Korean J Radiol.  2017 Oct;18(5):786-798. 10.3348/kjr.2017.18.5.786.

CT Features of Vasculitides Based on the 2012 International Chapel Hill Consensus Conference Revised Classification

• Hur JH
• Chun EJ
• Kwag HJ
• Yoo JY
• Kim HY
• Kim JJ
• Lee KW

• KMID: 2427215
• DOI: http://doi.org/10.3348/kjr.2017.18.5.786

Abstract

Vasculitis, characterized by inflammation of vessel walls, is comprised of heterogeneous clinicopathological entities, and thus poses a diagnostic challenge. The most widely used approach for classifying vasculitides is based on the International Chapel Hill Consensus Conference (CHCC) nomenclature system. Based on the recently revised CHCC 2012, we propose computed tomography (CT) features of vasculitides and a differential diagnosis based on location and morphological characteristics. Finally, vasculitis mimics should be differentiated, because erroneous application of immunosuppressive drugs on vasculitis mimics may be ineffective, even deteriorating. This article presents the utility of CT in the diagnosis and differential diagnosis of vasculitides.

Keyword

Vasculitis; Vasculitides; Computed tomography angiography; Computed tomography; Diagnosis

MeSH Terms

Behcet Syndrome/diagnosis/diagnostic imaging
Cogan Syndrome/diagnosis/diagnostic imaging
Consensus
Diagnosis, Differential
Giant Cell Arteritis/diagnosis/diagnostic imaging
Granulomatosis with Polyangiitis/diagnosis/diagnostic imaging
Humans
Mucocutaneous Lymph Node Syndrome/diagnosis/diagnostic imaging
Polyarteritis Nodosa/diagnosis/diagnostic imaging
Takayasu Arteritis/diagnosis/diagnostic imaging
Tomography, X-Ray Computed
Vasculitis/*diagnosis/diagnostic imaging

Figure

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  Fig. 1 Diagram of vasculitides categorized based on frequently affected vessel size and types. Drawing shows aorta, large artery, medium arteries, and small arteries or arterioles, capillaries, venules, and veins, in sequence from left to right. Modified from Jennette et al. Arthritis Rheum 2013;65:1-11 (4). ANCA = anti-neutrophil cytoplasmic antibody, Anti-GBM = anti-glomerular basement membrane, IgA = immunoglobulin A

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  Fig. 2 Early phase Takayasu arteritis in 55-year-old woman with bilateral arm pain. Axial CT scans obtained during delayed phase show typical concentric wall thickening with double rings (inner low and outer high enhancement) at ascending and descending aorta and ostia of both subclavian arteries (arrows).

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  Fig. 3 Late phase Takayasu arteritis in 71-year-old woman with dizziness. A. Volume rendered CT image shows massive patchy calcifications at aorta, indicating long-standing aortitis. B. Axial CT scan shows concentric wall calcification at ascending aorta (arrows). C. At level of arch vessels, total occlusion at left subclavian artery (arrows) was noted.

• 

  Fig. 4 Polyarteritis nodosa in 44-year-old man with hypertension. Curved MPR image clearly demonstrates right renal artery aneurysm (arrow). Focal stenosis (arrowheads) at ostium of right renal artery is noted. MPR = multiplanar reformatted

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  Fig. 5 Kawasaki disease in 18-year-old woman with chest pain. A. Curved MPR image shows multifocal aneurysms (arrows) with thrombi (arrowheads) at left circumflex coronary artery. B. Invasive angiogram reveals aneurysms (arrows) with stenosis (arrowhead) at left circumflex coronary artery.

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  Fig. 6 Granulomatosis with polyangiitis in 58-year-old man with epistaxis. A. CT scan demonstrates multiple non-cavitary nodules (arrows) in bilateral lungs, which are mainly distributed along bronchovascular bundles. B. PET scan shows multiple hypermetabolic pulmonary nodules (arrows; maximum SUV = 10.0). C. Bronchoscopy shows multiple whitish lesions at bilateral main bronchi. Biopsy reveals necrot

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  Fig. 7 Eosinophilic granulomatosis with polyangiitis in 53-year-old man with known asthma, generalized petechiae, and cardiac enzyme elevation. A. CT scan shows uneven distribution of left ventricular (LV) hypertrophy (double-headed arrows) at midventricular LV wall. B. Delayed enhanced MR image shows multifocal patchy enhancement (arrows) at subendocardial and mid-layer of midventricular LV wall. C. Chest CT scan shows bronchovascular bundle thickening, and interlobular septal thickening with some peribronchial and subpleural ground-glass opacities in bilateral lungs.

• 

  Fig. 8 Behçet's disease in 47-year-old man with hemoptysis. A. Initial chest CT shows large pulmonary artery pseudoaneurysm (arrow) at right lower lobe, which is not resolved with pulmonary artery embolization. B. Follow up CT 6 months after initial scan shows another pulmonary artery pseudoaneurysm (arrows) at left lower lobe.

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  Fig. 9 Behçet's disease in 49-year-old man with history of aortic valve replacement. Two months after aortic valve replacement, CT image shows paravalvular pseudoaneurysm (arrows) around aortic root.

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  Fig. 10 Cogan syndrome in 47-year-old female with interstitial keratitis and sensory neural hearing loss. A. Volume-rendered image shows tight stenosis (arrows) at ostia of right brachiocephalic, left common carotid, and left subclavian arteries. B. Sagittal reformatted CT image shows luminal irregularity (arrowheads) of abdominal aorta and tight stenosis (arrows) at ostia of celiac axis and superior mesenteric artery.

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  Fig. 11 IgG4-related sclerosing disease in 70-year-old man, with incidentally found abnormality of pancreas on routine ultrasound exam. A. CT shows enlarged pancreas tail and peripancreatic low attenuation (arrowheads) indicative of autoimmune pancreatitis. Homogeneous enhancement of aorta (arrows) is also noted. B. Circumferential wall thickening with homogeneous enhancement around infrarenal abdominal aorta (arrows) are noted. C. PET scan demonstrates hypermetabolism around abdominal aorta (arrows).

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  Fig. 12 IgG4-related periarteritis in 67-year-old man with chest pain. A. Coronary curved MPR image shows well-defined soft tissue encasement (arrows) along left anterior descending artery. B. Conventional angiography shows limitation in identifying coronary abnormalities in absence of any luminal abnormalities.

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  Fig. 13 Fibromuscular dysplasia in 20-year-old man with poorly controlled high blood pressure. A. CT shows segmental narrowing (arrow) and luminal irregularity of right renal artery. B. Angiogram of right renal artery shows focal narrowing of right renal artery, with classic string of beads appearance (arrow).

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  Fig. 14 Segmental arterial mediolysis in 40-year-old woman with abdominal pain Serial axial CT scans show focal dissection (arrowhead) at ostium of celiac axis and diffuse severe stenosis with extensive concentric wall thickening and beaded appearance at common and proper hepatic arteries (arrows).

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  Fig. 15 Diagram of key findings and sites of predilection for various vasculitides Section of vasculitis involving pulmonary artery is modified from Chung et al. Radiology 2010;255:322-341 (39). *Vasculitis mimics. SMA = superior mesenteric artery

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