A Successful Infliximab Treatment of a Pediatric Case of Severe Polyarteritis Nodosa With a Cerebral Infarction and a Decreased Adenosine Deaminase 2 Activity

Abstract

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis common in males over 50 years of age that causes various organ symptoms. In recent years, it has become important to distinguish deficiency of adenosine deaminase 2 (DADA2) from childhood-onset PAN. A 13-year-old girl was urgently transferred to our hospital with sudden weakness in her right upper and lower limbs. The National Institutes of Health Stroke Scale (NIHSS) was 8. Plain MRI of the brain indicated high-signal areas in the right caudate nucleus, internal capsule, and left basal ganglia when applying T2-weighted, fluid-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging (DWI); and low signals in the same regions in an apparent diffusion coefficient (ADC) map. It demonstrated inflammatory demyelinating disease of the central nervous system or multiple cerebral infarctions attributable to vasculitis, and it is difficult to differentiate between them based on image findings alone, and cannot be determined without following the clinical course. Hence, we treated with steroid therapy, which is effective for both conditions. Although the paralysis was alleviated, an MRI of the brain reperformed on day 7 revealed expansion of the lesion with contrast enhancement in the feeding area of the left lateral striatal artery, a high signal in DWI, and a low signal in an ADC map. Based on the clinical and radiological findings, we diagnosed a cerebral infarction attributable to vasculitis. Contrast computed tomography (CT) of her chest and abdominal CT angiography revealed that she met the diagnostic criteria for PAN, and adenosine deaminase 2 (AD2) activity level was low. The patient was treated with steroids combined with azathioprine and cyclophosphamide but three weeks after discharge developed a new cerebral infarction in the right basal ganglia. We commenced infliximab; no recurrence of cerebral infarction has been noted. The low AD2 activity may explain the intractable atypical course of this case. Further studies are needed to reveal the role of AD2 in patients with residual enzyme activity and reevaluation of the PAN diagnostic criteria is essential.

Keywords: adenosine deaminase 2; childhood cerebral infarction; deficiency of adenosine deaminase 2; polyarteritis nodosa; tumor necrosis factor-α(tnf-α) inhibitor.