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 Table of Contents  
Year : 2018  |  Volume : 13  |  Issue : 3  |  Page : 204-206

Childhood polyarteritis nodosa presenting with posterior reversible encephalopathy syndrome

1 Division of Pediatric Rheumatology, Sri Ramachandra University, Chennai, Tamil Nadu, India
2 Department of Pediatrics, Sri Ramachandra University, Chennai, Tamil Nadu, India

Date of Web Publication21-Aug-2018

Correspondence Address:
Dr. Mahesh Janarthanan
Division of Pediatric Rheumatology, Sri Ramachandra University, Porur, Chennai - 600 116, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_38_18

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Keywords: Childhood polyarteritis nodosa, posterior reversible encephalopathy syndrome, pyrexia of unknown origin

How to cite this article:
Janarthanan M, Geminiganesan S, Kharlukhi J, James S. Childhood polyarteritis nodosa presenting with posterior reversible encephalopathy syndrome. Indian J Rheumatol 2018;13:204-6

How to cite this URL:
Janarthanan M, Geminiganesan S, Kharlukhi J, James S. Childhood polyarteritis nodosa presenting with posterior reversible encephalopathy syndrome. Indian J Rheumatol [serial online] 2018 [cited 2022 Nov 28];13:204-6. Available from:

A 9-year-old developmentally normal male child born from 2nd degree consanguineous parents was admitted with a history of low-grade fever for 45 days and status epilepticus. Before this admission, he had been evaluated as an outpatient for fever. He had been worked up for infective causes such as tuberculosis, typhoid, malaria, and  Brucellosis More Details. The results of these investigations were negative. Since he had been otherwise well, he had been managed as an outpatient with antipyretics. He had been admitted to the hospital in view of refractory seizures. He was ventilated and started on anticonvulsants to achieve seizure control. Persistent hypertension above the 99th centile for his age and height was observed. Fundus examination showed Grade 1 hypertensive retinopathy changes. He had no rashes, generalized lymphadenopathy, or hepatosplenomegaly. He was started on empirical antibiotics and antiviral agents initially. These were stopped when results of blood and cerebrospinal fluid bacterial cultures and viral panel for qualitative polymerase chain reaction were negative. He required four antihypertensives to control blood pressure. Although his seizures stopped and his Glasgow Coma Scale improved, he continued to have persistent low-grade fever spikes and hypertension.

Initial investigations showed white cell counts of 11,900/cmm (4000–11,000 cells/cmm), hemoglobin of 11 mg/dL (12–15 g/dl), platelets of 760,000/cmm (150,000–450,000/cmm, elevated C reactive protein 2.4 mg/dL (<0.6 mg/dl), and erythrocyte sedimentation rate of 34 mm/h (4–10 mm/h) with normal renal function, liver function, and routine urine examination. Other investigations for infections including hepatitis B and blood tests for autoimmune disorders including antinuclear antibodies and antineutrophil cytoplasmic antibodies were negative. An ultrasound abdomen and Doppler of renal vessels were also reported normal. A magnetic resonance imaging (MRI) of the brain was done to investigate the underlying cause of seizures, and this showed vasogenic edema of the occipital and parietal lobes suggestive of posterior reversible encephalopathy syndrome [Figure 1].
Figure 1: Magnetic resonance imaging brain showing edema of occipital and parietal lobes suggestive of posterior reversible encephalopathy syndrome

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In view of persistent low-grade fevers and hypertension, a computed tomography angiogram was done and this revealed multiple renal infarcts, bilateral renal artery stenosis, alternate constriction and dilation with classical “beads on string appearance” of hepatic, superior, inferior, mesenteric, and bilateral internal and external iliac arteries suggestive of polyarteritis nodosa (PAN) [Figure 2].
Figure 2: Computed tomography angiogram abdomen showing bilateral renal artery stenosis, alternate constriction, and dilatation of branches of the celiac trunk – “beads on string appearance”

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The patient was treated initially with oral and parenteral antihypertensive medication including labetalol infusion, followed by oral antihypertensives and anticonvulsants. He also received steroids and 6 doses of IV cyclophosphamide at 750 mg/m 2. This was followed by weekly oral methotrexate (15 mg/m 2) and folic acid. He responded well to treatment.

