441 lines
25 KiB
Markdown
441 lines
25 KiB
Markdown
---
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title: "Giant Cell Arteritis"
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docid: "208eca17-81b8-448c-b8be-80e274dccc42"
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authors:
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- key: "ee6ece9d-ad74-458c-a8df-11628ae7f879"
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value: "Arzu Canan, MD"
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- key: "3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1"
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value: "Suhny Abbara, MD, FACR, MSCCT, FNASCI"
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- key: "10bb95ac-a27a-4ebe-833b-e59fea07734b"
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value: "Santiago Mart\u00ednez-Jim\u00e9nez, MD, FACR"
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breadcrumbs:
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-
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name: "Cardiac"
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slug: "cardiac"
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treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
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-
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name: "Diagnosis"
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slug: "diagnosis"
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treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
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-
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name: "Aorta"
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slug: "aorta"
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treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
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-
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name: "Giant Cell Arteritis"
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slug: "giant-cell-arteritis"
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treeNodeId: null
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category: "Cardiac"
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documentVersionId: "9aabd711-33a6-4598-b5e2-495eff0adf14"
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imageCount: 16
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lastUpdated: "11/14/24"
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pageDescription: "Giant Cell Arteritis"
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pageKeywords: "Cardiac, Diagnosis, Aorta, Giant Cell Arteritis"
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pageTitle: "Giant Cell Arteritis | STATdx"
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enhancedTitle: "Giant Cell Arteritis"
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type: "DX"
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references: true
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breadcrumbs:
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- "Cardiac"
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- "Diagnosis"
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- "Aorta"
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- "Giant Cell Arteritis"
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---
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# KEY FACTS
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- ## Terminology
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- Chronic, systemic, large or medium-sized, often granulomatous vasculitis
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- Often involves thoracic aorta and major branches
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- Often involves temporal artery
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- ## Imaging
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- CTA
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- Concentric aortic thickening (> 2 mm)
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- Aortic aneurysm; classically ascending aorta
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- Aortic dissection: Intimomedial flap
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- MR
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- Assessment of active inflammation
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- Delayed enhancement after gadolinium
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- Ultrasonography
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- High specificity and sensitivity; operator dependent
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- Hypoechoic halo temporal &/or axillary arteries
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- PET
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- Active inflammation demonstrates ↑ FDG uptake
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- ## Top Differential Diagnoses
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- Takayasu arteritis
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- May be identical to GCA
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- Extremely rare in patients > 50 years
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- Atherosclerotic disease
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- May be difficult to differentiate radiographically, though clinical symptoms often facilitate process
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- Similar age group
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- ## Clinical Issues
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- Headache, visual disturbances, jaw claudication
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- Polymyalgia rheumatica
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- Serologic markers
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- ↑ sedimentation rate
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- ↑ C-reactive protein
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- Thrombocytosis
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- Treatment
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- Corticosteroids
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# TERMINOLOGY
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- ## Abbreviations
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- Giant cell arteritis (GCA)
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- ## Synonyms
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- Temporal arteritis
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- Cranial GCA (C-GCA) often referred to as temporal arteritis; terminology not longer recommended, as sparing of temporal artery is not uncommon and because disease may involve large vessels
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- Horton disease
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- ## Definitions
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- Granulomatous autoimmune vasculitis affecting larger arteries and aorta
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- C-GCA: Often involves temporal artery and other head/neck vessels, but may also involve aorta and major branches
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- Large-vessel GCA (LV-GCA): Often involves thoracic aorta and major branches
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- Frequently associated with polymyalgia rheumatica (PMR)
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- Aching and morning stiffness in shoulders, hip girdle, and neck
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# IMAGING
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- ## General Features
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- ### Location
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- Temporal artery
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- Aorta and aortic branches
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- ## CT Findings
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- ### NECT
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- Typically, GCA involving aorta is not as apparent or dense as intramural hematoma; however, there can be hyperdensity if associated with hemorrhage or calcification
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- Transmural calcification is often similar to calcified atherosclerotic plaques (common)
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- ### CTA
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- Concentric aortic thickening (> 2 mm)
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- Aortic stenosis
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- Aortic aneurysm; classically ascending aorta
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- Aortic dissection: Intimomedial flap
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- Limited role in C-GCA
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- ## MR Findings
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- Equally accurate as CT for morphologic assessment on several sequences (e.g., T1WI, T2WI, HASTE, SSFP, etc.)
