Primary sclerosing cholangitis in children and adolescents: a clinicopathologic study with a proposal of criteria for early diagnosis

Abstract

INTRODUCTION: Primary sclerosing cholangitis (PSC) has been increasingly diagnosed among children and adolescents due to better recognition of clinical, imaging and pathological features. Thus more patients are diagnosed at a younger age due to imaging and sensitivity optimization. OBJECTIVE: Early liver histopathological (LH) changes are not well described and PSC is not commonly recognized before typical bile duct changes occur on cholangiography (CG). Currently, CG is considered gold standard for adults but nothing is known for early diagnosis in the pediatric age group (0- 20 years old). METHODS: We reviewed clinical history, LH and CG from 47 children and adolescents with PSC (35 males, mean age 13 years old). Forty-three out of 47 patients had been through LH examination from whom 33 had also undergone CG. A clinicopathological correlation was performed. RESULTS: LH showed active neutrophilic cholangitis in 19 patients, moderate neutrophilic pericholangitis in nine, dystrophic changes in the bile duct in eight, and concentric periductal fibrosis in 24 patients. Abnormal CG was found in 24 out of 33 patients and nine had normal results. Eleven out of these 24 patients had abnormal histology before abnormal CG and four patients had abnormal CG before histology. Data of two out of 24 patients were insufficient for correlation and 11 out of 24 had both abnormal liver histology and abnormal imaging findings. CONCLUSION: Our study emphasizes that even when CG is normal, PSC should be exclusively diagnosed by liver biopsy, hence cholangiography being unnecessary. Chronic portal inflammation, neutrophilic pericholangitis, periductal sclerosis and "onion skinning" are characteristic histopathological findings. Neutrophilic pericholangitis may be subtle and easily overlooked in early disease, leading to strong suspicion of PSC.