MR cholangiography in the diagnosis of sclerosing cholangitis in Langerhans' cell histiocytosis

Langerhans' cell histiocytosis (LCH) is a disorder of histiocytic proliferation that primarily affects infants. Imaging findings of a rare case of lung and liver involvement in an adult are presented. High-resolution computed tomography (HRCT) of the lungs showed confluent thin-walled cystic air spaces compatible with advanced LCH. Liver CT and MRI revealed unspecific signs of fatty infiltration. Irregular widening of peripheral bile ducts was displayed in breath-hold MR cholangiography. This pattern is considered characteristic for sclerosing cholangitis and should support the diagnosis of LCH in case of concomitant cystic pulmonary disease, even in adult patients.