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Polyarteritis Nodosa with Miocardial and Kidney Infarcts, Multiple Aneurysm and Hemoperitoneum

Author(s)

Palma Ceppi, Rodrigo; Astudillo Jara, Jaime; Cermenati Bahrs, Tomás; Palavecino Bustos, Tamara; Palavecino Rubilar, Patricio; Ramos Gómez, Cristóbal; Herquiñigo Reckmann, David

Hospital Clínico Universidad de Chile, Universidad de Chile; Santos Dumont 999, 8380456 Santiago; Email:palmarodrigo@gmail.com

Patient

female, 56 year(s)

Clinical History

A 56-year-old female presented with sudden onset of chest pain irradiating to the upper extremities.
Medical antecedent: Hypertension. Upper extremity paresthesias.

Imaging Findings

The patient underwent CT angiography of the chest; this showed a hypodense area located at the level of the anterolateral wall (Fig. 1a-d). Due to the intensity of the symptoms, and tomographic images, the patient was further examined with coronariography which revealed occlusion of the first marginal branch of the left circumflex artery (Fig. 2).
On the 5th day of treatment, the patient has abdominal pain, hemodynamic impairment and decreased hematocrit. Abdominal contrast-enhanced CT shows a demarcated, wedge-shaped hypodense area on the right kidney (Fig. 3a, b). CT images revealed free peritoneal fluid with higher attenuation in the left para-colic gutter and active bleeding with extravasation of contrast at the omentum (Fig. 3c).
VRT and MIP reconstructions showed multiple splenic, gastric, mesenteric aneurysms
(Fig. 4a-c) and renal (not show). Selective arteriography of the celiac trunk and mesenteric artery showed splenic, mesenteric and gastroomental aneurysm (Fig 4d, e).
The patient was treated successfully with embolization.

Discussion

Polyarteritis nodosa (PAN) is a necrotizing vasculitis characterized by the presence of inflammatory reactions of blood vessels of medium or small caliber that lead to necrosis and destruction of the vessel walls [1]. The incidence is unknown, but this uncommon vasculitis affects people in their fourth and fifth decades of life. It is a disease that primarily affects males [2].

The primary abnormal changes of the involved vessel consist of fibrinoid or hyaline necrosis of the media with simultaneous or subsequent involvement of the intima and adventitia. Secondary changes include aneurysm formation, haemorrhage, and thrombosis [2].

Clinical manifestations can be varied. Patients usually present with vague signs and symptoms such as fever, weight loss, weakness, malaise, headache, and mialgia. Specific complaints related to vascular involvement within a particular organ system include renal failure or hypertension in renal involvement; peripheral neuropathy in peripheral nervous system involvement; congestive heart failure, myocardial infarction, or pericarditis in cardiac involvement; and rash, purpura, or nodules in skin involvement [3].

The kidney is most commonly involved (80–100%), followed by the heart (70%), gastrointestinal tract, peripheral nerves, and skin (50%), skeletal muscles and mesentery (30%) and the central nervous system in 10% [1, 2]. Coronary arteries can occasionally be affected in patients with systemic necrotizing vasculitides, especially in the PAN [4].

The diagnostic criteria of PAN have been classified by the American College of Rheumatology. Three of the 10 criteria must be present for the diagnosis of PAN. A positive angiogram with typical findings is one of the 10 criteria [1]. Antineutrophilic cytoplasmic autoantibodies are antibodies directed against certain proteins in the cytoplasm of neutrophils and are often present in the serum of patients with polyarteritis nodosa and correlate with their disease activity. However, this test is not specific for cases of PAN [2]. Angiographic findings, including aneurysms, ectasia, or occlusive disease, are present in about 40%–90% of patients at the time clinical symptoms appear [1].

Findings of multiple, small-sized aneurysms on angiography are pathognomonic for the disease. The aneuryms are usually multiple (most often 10 or more in any one visceral circulation) and 2–5 mm, commonly affecting the branch points of arteries [2]. Consequence thereof can also find multiple small renal infarcts, liver infarction, interstitial hepatitis, active bleeding and hemoperitoneum [1-3]. The gastrointestinal lesions may take the form of ulceration, perforation, hemorrhage, or infarct [2].