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- Contrast-enhanced MRA is more accurate to assess areas of stenosis and aneurysm
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- Assessment of active inflammation
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- Contrast-enhanced sequences: Delayed enhancement (i.e., ↑ signal) of vessel wall after gadolinium
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- Fat-saturated STIR sequence: High signal of thickened vessel wall
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- Cranial (temporal artery) involvement
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- High sensitivity and specificity
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- Mural thickening (> 0.5 mm)
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- Mural high T2 signal and contrast enhancement
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- ## Ultrasonographic Findings
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- ### Grayscale ultrasound
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- C-GCA
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- High specificity and sensitivity; operator dependent
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- Hypoechoic halo (i.e., **halo sign**) in temporal &/or axillary arteries
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- **Compression sign**: Persistence of halo during compression of vessel lumen by ultrasound probe
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- ### Color Doppler
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- Always in conjunction with grayscale ultrasound
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- Helpful to localize temporal artery
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- ## Angiographic Findings
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- Stenosis (often long, regular, and smooth-walled)
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- Occlusion
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- Aneurysm
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- Limited in diagnosis of early vasculitis
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- ## Nuclear Medicine Findings
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- ### PET
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- LV-GCA: Active inflammation demonstrates ↑ FDG uptake
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- Subclinical inflammation of large vessels in 80% with GCA and ~ 30% PMR
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- Response to treatment correlates with ↓ FDG uptake
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- Limited role in C-GCA, not recommended
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- ## Imaging Recommendations
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- ### Best imaging tool
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- MR
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- STIR: Thickening and high signal of aortic wall
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- Contrast-enhanced MR: Thickening and enhancement of aortic wall
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- MRA is helpful to detect areas of stenosis and aneurysm
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- ### Protocol advice
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- Consider concomitant NECT to differentiate from intramural hematoma
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- Caveat: GCA can occasionally be hyperdense
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- PET
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- Recognized role in patient with fever &/or inflammation of unknown origin
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- Unclear role in follow-up, especially asymptomatic patients without elevated inflammatory markers
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# DIFFERENTIAL DIAGNOSIS
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- [Takayasu Arteritis](/document/takayasu-arteritis/3b589c7b-d975-4f2c-a5b1-ff83dd856ee7)
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- May have similar imaging appearance to GCA
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- Rare in patients > 50 years old
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- [Other Systemic Vasculitides](/document/polyarteritis-nodosa/5c1ed46f-9132-4903-830d-1907a0774c7d)
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- e.g., polyarteritis nodosa, syphilitic aortitis
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- Occurs most often in small and medium-sized arteries
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- Biopsy and pattern of distribution often help differentiation
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- ## Fibromuscular Dysplasia
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- Most often affects renal arteries
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- Can also involve carotid arteries
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- Results in stenoses; occasional spontaneous dissection
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- [Atherosclerotic Disease](/document/atherosclerosis/41278e3c-2240-4122-b555-8776d0918082)
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- May be difficult to differentiate radiographically, though clinical symptoms often facilitate process
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- Similar age group
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Unknown
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- Most accepted hypothesis: Antigen-driven disease mediated by T cells and macrophages that reach aortic wall via vasa vasorum
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- ## Staging, Grading, & Classification
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- Temporal artery biopsy remains diagnostic gold standard for C-GCA
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- Predictors of positive temporal artery biopsy
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- Jaw claudication
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- Neck pain
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- C-reactive protein > 2.45 mg/dL
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- Sedimentation rate > 47 mm/h
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- Thrombocytosis
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- Pallid optic disc edema
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- Temporal artery abnormalities
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- Temporal artery biopsy can be negative (10-15%)
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- ## Gross Pathologic & Surgical Features
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- Involvement of aorta (65.0%)
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- Involvement of main aortic tributaries (57.5%)
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- Brachiocephalic trunk (47.5%)
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- Subclavian arteries (42.5%)
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- Carotid arteries (35.0%)
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- Femoral arteries (30.0%)
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- Splanchnic arteries (22.5%)
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- Axillary arteries (17.5%)
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- Iliac arteries (15.0%)
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- Renal arteries (7.5%)
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- ## Microscopic Features
