Guest guest Posted January 28, 2009 Report Share Posted January 28, 2009 That's interesting, Dave. Drs have never mentioned whether my PSC is small or large duct, but I believe I had/have the small. It might account for the fact that I've skipped many of the worst symptoms, especially the frequent painful cholangitis attacks. Are they able to tell if one has small or large? Don Please be a blood/organ donor Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 28, 2009 Report Share Posted January 28, 2009 Hi Don; As Debbie has already mentioned, it is pretty challenging to diagnose small-duct PSC. Apparently the diagnosis is made based on a normal- looking ERCP (where only medium and large ducts can be visualized), but where the patient has elevated liver enzymes consistent with bile- duct involvement/cholestasis (that is, elevated alkaline phophatase and gamma-glutamyltranspeptidase), and where no other cause of small- duct injury can be identified. They would have to rule out PBC (which mostly affects women), and this is done with an antibody test. Most PBCers (over 95%) have anti-mitochondrial antibodies. But then, some PBCers (~5 %) are anti-mitochondrial antibody negative. I believe that anti-mitochondrial antibody negative PBC is sometimes called autoimmune cholangitis. Liver biopsy might help distinguish between these three (PSC, PBC and autoimmune cholangitis), and other autoimmune liver diseases such as autoimmune hepatitis. Finally, they would also have to rule out drug-induced small-duct loss. There's a whole series of " ductopenias " (mostly affecting the small-ducts) that can result from adverse reactions to various drugs. Best regards, Dave (father of (23); PSC 07/03; UC 08/03) > That's interesting, Dave. Drs have never mentioned whether my PSC is small or large duct, but I believe I had/have the small. It might account for the fact that I've skipped many of the worst symptoms, especially the frequent painful cholangitis attacks. Are they able to tell if one has small or large? Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 29, 2009 Report Share Posted January 29, 2009 > > Hi Don; > > As Debbie has already mentioned, it is pretty challenging to diagnose > small-duct PSC. Hi again Dave, I've had few ERCP's,compared to most of the group. The one that made a big impression on me was when I had my one big episode of cholangitis, or whatever it was. Chills and fever for 3 days. I refused to go into the ER, as is my Norwegian MO, until I was really a mess, and they told me at work I was turning yellow. My own DR (not my present GI)was not around and his partner decided to remove my gall bladder. They did an ERCP first, and that's the only X-ray of my liver I've seen. It showed all the ducts with the classic beaded appearance. Were these small ducts or medium or large? Is the large duct just the main bile duct, outside the liver? This discussion is fascinating, but perhaps only idle curiousity on my part. Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 29, 2009 Report Share Posted January 29, 2009 I am by no means an expert, But from what I was told, at least for me, the MRCP shows both the small and large and my small ducts had the beaded appearance but the large did not; however, my last MRCP showed that I was begining to develop narrowing of the large ducts. I have no external duct involvement as of my last MRCP almost 2 years ago. When I look at my MRCP's you can clearly see the differences between them and and I could even see where some of the small ducts were no longer visable on the last one compared to some of the previous ones. I do agree this is a fasinating discussion Dawn > > > Were these small ducts or medium or large? Is the large duct just the > main bile duct, outside the liver? > > This discussion is fascinating, but perhaps only idle curiousity on my > part. > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 29, 2009 Report Share Posted January 29, 2009 I am by no means an expert, But from what I was told, at least for me, the MRCP shows both the small and large and my small ducts had the beaded appearance but the large did not; however, my last MRCP showed that I was begining to develop narrowing of the large ducts. I have no external duct involvement as of my last MRCP almost 2 years ago. When I look at my MRCP's you can clearly see the differences between them and and I could even see where some of the small ducts were no longer visable on the last one compared to some of the previous ones. I do agree this is a fasinating discussion Dawn > > > Were these small ducts or medium or large? Is the large duct just the > main bile duct, outside the liver? > > This discussion is fascinating, but perhaps only idle curiousity on my > part. > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 29, 2009 Report Share Posted January 29, 2009 > > > I am by no means an expert, But