRP alone

Question 9

Adverse events of Corticosteroid treatment

Answer 9

Infection
Glaucoma
Cataracts
Hypertension
Diabetes
Osteoporosis
Skin atrophy
Easy bruisability

Question 10

How to monitor for GCA

Answer 10

Asking about GCA symptoms at follow-up visits

At clinic visits monitor examination for:

	- unequal blood pressures between 			extremities
	- bruits
	- new murmurs of aortic insufficiency

Question 11

GCA/TA Characteristics

Answer 11

Inflammatory disease of large-and medium-sized vessels
Most commonly affected vessels:
extracranial branches of carotid arteries
primary branch arteries of aorta (any branch)

Question 12

GCA histopathology

Answer 12

Granulomatous inflammation
Multinucleated giant cells
Inflammation causes luminal narrowing
Visual loss
Strokes

Question 13

GCA epidemiology

Answer 13

Most common systemic vasculitis in humans

18 of 100,000 people

Mean age–74

Question 14

Symptoms of GCA

Answer 14

Headache
Jaw claudication
PMR
Fever
Constitutional
Amarosus fugax
Irreversible blindness

Question 15

Non specific symptoms of GCA

Answer 15

Cough

Throat pain

Tongue pain

Aortic branch vessel involvement

Arm or leg claudication

Question 16

Complications associated with GCA

Answer 16

Stroke
Aneurysm formation
Visual complications
Visual loss –30%

Question 17

Physical Exam for GCA

Answer 17

Abnormal temporal artery
Asymmetric blood pressures
Bruits
Murmur of aortic insufficiency

Question 18

Diagnosis of GCA

Answer 18

Clinical features supported by laboratory values
Erythrocyte sedimentation rate
C-reactive protein– always high
Thrombocytosis
Elevated alkaline phosphatase
No serological test specific for GCA
Temporal artery biopsy --gold standard
Imaging to look for large vessel involvement

Question 19

Main treatment of GCA

Answer 19

Prednisone--treatment of choice
	Improves symptoms
	Prevents blindness
Therapy should not be withheld to pursue biopsy
Initial dose:
	Prednisone 40-60 mg per day

Question 20

ASA for GCA

Answer 20

Daily, oral aspirin
81 mg per day
Reduce risk of stroke and blindness
No increased risk of gastrointestinal complications
Aspirin should be given to all patients with GCA who do not have contraindications.

Question 21

Outcomes of GCA

Answer 21

Mortality risk from the late development of aortic aneurysms
-Dissection or rupture

Patients with GCA have 17x higher frequency of thoracic aortic aneurysm

Annual monitoring for aneurysm development

At clinic visits monitor examination for:

- murmur of aortic insufficiency
- bruits
- unequal blood pressures between extremities