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- Focal chronic inflammatory cell infiltrates
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- Granulomas in vessel wall formed by CD4(+) T cells and macrophages
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- Focal areas of intimal hyperplasia
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- Proliferation of smooth muscle cells, which leads to narrowing of arterial lumen and eventually ischemia
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- Focal areas of fragmentation of inner elastic lamina
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- Focal concentric scars around inner elastic lamina
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Headache
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- Visual disturbances
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- Jaw claudication
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- ### Other signs/symptoms
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- PMR
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- Present in 50% of patients at diagnosis of GCA
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- 20% of PMR will develop GCA
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- Clinical manifestations
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- Morning stiffness
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- Pain (shoulder > hip or neck)
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- Synovitis and bursitis
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- Swelling and tenosynovitis
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- ↓ range of motion
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- Muscle tenderness
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- Subjective weakness
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- Systemic signs and symptoms (e.g., malaise, fatigue, depression, anorexia, weight loss, fever)
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- ### Clinical profile
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- Clinical phenotypes
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- C-GCA (temporal arteritis with headache and visual disturbance)
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- LV-GCA (arm/limb claudication, chest pain)
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- PMR
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- Phenotypes can overlap
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- Serologic markers
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- ↑ erythrocyte sedimentation rate
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- ↑ C-reactive protein
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- Thrombocytosis
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- Association with HLA-DRB1*04
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- LV-GCA linked to other systematic diseases, such as Behçet disease or hyper-IgG4 syndrome
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- Factors for aneurysm formation
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- Aortic insufficiency
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- Murmur at time of diagnosis
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- Hyperlipemia
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- ↑ eritrosedimentation in combination with polymyalgia symptoms
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- ↑ levels of IL-2
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- ## Demographics
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- ### Age
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- Patients > 50 years old
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- Incidence ↑ steadily with age
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- ### Sex
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- Women > men
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- ### Ethnicity
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- More common in people of Northern European and Scandinavian descent
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- ### Epidemiology
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- Prevalence in USA: 1 in 160,000
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- Lifetime risk of developing GCA in USA: 1% in women and 0.5% in men
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- ## Natural History & Prognosis
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- Prognosis for visual recovery is poor
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- ↑ risk aortic aneurysm formation and dissection: 17-fold and 2.5x higher risk of thoracic and abdominal aortic aneurysms
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- ↓ survival rate
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- Involvement of coronary arteries may result in myocardial infarction or congestive heart failure
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- Bowel necrosis (uncommon)
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- 15-30% of PMR cases eventually develop GCA
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- ## Treatment
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- GCA and PMR: Corticosteroids
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- Aspirin
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- Other (2nd-line therapy)
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- Methotrexate
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- Azathioprine
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- Tocilizumab (IL-6 receptor alpha inhibitor)
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# DIAGNOSTIC CHECKLIST
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- ## Consider
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- Annual surveillance to assess for aneurysm and dissection
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- Alternatives for follow-up
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- Chest radiograph + echocardiogram + abdominal Doppler ultrasound
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- Contrast-enhanced CT of chest and abdomen
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a728420c-dc69-475b-9a5a-54a5aee00e78
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## References
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# Selected References
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1. [Pepper K: Giant cell arteritis. Postgrad Med. 135(sup1):22-32, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37021621%5Bpmid%5D)
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1. [Braun J et al: The role of 18F-FDG positron emission tomography for the diagnosis of vasculitides. Clin Exp Rheumatol. 36 Suppl 114(5):108-14, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30296989%5Bpmid%5D)
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1. [Dejaco C et al: The spectrum of giant cell arteritis and polymyalgia rheumatica: revisiting the concept of the disease. Rheumatology (Oxford). 56(4):506-15, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27481272%5Bpmid%5D)
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1. [Gomułka K et al: Horton's disease: still an important medical problem in elderly patients: a review and case report. Postepy Dermatol Alergol. 34(5):510-3, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29507571%5Bpmid%5D)
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1. [Buttgereit F et al: Polymyalgia rheumatica and giant cell arteritis: a systematic review. JAMA. 315(22):2442-58, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27299619%5Bpmid%5D)
