Eosinophilic Cystitis in a 4-Year-Old Boy: Successful Long-Term Treatment With Cyclosporin A

Abstract

ABSTRACT. A 4-year-old Jewish boy presented with dysuria, urinary dribbling, increased urinary frequency, and new onset of diurnal enuresis. An infiltrating solid mass involving the entire bladder wall was found. Biopsy revealed "tumor-forming" eosinophilic cystitis, a rare bladder lesion of unclear cause. Antitoxocariasis treatment was unsuccessful. High-dose corticosteroids failed. The child's clinical condition and bladder sonographic findings continued to deteriorate. Treatment with cyclosporin A was given for 8 months, with a complete clinical, radiologic, and histopathologic cure and no side effects. Two years of follow-up showed a complete recovery. Pediatrics 2001;108(6). URL: http://www. pediatrics.org/cgi/content/full/108/6/e113; cystitis, eosinophilic, cyclosporin A.E osinophilic cystitis is a rare disorder of the urinary bladder, characterized by extensive local eosinophilic infiltration of all layers of the bladder wall, probably induced by a regulatory disorder of the immune system. Many possible causes have been postulated; nevertheless, the exact mechanism of the disease still needs to be clarified. This uncommon entity was first reported almost 40 years ago 1,2 and can be encountered in any age group, although it seems to be more common in adults. Eosinophilic cystitis has a broad spectrum of clinical and pathologic manifestations, ranging from mild inflammatory cystitis to severe chronic or recurrent relentless inflammation. [3][4][5][6][7][8] This disorder eventually can progress to a complete fibrosis of the urinary bladder with secondary involvement of the rest of the urinary tract, resulting in obstructive nephropathy with variable degrees of renal insufficiency. [3][4][5][6][7][8][9] We report a child who had severe progressive eosinophilic cystitis and did not respond to high-dose corticosteroid therapy and specific therapy for toxocariasis. Repeated bladder sonography suggested progressive reduction of the urinary bladder lumen with extensive fibrosis of the bladder wall. Longterm treatment with cyclosporin A resulted in clinical recovery with complete disappearance of the bladder eosinophilic infiltration. CASE REPORTA 4-year-old Jewish boy, who was born to a healthy mother of Turkish origin and a Moroccan father, had received a diagnosis of celiac disease. His 6-year-old sister was healthy. The patient was a term infant, appropriate for gestational age, and born after an uneventful pregnancy and delivery. Growth and development were normal. At age 1.5 years, he underwent a left orchidopexy for an undescended testis.He presented with a 2.5-month history of dysuria, urinary frequency and urgency, dribbling, and diurnal enuresis. Body temperature was normal. No abnormalities were detected in the physical examination. The laboratory results showed a white cell count of 12 000/ L with 32% neutrophils, 60% lymphocytes, 6% monocytes, and 2% eosinophils.Hemoglobin was 11.8 g/dL, and the red blood cell count was 4.54 ϫ 10 6 / L, with normal indices. Platelet count, erythrocyte sedimentation rat...

Multimodality imaging of renal inflammatory lesions

“…It clinically presents with hematuria, frequency and irritative symptoms. The mean age at diagnosis is 41.6 years with an equal sex distribution [21] . On imaging, there is diffuse bladder wall thickening which is often more than 10 mm with characteristic preservation of the mucosal line and enhancement on delayed images ( Figure 20) [22,23] .…”

How should eosinophilic cystitis be treated in patients with chronic granulomatous disease?

“…EC is a rare pediatric inflammatory disorder of the bladder characterized by extensive eosinophilic infiltration of all Table 1 Clinical, diagnostic, and therapeutic findings of eosinophilic cystitis in patients with chronic granulomatus disease (CGD) Kontras et al (1971) [13] Bauer et al (1991) [14] R e d m a n e t a l . WBC white blood cells, E eosinophils, N neutrophils layers of the bladder wall [4]. EC seems to be related to dysregulation of the immune system, as suggested by reports of EC occurring in patients with a history of allergies [5][6][7][8] or subsequent to drug administration (penicillin, mitomycin, clometacin, cyclophosphamide, coumadin, tranilast, and thiotepa) [5,6].…”

“…EC seems to be related to dysregulation of the immune system, as suggested by reports of EC occurring in patients with a history of allergies [5][6][7][8] or subsequent to drug administration (penicillin, mitomycin, clometacin, cyclophosphamide, coumadin, tranilast, and thiotepa) [5,6]. The detection of transmural eosinophilic infiltration should also prompt suspicion of a differential diagnosis, including parasitosis, tumors, tuberculous cystitis, and bladder instrumentation [4,9]. Is not clear whether the EC in CGD patients depends on the natural course of the immunodeficiency related to hyperinflammation or if it develops in response to other triggers, such as drugs chronically used in these patients.…”

“…Is not clear whether the EC in CGD patients depends on the natural course of the immunodeficiency related to hyperinflammation or if it develops in response to other triggers, such as drugs chronically used in these patients. EC usually presents with symptoms of irritative nature, such as dysuria, hematuria, urgency, frequency, and suprapubic tenderness [4,5,7,9]. Patients may also present with an obstructive bladder mass resembling malignancy and causing hydroureteronephrosis [5,10].…”

An unusual cause of terminal hematuria in a child: Eosinophilic cystitis

“…1 Gross hematuria in children is rare and is often associated with benign conditions, involving an infection or a urolithiasis. 2,3 Eosinophilic cystitis (EC) is a rare inflammatory disease of the bladder with unknown etiology, characterized by numerous eosinophilic infiltrations of the bladder. 4 Patients with eosinophilic cystitis show irritative urination symptoms frequently, with a possible need for urgency, alongside dysuria, gross hematuria, suprapubic pain and painful urination.…”