Imaging in Polyarteritis Nodosa

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Polyarteritis nodosa (PAN) is an autoimmune systemic inflammatory vasculitis that results in transmural fibrinoid necrosis with surrounding inflammation in small and medium-size vessels (see the following images). This condition commonly affects the kidneys, heart, liver, and gastrointestinal (GI) tract, with the kidney being the organ most commonly involved (79% of cases at autopsy). ^{[1, 2, 3, 10]}

For an accurate diagnosis of polyarteritis nodosa, selective arteriography is the criterion standard. In the past, biopsy was used. Analysis of biopsy specimens from the subcutaneous nodules or skeletal muscle may be helpful in establishing the diagnosis, although biopsy has a success rate of only 20-35%. Although biopsy was used more extensively in the past, this procedure has fallen into disfavor because of sampling errors and its low success rate. Angiography can be helpful in confirming or supporting the clinical diagnosis of polyarteritis nodosa; however, this procedure is an invasive test. ^{[4, 5, 6, 7]}

The differential diagnosis includes Churg-Strauss disease, Churg-Strauss syndrome, leukocytoclastic vasculitis, systemic lupus erythematosus, and Wegener granulomatosis. Other conditions that should be considered are acute glomerulonephritis, drug-induced vasculitis, and vasculitis associated with lupus, scleroderma, rheumatoid arthritis, and Wegener granulomatosis.

Transcatheter embolization should be considered only in cases involving larger aneurysms, because of the potential for rupture, and when bleeding occurs from rupture of the aneurysm.

Excretory urographic findings are often normal in patients with polyarteritis nodosa. Subtle findings can include increased renal size, ureteral dilatation, perinephric hematoma, and renal infarction. ^[8]

CT scan findings are nonspecific, but they include bowel wall thickening; vascular engorgement; haziness in the mesentery; ascites; ureteral dilatation; renal, hepatic, and splenic infarctions; and perinephric hematoma. ^{[9, 11]}

Selective arteriography is the best modality for evaluating and diagnosing polyarteritis nodosa. The most striking pathognomonic finding is the appearance of aneurysms, usually microaneurysms, which are believed to be caused by rupture of a vessel wall (see the images below).

Microaneurysms are often multiple, usually numbering 10 or more in any single visceral circulation. In most patients, they are 2-5 mm in size. Saccular aneurysms (1-5 mm) of small and medium-sized vessels are typically found. Usually, microaneurysms can be identified in 60-80% of patients, and although they are considered to be pathognomonic for polyarteritis nodosa, these lesions can be encountered in other vasculitides. The aneurysms are caused by segmental occlusion of the arteries with weakening of the arterial wall, which occurs as a result of the necrotizing inflammatory process.

The most common sites of involvement of polyarteritis nodosa are the branching points and bifurcations of arteries. Common sequelae of this condition are intravascular thrombosis, aneurysm rupture, and arterial occlusion with resultant infarctions. In the kidneys, these sequelae can cause ischemic atrophy and hemorrhage. The hemorrhage is usually intrarenal, perinephric, subcapsular, or retroperitoneal.

Other angiographic features of polyarteritis nodosa include tortuous vessels with irregular lumina, segmental luminal narrowing or dilatation, infarctions, vascular irregularity, segmental occlusions, and hypervascularity in regions of PAN. ^{[4, 5, 6]}

Although angiographic findings of microaneurysm, ectasia, and/or occlusive disease suggest the diagnosis of polyarteritis nodosa, these findings may be seen in other vasculitides, including rheumatoid vasculitis, Churg-Strauss syndrome, necrotizing angiitis associated with drug abuse, and systemic lupus erythematosus. Correlation with the clinical evaluation is important.

Conn DL. Polyarteritis. Rheum Dis Clin North Am. 1990 May. 16(2):341-62. [Medline].

