Tuesday, April 10, 2012

PBC Familial Clusters Involve Mother-Daughter Pairs

Whether coeliac disease leads to severe liver disease and failure is the subject of ongoing debate. Patients with chronic liver disease have been found to have a higher prevalence of coeliac disease than the general population. A study of 327 patients with 'chronic liver disease' from Sweden found the prevalence of coeliac disease was increased at least 15-fold. Patients with severe liver disease were investigated in a study from Finland. In those considered for liver transplant for gross liver disease, coeliac disease was found in four and on a gluten-free diet a dramatic response ensued in the three compliant patients and in the fourth, a poorly compliant patient, a partial response followed. A related study of 185 Finnish patients who underwent liver transplantation found eight had adult coeliac disease; four to 10 times the expected prevalence. Seven of the eight adult coeliac disease patients were non-compliant long term. The liver biopsies showed a number of pathologies; autoimmune hepatitis (one), primary biliary cirrhosis (two), steatosis, primary biliary cirrhosis (one), primary sclerosing cholangitis (one), congenital liver fibrosis (one), chronic active hepatitis (one), secondary sclerosing cholangitis following cholecystectomy (one).

There are, moreover, numerous reports of a link to primary biliary cirrhosis with evidence of improvement on a gluten-free diet, leading to the recommendation that all patients with primary biliary cirrhosis be screened for coeliac disease.

There is evidence for links between coeliac disease, often silent, and a wide variety of liver diseases particularly a mild silent hepatitis and primary biliary cirrhosis.


And if anyone doubts my worry for Zoe:

...data suggest that first-degree relatives of PBC patients have an increased risk of developing the disease. Most often, these familial clusters involve mother-daughter pairs, which is consistent with the female preponderance of the disease...

I am justified to worry!


1 comment:

  1. HEllo,

    Where do you live? HAve you come in touch with the PBC Foundation in Edinburgh, UK? I am their representative and volunteer in Romania. cristina.enescu@pbcfoundation.org.uk , if you wish get in touch with me please.

    All the best!