PAN presenting with posterior reversible encephalopathy synd
The present article has been published in the Indian Journal of Rheumatology.

A 9-year-old male child born from 2nd degree consanguineous parents was admitted with a history of low-grade fever for 45 days and status epilepticus. 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.

Brain MRI revealed vasogenic edema of the occipital and parietal lobes suggestive of posterior reversible encephalopathy syndrome. 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).

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.

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