Two years after the diagnosis, the child is well on antihypertensive medication and methotrexate. He has been referred to vascular surgeons for surgical intervention of renal artery stenosis.

  Discussion Top

Posterior reversible encephalopathy syndrome is considered to be a neurotoxic state usually associated with characteristic MRI brain findings. The parietal and occipital lobes are the most commonly involved. Clinical features include headache, nausea, altered sensorium, seizures, and coma. Although the cause is not clearly understood, hypertension associated with failed autoregulation and hyperperfusion are considered possible mechanisms.[1]

On the other hand, childhood PAN may present as fever, myalgia, arthritis, abdominal pain, livedo reticularis, digital gangrene, subcutaneous nodules, abdominal pain, renal manifestations, and rarely neurological manifestations. Classification criteria for childhood PAN (2008) require the presence of histological evidence of necrotizing vasculitis of medium- or small-sized arteries/angiographic abnormalities – as mandatory, plus one of the following: (1) skin involvement, (2) myalgia, (3) systemic hypertension, (4) peripheral neuropathy, and (5) renal involvement.[2]

The patient presented with seizures and no cutaneous or systemic findings except fever. Except for a platelet response, the other inflammatory markers and white cell count were not grossly elevated as would be expected normally in a case of vasculitis. Diagnosis was made on the basis of hypertension and extensive angiographic abnormalities. Although hypertension is one of the criteria to diagnose polyarteritis nodosa, posterior reversible encephalopathy syndrome as initial presenting manifestation is rare.[3],[4] In a large single-center retrospective study of 69 children seen over 32 years with PAN, only 7 (10%) had the central nervous system involvement with mononeuritis multiplex, peripheral neuropathy, meningitis, stroke, or cranial nerve palsy.[5] In a case series and systematic review of 108 children with posterior reversible encephalopathy syndrome, underlying kidney diseases and malignancies were common causes of this condition.[6] We considered adenosine deaminase 2 deficiency as a cause of PAN in this child but were unable to request genetic studies due to financial constraints in the family and as the child was responding well to therapy.[7]

Due to rarity of this type of presentation, a high index of suspicion may be necessary to make a diagnosis when dealing with a child with fever and posterior reversible encephalopathy syndrome. An angiogram may be the only useful investigation in this situation. Prompt recognition and appropriate therapy can possibly reduce mortality and long-term neurological sequelae.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Bartynski WS. Posterior reversible encephalopathy syndrome, part 1: Fundamental imaging and clinical features. AJNR Am J Neuroradiol 2008;29:1036-42.  Back to cited text no. 1
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for henoch-schönlein purpura, childhood polyarteritis nodosa, childhood wegener granulomatosis and childhood takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 2010;69:798-806.  Back to cited text no. 2
Guirola R, Hunter JV, Perez M, Muscal E. Childhood polyarteritis nodosa presenting with central nervous system manifestations and the posterior reversible encephalopathy syndrome. J Child Neurol 2014;29:103-7.  Back to cited text no. 3
Navinan MR, Subasinghe CJ, Kandeepan T, Kulatunga A. Polyarteritis nodosa complicated by posterior reversible encephalopathy syndrome: A case report. BMC Res Notes 2014;7:89.  Back to cited text no. 4
Eleftheriou D, Dillon MJ, Tullus K, Marks SD, Pilkington CA, Roebuck DJ, et al. Systemic polyarteritis nodosa in the young: A single-center experience over thirty-two years. Arthritis Rheum 2013;65:2476-85.  Back to cited text no. 5
Chen TH, Lin WC, Tseng YH, Tseng CM, Chang TT, Lin TJ, et al. Posterior reversible encephalopathy syndrome in children: Case series and systematic review. J Child Neurol 2013;28:1378-86.  Back to cited text no. 6
Caorsi R, Penco F, Grossi A, Insalaco A, Omenetti A, Alessio M, et al. ADA2 deficiency (DADA2) as an unrecognised cause of early onset polyarteritis nodosa and stroke: A multicentre national study. Ann Rheum Dis 2017;76:1648-56.  Back to cited text no. 7


  [Figure 1], [Figure 2]


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