EOSINOPHILIC CYSTITIS IN A PEDIATRIC PATIENT WITH X-LINKED CHRONIC GRANULOMATOUS DISEASE

Abstract

<h3>Introduction</h3> Pediatric Eosinophilic Cystitis is an uncommon inflammatory disease associated with X-linked Chronic Granulomatous Disease (X-CGD)¹. We present a rare case of a teenage patient with refractory eosinophilic cystitis. <h3>Case Description</h3> Patient is a 14-year-old male with X-CGD, colitis, and complications from chronic steroid use, including bilateral cataracts, osteoporosis, and adrenal insufficiency. His colitis was well-controlled with vedolizumab when he presented for evaluation of new onset dysuria and intermittent scrotal pain that improved with voiding. Infectious workup was unrevealing. CT of abdomen and pelvis demonstrated asymmetric, irregular thickening, nodularity and enhancement of the left bladder wall and bladder base. Pathology from cystoscopy was significant for scattered eosinophils, suspicious for eosinophilic cystitis. He demonstrated mild peripheral eosinophilia (0.63 K/µL). He was started on intravesical dexamethasone 10mg, to minimize complications from systemic steroid use, and oral loratadine 5mg on intravesical treatment days. However, his symptoms did not resolve and intravesical dexamethasone was increased to 25mg then to 50mg. Loratadine was increased to 10mg daily. His bladder ultrasound normalized after 12 doses of intravesical dexamethasone and daily 10mg loratadine. Three weeks after loratadine was weaned to every other day, his urinary symptoms returned. Loratadine was increased to 10mg daily and he remained asymptomatic off steroids. <h3>Discussion</h3> Reported cases of pediatric eosinophilic cystitis are mostly in young children, only requiring a short course of intravesical steroids and oral antihistamine, unlike our patient^2,3. More research is needed to elucidate the history, optimal diagnosis and guide appropriate management of eosinophilic cystitis in pediatric X-CGD patients.