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1. [Aschwanden M et al: The ultrasound compression sign to diagnose temporal giant cell arteritis shows an excellent interobserver agreement. Clin Exp Rheumatol. 33(2 Suppl 89):S-113-5, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26016760%5Bpmid%5D)
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1. [Khan A et al: Imaging in giant cell arteritis. Curr Rheumatol Rep. 17(8):527, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26113013%5Bpmid%5D)
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1. [Hartlage GR et al: Multimodality imaging of aortitis. JACC Cardiovasc Imaging. 7(6):605-19, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24925329%5Bpmid%5D)
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1. [Schmidt WA: Ultrasound in vasculitis. Clin Exp Rheumatol. 32(1 Suppl 80):S71-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24529335%5Bpmid%5D)
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1. [Jennette JC et al: 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 65(1):1-11, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23045170%5Bpmid%5D)
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1. [Blockmans D: Diagnosis and extension of giant cell arteritis. Contribution of imaging techniques. Presse Med. 41(10):948-54, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22795837%5Bpmid%5D)
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1. [Castañer E et al: Imaging findings in pulmonary vasculitis. Semin Ultrasound CT MR. 33(6):567-79, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23168065%5Bpmid%5D)
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1. [Bossert M et al: Aortic involvement in giant cell arteritis: current data. Joint Bone Spine. 78(3):246-51, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21030278%5Bpmid%5D)
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1. [Falardeau J: Giant cell arteritis. Neurol Clin. 28(3):581-91, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20637990%5Bpmid%5D)
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1. [Bley TA et al: Diagnostic value of high-resolution MR imaging in giant cell arteritis. AJNR Am J Neuroradiol. 28(9):1722-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17885247%5Bpmid%5D)
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## Images
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### Selected Images
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*Axial CTA of the chest in a patient with giant cell arteritis (GCA) shows soft tissue <img src='img/arrows/WC.png'/> density material surrounding the great vessels. (Courtesy C. S. Restrepo, MD.)*
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*Axial CTA of the chest in a patient with giant cell arteritis (GCA) shows soft tissue <img src='img/arrows/WC.png'/> density material surrounding the great vessels. (Courtesy C. S. Restrepo, MD.)*
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*Axial chest CTA in the same patient shows concentric thickening of the thoracic aorta, which is a common finding in patients with GCA but is indistinguishable from Takayasu arteritis. GCA is more common in patients > 50 years old. Concomitant NECT is recommended to help differentiate from intramural hematoma. (Courtesy C. S. Restrepo, MD.)*
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*Coronal FDG PET/CT in the same patient shows marked uptake of FDG along the ascending aortic wall <img src='img/arrows/BC.png'/>. FDG PET has excellent sensitivity and specificity for the diagnosis of GCA and may be used when clinical or serological discrepancies arise during or after treatment of this condition.*
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*Coronal FDG PET/CT in a patient with GCA shows diffuse uptake along the ascending aortic wall <img src='img/arrows/WC.png'/> as well as along the subclavian and axillary arteries bilaterally <img src='img/arrows/WS.png'/>.*
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*Axial CTA in a patient with GCA shows diffuse arterial wall thickening <img src='img/arrows/CS.png'/> and stranding of the periaortic fat. Note the reactive left pleural effusion <img src='img/arrows/CC.png'/>.*
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*Axial double inversion recovery FS MR in the same patient at different levels shows diffuse high signal of the aortic wall <img src='img/arrows/CS.png'/> as well as head and neck vessels <img src='img/arrows/CC.png'/>. MR is the preferred method to assess for active inflammation also seen in the form of vessel parietal enhancement after intravenous gadolinium.*
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*Sagittal reformat CECT in a patient with GCA before and after contrast shows focal parietal thickening along the posterior descending thoracic aorta, only evident on CECT <img src='img/arrows/CS.png'/>. Typically, vasculitis is not hyperdense on NECT as opposed to intramural hematoma.*
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*3D GRE MR (unenhanced and post contrast) at the same level shows progressive enhancement of the aortic wall after administration of intravenous contrast <img src='img/arrows/CS.png'/>.*
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*Axial CTA in a patient with unsuspected GCA who underwent reconstruction of the ascending aorta due to aneurysm is shown. Note the aneurysmal ascending <img src='img/arrows/CS.png'/> and descending aorta <img src='img/arrows/CC.png'/>.*
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*Sagittal CECT MIP in the same patient shows diffuse aneurysmal thoracic aorta <img src='img/arrows/CS.png'/>. Note also the aneurysmal right brachiocephalic trunk <img src='img/arrows/CO.png'/>. Aneurysm is a very common complication of undiagnosed and untreated GCA only evident after resection.*
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### Additional Images
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*Axial CTA in a young patient shows mural thickening of the supraaortic great vessels <img src='img/arrows/WS.png'/> and stranding of the adjacent perivascular fat due to a vasculitis. Note the stenosis of the left common carotid artery <img src='img/arrows/WO.png'/>.*
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*Axial GRE MR following gadolinium administration shows marked circumferential mural thickening and enhancement of the descending thoracic aorta <img src='img/arrows/WO.png'/>, consistent with active arteritis.*
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*Axial GRE MR following gadolinium administration in the same patient confirms the presence of mural thickening and enhancement of the supraaortic arteries <img src='img/arrows/WC.png'/>, consistent with active GCA.*
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*Coronal contrast-enhanced MRA MIP in the same patient confirms multiple stenoses <img src='img/arrows/WC.png'/> of the proximal pulmonary arteries without intraluminal thrombus. These are nonspecific features that are consistent with a pulmonary vasculitis, including GCA.*
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*Axial CTA shows circumferential soft tissue thickening of the aortic arch in a patient with GCA. This represents an inflammatory reaction resulting in aortic mural thickening <img src='img/arrows/WO.png'/>.*
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*Axial CTA shows irregular mural thickening of the descending thoracic aorta <img src='img/arrows/WS.png'/> and pulmonary arteries <img src='img/arrows/WO.png'/>. Mural thickening represents a common sequela of inflammatory arteritis.*
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