Final Diagnosis

Differential Diagnosis List

References

[1]

Stanson AW, Friese JL, Johnson CM, McKusick MA, Breen JF, Sabater EA, Andrews JC. (2001) Polyarteritis nodosa: spectrum of angiographic findings.. Radiographics 21(1):151-9
[2]

Jee KN, Ha HK, Lee IJ, Kim JK, Sung KB, Cho KS, Kim PN, Lee MG, Lim HK, Choi CS, Auh YH. (2000) Radiologic findings of abdominal polyarteritis nodosa.. AJR Am J Roentgenol 174(6):1675-9
[3]

Ha HK, Lee SH, Rha SE, Kim JH, Byun JY, Lim HK, Chung JW, Kim JG, Kim PN, Lee MG, Auh YH. (2000) Radiologic features of vasculitis involving the gastrointestinal tract. Radiographics 20(3):779-94
[4]

Mavrogeni S, Manoussakis MN, Karagiorga TC, Douskou M, Panagiotakos D, Bournia V, Cokkinos DV, Moutsopoulos HM. (2009) Detection of coronary artery lesions and myocardial necrosis by magnetic resonance in systemic necrotizing vasculitides. Arthritis Rheum. 15;61(8):1121-9

Citation

Palma Ceppi, Rodrigo; Astudillo Jara, Jaime; Cermenati Bahrs, Tomás; Palavecino Bustos, Tamara; Palavecino Rubilar, Patricio; Ramos Gómez, Cristóbal; Herquiñigo Reckmann, David (2016, Nov 17).

Polyarteritis Nodosa with Miocardial and Kidney Infarcts, Multiple Aneurysm and Hemoperitoneum, {Online}.

URL: http://www.eurorad.org/case.php?id=14199

DOI: 10.1594/EURORAD/CASE.14199

ISSN: 1563-4086

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This work is licensed under the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

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Published 17.11.2016
DOI 10.1594/EURORAD/CASE.14199
Section Cardiovascular
Case Type Clinical Cases
Views 941
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Figure 1
Acute myocardial infarction

a

Centro de Imagenología Hospital Clínico Universidad de Chile, Santiago, Chile.

b

Centro de Imagenología Hospital Clínico Universidad de Chile, Santiago, Chile.

c

Centro de Imagenología Hospital Clínico Universidad de Chile, Santiago, Chile.

d

Centro de Imagenología Hospital Clínico Universidad de Chile, Santiago, Chile.

Vertical long axis, short axis and four chamber views show hypodense myocardium in the antero-lateral wall an papilary muscle.

Area of Interest: Cardiovascular system; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Ischaemia / Infarction;

Vertical long axis, short axis and four chamber views show hypodense myocardium in the antero-lateral wall an papilary muscle.

Area of Interest: Cardiovascular system; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Ischaemia / Infarction;

Vertical long axis, short axis and four chamber views show hypodense myocardium in the antero-lateral wall an papilary muscle.

Area of Interest: Cardiovascular system; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Ischaemia / Infarction;

Vertical long axis, short axis and four chamber views show hypodense myocardium in the antero-lateral wall an papilary muscle.

Area of Interest: Cardiovascular system; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Ischaemia / Infarction;
Figure 2
First marginal branch occluded of LCX

Coronarography show the first marginal branch of the left circumflex artery (LCX) occluded.

Area of Interest: Cardiovascular system; Imaging Technique: Fluoroscopy; Procedure: Angioscopy; Special Focus: Ischaemia / Infarction;
Figure 3
Kidney infarction and haemoperitoneum

a

Centro de Imagenología Hospital Clinico Universidad de Chile, Santiago, Chile.

b

Centro de Imagenología Hospital Clinico Universidad de Chile, Santiago, Chile.

c

Centro de Imagenología Hospital Clinico Universidad de Chile, Santiago, Chile.

