Sclerosing cholangitis in immunocompromised patients associated with Cryptosporidium infection

Abstract

Introduction: Cryptosporidium is a protozoan who parasites the gastrointestinal tract, with a significantly higher incidence in children than in adults. It mainly infects the small intestine and causes acute diarrhea in immunocompetent patients. However, in immunocompromised patients, cryptosporidiosis can be a severe and chronic disease with persistent symptoms, and cause atypical manifestations, such as atypical gastrointestinal disease, biliary tract disease, respiratory tract disease and pancreatitis. Cryptosporidium parvum infection appears to be strongly associated with the development of cholangitis. Nonetheless, the available treatment modalities are limited, and prevention and risk reduction should be the main interventions. Objective: Report the current knowledge landscape and provide information on cholangitis associated with cryptosporidiosis in immunosuppressed patients in the pediatric age, thus contributing to the diagnosis and therapeutic behaviors. Methodology: It was reviewed the main databases: Institute of Health PUBMED, Scientific Electronic Library Online (SciELO) - searching for articles that considered the subject and using and crossing the descriptors: Cholangitis, Immunodeficiency, Cryptosporidiosis, Pediatrics. Articles were searched in Portuguese, English and Spanish, containing texts from 2001 to 2018. Discussion: The suspicion of chronic liver disease arises with the appearance of considerable hepatomegaly and laboratory abnormalities (hepatic transaminases, alkaline phosphatase and gamma GT with increased serum levels) in patients with previous immunodeficiency diagnosis. Several studies have shown that the therapeutic arsenal - antiparasitic agents and macrolide antibiotics - was not effective in eradicating infection and preventing the progression of the disease. Therefore, liver transplantation becomes necessary with the evolution of the disease. However, not even the procedure is capable of improving the survival rates of this group of patients, due to the complications of the procedure, such as absence of immunocompetence, use of medications, or graft rejection. Recurrence can reach a fifth of patients. Conclusion: Sclerosing cholangitis secondary to cryptosporidiosis should be considered in the differential diagnosis of chronic liver disease in children. Diagnosis can be made by associating protozoal infection in the hepatic and biliary tract with particular cholangiographic alterations of the bile duct. Such changes in children are often subtle. Therefore, prospective, controlled and collaborative trials in patients with cryptosporidiosis sclerosing cholangitis are necessary to provide a better understanding of the prevalence, pathogenesis, potential treatment and prognosis.