Original case by Brandon Fainstad, MD.
Edits and graphics by Yilin Zhang, MD.
- Differentiate the causes of aortitis
- Evaluate for cardiac tamponade in a patient with pericardial effusion
- Recognize the clinical presentation of giant cell arteritis
70 year old woman was in her usual, high functioning state of health until 8 weeks prior to presentation when she developed an episodic headache that started over the top of her head and radiated to her temples and jaw. Her headache was worse with eating but had no associated vision changes and spontaneously resolved 2 weeks ago. About 4 weeks ago she developed a dry cough and for the past two weeks she has experienced chest pressure and SOB when climbing a flight of stairs. Initially, the chest pain and SOB would resolve after a brief rest but today they developed while walking on flat ground and did not resolve until after resting for 10 min. She also admits to new 2 pillow orthopnea, increased swelling in her legs and 5lb weight gain over the past 10 days. She denies any ongoing headache or fevers, chills, nasal congestion, sore throat or rashes. She has no known medical problems and does not take any medications. She has never smoked and rarely drinks a glass of wine.
On exam she is afebrile, HR 87, BP 170/90 in both arms, RR of 22 and SpO2 of 97% on room air. She appears comfortable at rest, no tenderness over her jaw or temples, no increased work of breathing, but does have dullness to percussion at L lung base with bilateral basilar crackles. Heart rhythm is regular with a 3/6 systolic murmur loudest at the apex and a friction rub audible over the anterior precordium, JVP to 8 cm H2O and and trace dependent edema. No rashes appreciated.
- Na 132, BUN 25, Cr 1.2, Glu 125
- WBC 12, Hct 26, Plt 400
- Alb 3.0 otherwise LFTs nml
- Trop 0.05
- BNP 250
- ESR 115, CRP 150
What is your differential for his cardiac silhouette and what would be your next step in evaluation?
Differential includes dilated cardiomyopathy or pericardial effusion. This can be further evaluated with echocardiogram or cross sectional imaging (e.g., CT scan).Click for CT scan interpretation.
TTE showed non-dilated LV with LVEF 45% and apical wall motion abnormalities. Dilated ascending aorta measuring 4.7cm. Moderate circumferential pericardial effusion.
What could you do to further assess the patient before calling cardiology?
How can you tie together this patient’s presentation and clinical findings?
How can you differentiate between aortitis (inflammatory) versus non-inflammatory aortic aneurysm?
Suspected Giant Cell Arteritis – Based on the history of bitemporal and jaw pain in an elderly woman there was high suspicion for GCA. High dose prednisone was started on admission and opthomology performed temporal artery biopsies a week later which were negative for evidence of GCA. However, black blood MRI of the chest confirmed aortitis and large vessel vasculitis of the subclavian arteries.
Reduced EF with LAD distribution wall motion abnormalities – Noted on TTE and subsequent nuclear perfusion stress test confirmed prior LAD distribution infarct which was felt to be a combination of underlying atherosclerosis and vasculitis. She was initiated on standard heart failure medications. At one month follow-up she was NYHA I without evidence of volume overload.
Pericardial Effusion – The patient’s moderate effusion was thought to be related to adjacent vessel inflammation. She did not have clinical evidence of tamponade, and with initiation of prednisone, the effusion almost completely resolved by the time of discharge.
Ascending Aortic Aneurysm – Dilated to 4.7 cm on TTE , reduced to 4.1cm on subsequent MRI was felt to be related to her GCA. The plan was to involve CT surgery if the aneurysm exceeded 5 cm.
TEACHING POINT (adds ~ 5-7 min)
Giant Cell Arteritis (GCA)
Giant cell arteritis is a medium and large vessel vasculitis that predominantly affects the elderly. The aorta and its branches are most commonly affected.
- Incidence of disease increases with age and is rare in patients younger than 50 years old2
- Women are affected 2-3x more often than men2
- Large vessel complications of disease such as aortitis and aortic aneurysms occur in ~ 25% of patients with disease (click for additional information on large vessel complications)
- Many cases of subclinical and asymptomatic
- 18-33% of patients develop aortic dilation or aneurysms
- Large vessel complications can infrequently occur in the absence of cranial symptoms
- Suggested by typical clinical signs/symptoms and elevated inflammatory markers (ESR, CRP)
- Gold standard is temporal artery biopsy
- High dose steroids
- Should be initiated prior pathologic confirmation of diagnosis if there is high suspicion of disease
- Delay in initiating treatment can result in irreversible complications such as blindness
- No international consensus on monitoring for large vessel complications of disease and aortic involvement
- A French group (GEFA, Groupe d’E´tude Francais des Arterites) recommends imaging to screen for complications at diagnosis2
- More commonly, evaluation of large vessel complications is driven by clinical signs/symptoms
TAKE HOME POINTS:
- Don’t delay steroids for biopsy in suspected GCA
- Clinical detection of aortic aneurysm occurs in 10-20% of patients with GCA, but is often clinically silent and this is likely an underestimation the overall incidence.
- Cardiac tamponade is a clinical diagnosis based on hypotension, elevated JVP, and pulsus paradoxus
- Roy CL, Minor MA, Brookhart MA, Choudhry NK. Does This Patient With a Pericardial Effusion Have Cardiac Tamponade?. JAMA. 2007;297(16):1810–1818.
- Hoffman GS. Giant Cell Arteritis. Ann Intern Med. 2016;165:ITC65–ITC80.
- Buttgereit F, Dejaco C, Matteson EL, Dasgupta B. Polymyalgia Rheumatica and Giant Cell Arteritis: A Systematic Review. JAMA. 2016;315(22):2442–2458