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

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].

Differential Diagnosis List

Final Diagnosis

Polyarteritis Nodosa

URL:	https://eurorad.org/case/14199
DOI:	10.1594/EURORAD/CASE.14199
ISSN:	1563-4086