autoimmune hepatitis and sclerosing cholangitis

Three years ago my husband became very ill with malaise, waisting,leg rash,prutitis,jointpain and fatigue.  He went to a gastrologist who discovered elevated liver levels and ordered a biopsy when all the usual viral heps were neg.  He told us that he believed it was AIH and began prednisone which made my husband feel better immediately. six weeks later his sugars were 800 and he spent a week in the hospital and stayed on insulin until he stopped prednisone 18 months later.  He had no symptoms until may 2004 when he became naseaus and his anylase and lipase was very high. ALP started to increase at this point.  His sugars went up and he went on insulin. He was showing pancreatitis symptoms and started a clear diet.  Ct showed mildly enlarged liver and normal pancreatic contour with increasing periportal.   My poor husband could barely get up the stairs at the end of the day. He soon became jaundiced and had ercp which showed CBD narrowed.  Stent was put in.  ALT,AST.GGTelevated with climbing ALP.  2 weeks later he was still itching and another ercp and a larger stent replaced the first.  After further liver tests showed bad sed. level and ALP still going up, doctor puts on Prednisone.  He feels better right away but we decide to go see Liver Specialist at NYU. We ask for all records.We get no blood tests and just his notes and results of original biopsy.  It states...[.Portal inflammation, fibrosis with bridging, focal stage 3 and ductupenia. Neg. D-Pas. Consider sclerosing cholangitis, Autoimmune cholangiopathy and drug hepatotoxiicity in the differential diagnosis.]  Also my husband has had abnormal lymphnodes showing in renal and abdominal area but we did the lymphoma route with no bad results.  SOOOOOO.....my question is :  Is it strange that the doctor never told us what was on the biopsy except it's AIH.  Is is posssible that he has Sclerosing cholangitis with an ALP of 1500 after the stent.  He mentioned over-lap symdrone but when I asked if he had a billiary disease he said no its from the pancreatitis.  First he said it was acute then the doctor who did ercp said its chronic.  Could it be caused by the cholangitis? His pancreatic biopsies showed no cancer but would they also have shown cholangitis if he had it.  Once he told my husband what you really need is a new liver but when I told him we were seeing a liver transplant doctor because he said he would reveiw the case, he said that is not necessary.  We figure he is the top man and will guide us well. I know my husband is not showing any signs of liver failure now but I am wondering if his last bout of illness had not further damaged his liver.  Sorry to drive you nuts but I want to know if I am right to be questioning some of whats happening.  We see liver specialist in two weeks.  Much gratitude for any insight.  I did demand all reports from the doctors office so we are prepared.  God bless.

[Kevin Pho, MD]

Yes, there is something known as the autoimmune/sclerosing cholangitis overlap syndrome, where some of the blood tests can be suggestive of autoimmune hepatitis, but also have features of sclerosing cholangitis.  

Yes, it is possible to have sclerosing cholangitis with an ALP that high.  Without being more familiar with the case, I cannot make specific recommendations.  But it is possible that the two doctors may have had differing opinions as to the chronic vs acute chlangitis, as well as the option of a liver transplantation.  

Regarding the liver biopsy - it still may be autoimmune hepatitis, but the differential diagnosis (i.e. other causes of the findings), should all be investigated as well.  

Following up with the liver specialist is the appropriate next step.  

Followup with your personal physician is essential.

This answer is not intended as and does not substitute for medical advice - the information presented is for patient education only. Please see your personal physician for further evaluation of your individual case.

Kevin, M.D.
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