Axial contrast-enhanced computed tomography showing a wedge-shaped and sharply demarcated hypodense area on the right kidney which was oriented towards the hilum.

Area of Interest: Kidney; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Ischaemia / Infarction;

Coronal contrast-enhanced computed tomography image after IV contrast administration confirming the presence of this wedge-shaped area on the right kidney also show hemoperitoneum in the left para-colic gutter.

Area of Interest: Kidney; Imaging Technique: CT; Procedure: Contrast agent-intravenous; Special Focus: Haemorrhage; Ischaemia / Infarction;

CT images before injection of contrast revealed free peritoneal fluid with higher attenuation in the left para-colic gutter. Post contrast images show active bleeding with extravasation of contrast in arterial, portal...

Area of Interest: Abdomen; Imaging Technique: CT-Angiography; Procedure: Contrast agent-intravenous; Special Focus: Haemorrhage;
Figure 4
Multiple Aneurysms

a

Centro de Imagenología. Hospital Clínico Universidad de Chile, Santiago, Chile.

b

Centro de Imagenología. Hospital Clínico Universidad de Chile, Santiago, Chile.

c

Centro de Imagenología. Hospital Clínico Universidad de Chile, Santiago, Chile.

d

Centro de Imagenología. Hospital Clínico Universidad de Chile, Santiago, Chile.

e

Centro de Imagenología. Hospital Clínico Universidad de Chile, Santiago, Chile.

VRT image with multiple aneurysms of the splenic artery.

Area of Interest: Arteries / Aorta; Imaging Technique: CT-Angiography; Procedure: Contrast agent-intravenous; Special Focus: Aneurysms;

MIP image with multiple aneurysms of the splenic artery.

Area of Interest: Arteries / Aorta; Imaging Technique: CT-Angiography; Procedure: Contrast agent-intravenous; Special Focus: Aneurysms;

MIP image with multiple aneurysms of the splenic and gastroomental artery.

Area of Interest: Arteries / Aorta; Imaging Technique: CT-Angiography; Procedure: Contrast agent-intravenous; Special Focus: Aneurysms;

Catheter arteriography image with multiple aneurysms of the splenic artery.

Area of Interest: Arteries / Aorta; Imaging Technique: Catheter arteriography; Procedure: Angioscopy; Special Focus: Aneurysms;

Catheter arteriography image with multiple aneurysms of the mesenteric artery.

Area of Interest: Arteries / Aorta; Imaging Technique: Catheter arteriography; Procedure: Angioscopy; Special Focus: Aneurysms;

Figure 1

Acute myocardial infarction

Figure 1a

Vertical long axis, short axis and four chamber views show hypodense myocardium in the antero-lateral wall an papilary muscle.

Figure 1b

Vertical long axis, short axis and four chamber views show hypodense myocardium in the antero-lateral wall an papilary muscle.

Figure 1c

Vertical long axis, short axis and four chamber views show hypodense myocardium in the antero-lateral wall an papilary muscle.

Figure 1d

Vertical long axis, short axis and four chamber views show hypodense myocardium in the antero-lateral wall an papilary muscle.

Figure 2

First marginal branch occluded of LCX

Coronarography show the first marginal branch of the left circumflex artery (LCX) occluded.

Figure 3

Kidney infarction and haemoperitoneum

Figure 3a

Axial contrast-enhanced computed tomography showing a wedge-shaped and sharply demarcated hypodense area on the right kidney which was oriented towards the hilum.

Figure 3b

Coronal contrast-enhanced computed tomography image after IV contrast administration confirming the presence of this wedge-shaped area on the right kidney also show hemoperitoneum in the left para-colic gutter.

Figure 3c

CT images before injection of contrast revealed free peritoneal fluid with higher attenuation in the left para-colic gutter. Post contrast images show active bleeding with extravasation of contrast in arterial, portal and late venous phases.

Figure 4

Multiple Aneurysms

Figure 4a