High Yield Review - Giant Cell ( Temporal ) Arteritis:
Case: A 50 year old woman, who started to have a Right sided head ache for the last few days. She has never had head aches prior. She points to to where her head ache is and shows you where it is tender ( see below ). She has also had blurry vision in her Right eye.
She says she thought it was her husband. She looks at him and says "you are giving me a GIANT head" because all he complains about is how terrible his fantasy football team is doing.
But she was also blaming it on her menopause symptoms because she has been feeling hot and feverish.
She has been having trouble with her morning exercising , because she has been feeling so stiff, and achey in her shoulders and hips and felt so tired she has not attended her yoga classes at all.
- She has decreased range of motion of shoulders and hips
- ESR 55
- Hemoglobin is 10 and MCV is 90
You suspect that she has Giant Cell Arteritis in association with Polymalgia Rheumatica, you are concerned because she has
blurry vision. With out delay you start her on high dose prednisone 1mg/kg/day. And send her for an emergent Ophthalmology evaluation and referral for temporal biopsy for confirming the diagnosis. She returns for a follow up and she
is rapidly improved in all her symptoms.
So What If ?
What If temporal biopsy is negative?
-Then there is the possibility of skip lesions and she may need a contralateral biopsy performed ( do this with Ultrasound guidance / dopplers )
What If she had no cranial symptoms or the biopsy is negative?
- MR CT Angio / MR Angio of the Chest and Neck
What If she relapses on steroids or is steroid resistant?
- Then you would start other immunodulator therapy ( like Methrotrexate )
Case Clues - Giant Cell Arteritis
- She is 50 years old which is the greatest risk factor GCA
- She has never had head aches prior and points to her right side so this is a new onset and localized head ache in a 50 y/o
- She has Tenderness and decreases pulses of the temporal artery
- She feels hot, fatigued so fever and fatigue in a patient > 50 y/o could be GCA/Polymyalgia Rheumatica
- She has morning stiffness in her shoulders and hips ( so is presenting like polymyalgia rheumatica ( that is considered the same spectrum of disease )
- ESR > 50 and she has a normocytic anemia ( labs you see in GCA/ Polymyalgia Rheumatica )
Notable Notes - Giant Cell Arteritis ( GCA)
- Large/ Medium Vessel Vasculitis
- Affects mostly branches of the carotid artery
- Accompanied with Polymyalgia Rheumatica up to 50% of the time
This is based on clinical criteria ( 3 of 5 needed )
- Age ( >/= 50 )
- Tender Temporal Artery, or decrease pulsations
- Temporal Artery Biopsy --> Shows Necrotizing Arteritis
- New onset Head Ache + Fever + Visual Disturbances
- 50 ESR
- If you suspect GCA ( 3/5 clinical criteria ) then start high dose steroids
- high dose steroids ( prednisone 1mg/kg/day and there will Rapid response to treatment , start tapering after 4-6 weeks
- Start low dose Aspirin to prevent cerebral ischemia
- If there are GCA Flares then other immunomodulators needed ( like methotrexate )
Why does GCA cause blindness ?
- Posterior ciliary and Ophthalmic arteries are affected resulting
in ischemia of the optic nerve
More Notable Notes - GCA
- The Temporal Artery biopsy may be negative due to skip lesions
- If the Temporal biopsy is negative, then biopsy of the contralateral artery is indicated ( but not commonly performed, we treat based on clinical criteria)
- Treat ASAP If there is clinical suspicion, there is no need for delay for the biopsy results ( why? because even if biopsy is obtained later after steroids have been started changes wont be seen until weeks later )
- What if there is NO cranial symptoms? What if the Temporal Artery biopsy is negative? A 50 y/o has fever and other constitutional symptoms suggestive of GCA. So now obtain imaging such as CT Angio or MR Angiography of neck and chest is needed ( Why? because GCA can involve the aorta and extra-cranial arteries of the head and neck like the carotid and subclavian).
If they show you the histology / biopsy on the exam
- Seen in patients > 50 years old
- Proximal muscle morning stiffness / Body aches/ and decrease ROM
- Associated with fevers and weight loss
- Considered the same spectrum of disease ( so consider a DDX of GCA in a patient with Polymyalgia Rheumatica)
Treating Polymyalgia Rheumatica
-Low dose steroids with tapering over 6 months
- Remember GCA was high dose steroids
Notable notes - Polymyalgia Rheumatica
- May see a normocytic anemia in the setting of acute phase reactants (ESR,CRP )
Know it for the boards- Giant Cell Arteritis!
- Suspect GCA in a patient with headache and fevers 50 y/o or older
- Both GCA and Polymyalgia Rheumatica have a rapid response to glucocortiocids
- GCA is treated with high dose Prednisone 1mg/kg/day
- Polymyalgia Rheumatica is treated with low dose Prednisone
- The Temporal artery biopsy may be negative due to skip lesions or because there is other vessel involvement
- If you suspect GCA and If there are no cranial symptoms then get CT Angio / MR Angio Neck and Chest
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