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Cengiz N, Demir S, Parmaksiz G, Temiz AK, Noyan A. Polyarteritis nodosa: a case presenting with renal mass. Eur J Pediatr. 2012 Dec. 171(12):1859-60. [Medline].

Ewald EA, Griffin D, McCune WJ. Correlation of angiographic abnormalities with disease manifestations and disease severity in polyarteritis nodosa. J Rheumatol. 1987 Oct. 14(5):952-6. [Medline].

Hekali P, Kajander H, Pajari R, et al. Diagnostic significance of angiographically observed visceral aneurysms with regard to polyarteritis nodosa. Acta Radiol. 1991 Mar. 32(2):143-8. [Medline].

Stanson AW, Friese JL, Johnson CM, et al. Polyarteritis nodosa: spectrum of angiographic findings. Radiographics. 2001 Jan-Feb. 21(1):151-9. [Medline].

McWilliams ET, Khonizy W, Jameel A. Polyarteritis nodosa presenting as acute myocardial infarction in a young man: importance of invasive angiography. Heart. 2013 Aug. 99(16):1219. [Medline].

Jee KN, Ha HK, Lee IJ, et al. Radiologic findings of abdominal polyarteritis nodosa. AJR Am J Roentgenol. 2000 Jun. 174(6):1675-9. [Medline].

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Howard T, Ahmad K, Swanson JA, Misra S. Polyarteritis nodosa. Tech Vasc Interv Radiol. 2014 Dec. 17 (4):247-51. [Medline].

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Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR Consultant Radiologist and Honorary Professor, North Manchester General Hospital Pennine Acute NHS Trust, UK

Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR is a member of the following medical societies: American Association for the Advancement of Science, American Institute of Ultrasound in Medicine, British Medical Association, Royal College of Physicians and Surgeons of the United States, British Society of Interventional Radiology, Royal College of Physicians, Royal College of Radiologists, Royal College of Surgeons of England

Disclosure: Nothing to disclose.

Sumaira Macdonald, MBChB, PhD, FRCP, FRCR, EBIR Chief Medical Officer, Silk Road Medical

Sumaira Macdonald, MBChB, PhD, FRCP, FRCR, EBIR is a member of the following medical societies: British Medical Association, Cardiovascular and Interventional Radiological Society of Europe, British Society of Interventional Radiology, International Society for Vascular Surgery, Royal College of Physicians, Royal College of Radiologists, British Society of Endovascular Therapy, Scottish Radiological Society, Vascular Society of Great Britain and Ireland

Disclosure: Received salary from Silk Road Medical for employment.

Bernard D Coombs, MB, ChB, PhD Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand

Disclosure: Nothing to disclose.

Kyung J Cho, MD, FACR, FSIR William Martel Emeritus Professor of Radiology (Interventional Radiology), Frankel Cardiovascular Center, University of Michigan Health System

Kyung J Cho, MD, FACR, FSIR is a member of the following medical societies: American College of Radiology, American Heart Association, American Medical Association, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America

Disclosure: Nothing to disclose.

Robert L Cirillo, Jr, MD, MBA Assistant Professor of Radiology, Florida State University College of Medicine; Medical Interventional Radiologist, Director/CEO, South Georgia Vascular Institute and South Georgia Laser Vein Center

Robert L Cirillo, Jr, MD, MBA is a member of the following medical societies: American Association for Physician Leadership, Society of Interventional Radiology, Society for Vascular Ultrasound, Cardiovascular and Interventional Radiological Society of Europe

Disclosure: Nothing to disclose.

Fredric A Hoffer, MD, FSIR Affiliate Professor of Radiology, University of Washington School of Medicine; Member, Quality Assurance Review Center

Fredric A Hoffer, MD, FSIR is a member of the following medical societies: Children’s Oncology Group, Radiological Society of North America, Society for Pediatric Radiology, Society of Interventional Radiology

Disclosure: Nothing to disclose.

Imaging in Polyarteritis Nodosa

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