ABSTRACT
OBJECTIVES: We studied clinicopathologic features of congenital hepatic fibrosis (CHF) that could aid the diagnosis of this relatively rare condition during adulthood. METHODS: Five consecutive adult CHF cases were identified in a single institution. RESULTS: Clinical manifestations of CHF varied from asymptomatic to requiring liver transplantation. Three of five cases had other disease associations, including Joubert syndrome, Caroli disease, polycystic kidney disease, and congenital anomaly of hepatic vasculature. No unique common radiologic findings were found. Histologically, all cases showed characteristic abnormal interlobular bile ducts embedded in fibrotic portal stroma, with varying degrees of liver fibrosis. CONCLUSIONS: While other disease associations and characteristic liver histomorphology are helpful clues to suspect the diagnosis of CHF in adult patients, other differential diagnoses should be excluded clinically and radiologically. This study highlights the importance of a multidisciplinary diagnostic approach by pathologists, radiologists, and hepatologists for the accurate diagnosis of CHF during adulthood.
Subject(s)
Genetic Diseases, Inborn/diagnosis , Liver Cirrhosis/diagnosis , Liver Transplantation , Adult , Aged , Female , Genetic Diseases, Inborn/pathology , Humans , Liver/pathology , Liver Cirrhosis/pathology , Male , Middle AgedSubject(s)
Dermatologic Agents/adverse effects , Liver Cirrhosis/complications , Liver/drug effects , Psoriasis/drug therapy , Ustekinumab/adverse effects , Aged , Dermatologic Agents/administration & dosage , Female , Humans , Liver/physiopathology , Liver Cirrhosis/blood , Liver Cirrhosis/physiopathology , Liver Function Tests , Male , Middle Aged , Psoriasis/blood , Psoriasis/complications , Retrospective Studies , Treatment Outcome , Ustekinumab/administration & dosageABSTRACT
The liver biopsy has been regarded as the reference standard method of monitoring hepatic fibrosis in psoriasis patients treated with methotrexate. It has also been subject to concerns over sampling error, internal and external variability, and potential for morbidity and mortality. During the past two decades, two imaging techniques, magnetic resonance elastography and transient elastography, have been developed and approved by the US Food and Drug Administration (FDA) for the assessment of hepatic fibrosis. Although high-quality, psoriasis-specific data are lacking, both methods have been shown to have outstanding efficacy in the detection of hepatic fibrosis, particularly the more advanced stages which may warrant the choice of a therapeutic alternative to methotrexate. Dermatologists should be aware of the availability of these tests and understand their limitations. Prospective studies in psoriasis and methotrexate management using these techniques are needed.
