Giant Cell Arteritis & Polymyalgia Rheumatica

Question 1

• Vasculitis involving major branches of the aorta, primarily cranial arteries

Answer 1

Giant cell arteritis aka temporal arteritis

Question 2

• syndrome of muscle pain and stiffness involving the neck, shoulder and hips

Answer 2

Polymyalgia rheumatica (PMR)

Question 3

• inflammatory cells in vessel wall with reactive damage, loss of vessel integrity. Narrowing of the vessel lumen leads to downstream tissue ischemia and organ damage
• categorized by vessel size: small, medium, large

Answer 3

Vasculitis

Question 4

when should you be suspicious of giant cell arteritis?

Answer 4

diagnosis should be considered in pt > 50 years with

• new headaches
• abrupt onset of visual disturbances, especially transient monocular visual loss
• jaw claudication (typically with exertion, aka chewing)
• unexplained fever, anemia, or other constitutional symptoms and signs
• high ESR and/or CRP
• current or prior PMR

Question 5

epidemiolgoy of GCA?

Answer 5

• most common vasculitis
• incidence progresses with age- average age 72, almost never < 50
• women:men, 2:1

Question 6

GCA can present as?

Answer 6

• fever/chills- most common rheumatic cause of FUO in elderly
• anorexia, weight loss
• night sweats
• weakness-generalized
• depression

Question 7

GCA laboratory features

Answer 7

• ESR > 100 is common
• mild to moderate anemia
• increased platelets

Question 8

ACR classification criteria for GCA?

Answer 8

• age >/= 50 at time of onset
• localized headache of new onset
• tenderness or decreased TA pulse
• ESR > 50mm/hour
• positive TA biopsy with necrotizing arteritis & mononuclear cells or a granulomatous process with multinucleated giant cells

Question 9

GCA treatment?

Answer 9

• Do not delay treatment
• first line- prednisone 1mg/kg (should be tapered slowly over 1-2 years
• temporal artery biopsy within 2-4 weeks of treatment
• steroid sparing- tocilizumab
• methylprednisone 1000mg IV for visual loss prevention

Question 10

What extra-cranial manifestiation is possible with GCA?

Answer 10

Ischemic optic neuropathy

Question 11

what should be told to pts with GCA?

Answer 11

Relapses common 1-2 years after diagnosis

Question 12

• Age greater than 50 years
• pain and stiffness > 4 weeks duration in muscles of the neck, shoulder girdle and pelvic girdle-symmetric fashion
• dramatic clinical response to small doses of prednisone
• lab evidence of systemic inflammation
• 2-3x more common than GCA

Answer 12

Polymyalgia rheumatica

Question 13

Clinical manifestations of PMR

Answer 13

subjective complaints

• proximal muscle stiffness
• malaise, fatigue, weight loss, anorexia
• high spiking fevers- consider GCA
• swelling of the hands, wrists, ankles and top of feet

Objective

• tenderness (but not as pronounced as subjective stiffness)-neck, shoulders, low back, hips, thighs
• muscle strenth is normal
• decreased ROM shoulders, neck, and hips
• peripheral synovitis in 10%

Question 14

laboratory findings in PMR?

Answer 14

• moderate to high elevation in ESR and CRP
• mild to moderate anemia
• normal WBC
• ANA, RF, CPK are normal

Question 15

Diagnosis of PMR?

Answer 15

• age > 50
• clinical symptoms of at least 2/3 areas: neck or torso, shoulders or proximal regions of arms, hips or proximal aspects of thighs
• elevated ESR
• rapid response to prednisone
• biopsy not indicated to r/o GCA

Question 16

Treatment of PMR

Answer 16

• lower doses of corticosteroids sufficient in most cases- prednisone 10-20mg/day
• if high doses needed to control sx, be concerned for underlying GCA
• minimum treatment duration of 1-2 years
• monitor patient closely for signs of GCA
• tolicizumab role uncertain

Question 17

GCA/PMR prognosis

Answer 17

• most significant morbidity- reduced blood flow to the eye and optic nerve leading to blindness
• rare CVA in GCA
• PMR- self limiting disease. A small proportion will develop a chronicm symmetric polyarthritis