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  Sclerosing Cholangitis



X-Linked Hyper IgM


X-linked hyper IgM (CD40L deficiency) - Patients are at risk for developing sclerosing cholangitis due to Cryptosporidium infection.  This serious complication may necessitate a liver transplantation and can be a significant cause of death in older patients with this disease.  



AR Hyper IgM Syndrome


AR Hyper IgM syndrome (CD40 deficiency) -Similar to patients with CD40L deficiency, Cryptosporidium infections and sclerosing cholangitis have been reported in patients with CD40 deficiency.  



MHC Class II Deficiency


MHC Class II deficiency - Patients develop sclerosing cholangitis secondary to Cryptosporidium at high frequency (>50% of patients).  It represents a major factor in prognosis of the disease.




CVID - Sclerosing cholangitis has been reported in patients with this disease.  However, it is not a typical complication. 


Other Serious T Cell Immunodeficiencies


Other serious T cell immunodeficiencies - Sclerosing cholangitis has been reported in other combined immunodeficiencies such as Wiskott-Aldrich syndrome and SCID.  Defective T cell immunity increases susceptibility to Cryptosporidium and thus sclerosing cholangitis. 

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