from what I was told, at least for me, > the MRCP shows both the small and large and my small ducts had the > beaded appearance but the large did not; however, my last MRCP showed > that I was begining to develop narrowing of the large ducts. I have no > external duct involvement as of my last MRCP almost 2 years ago. When > I look at my MRCP's you can clearly see the differences between them > and and I could even see where some of the small ducts were no longer > visable on the last one compared to some of the previous ones. > > I do agree this is a fasinating discussion > > Dawn > > Dawn, I just saw my new hepatologist last week, and he wanted to do an ERCP just to " get a baseline look " at my disease. I had the ERCP which diagnosed my disease in Oct of 2007, after an MRCP which did not show anything. I've had another, subsequent MRCP which also showed nothing, and my previous hepatologist indicated to me that he was fine with using the MRCP to monitor me, even though they are not seeing the damage that they know is there in the small ducts. I asked him, " What if year after year, the MRCP continues to show nothing? " He just smiled and said, " That will be a good thing. " My new hepatologist said that you can't see the small ducts on the MRCP & that's why he wants to do another ERCP. Maybe I misunderstood him, and maybe he just meant you can't see them as well. But it was interesting to me that your MRCP showed beading in your small ducts. I don't want another ERCP, unless it's really necessary. I'm not having any symptoms or problems right now. For now, my new hepatologist has agreed to put the ERCP off if I get images of my first ERCP to him. But I don't know if he is going to be willing to rely on MRCPs like my previous hepatologist was. Debbie Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 29, 2009 Report Share Posted January 29, 2009 > > > I am by no means an expert, But from what I was told, at least for me, > the MRCP shows both the small and large and my small ducts had the > beaded appearance but the large did not; however, my last MRCP showed > that I was begining to develop narrowing of the large ducts. I have no > external duct involvement as of my last MRCP almost 2 years ago. When > I look at my MRCP's you can clearly see the differences between them > and and I could even see where some of the small ducts were no longer > visable on the last one compared to some of the previous ones. > > I do agree this is a fasinating discussion > > Dawn > > Dawn, I just saw my new hepatologist last week, and he wanted to do an ERCP just to " get a baseline look " at my disease. I had the ERCP which diagnosed my disease in Oct of 2007, after an MRCP which did not show anything. I've had another, subsequent MRCP which also showed nothing, and my previous hepatologist indicated to me that he was fine with using the MRCP to monitor me, even though they are not seeing the damage that they know is there in the small ducts. I asked him, " What if year after year, the MRCP continues to show nothing? " He just smiled and said, " That will be a good thing. " My new hepatologist said that you can't see the small ducts on the MRCP & that's why he wants to do another ERCP. Maybe I misunderstood him, and maybe he just meant you can't see them as well. But it was interesting to me that your MRCP showed beading in your small ducts. I don't want another ERCP, unless it's really necessary. I'm not having any symptoms or problems right now. For now, my new hepatologist has agreed to put the ERCP off if I get images of my first ERCP to him. But I don't know if he is going to be willing to rely on MRCPs like my previous hepatologist was. Debbie Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 29, 2009 Report Share Posted January 29, 2009 Debbie, It is possible that I misunderstood my doctors, it has been a few years now. What I understood was that as the small ducts became scarred they showed up on the MRCP and then those would kind of fade away as they became obliterated. WHen you look at my MRCP pictures you can see the white beeds going the whole way through at some points and then some areas there is absolutely nothing but sponge like material (not sure howw to really describe this) In some of my previous MRCP's I had white bead like lines where there is now just the sponge like stuff. They did tell me that it was invading my large ducts so maybe I misunderstood. I can not have ERCP's any more so I don't have anything to go by there. I cholongiogram last year and they siad the large ducts look pretty good on it, but I did not see those films. I am beginning to think that every doctor has a different opinion about this disease!! Dawn > > > > > > I am by no means an expert, But from what I was told, at least for me, > > the MRCP shows both the small and large and my small ducts had the > > beaded appearance but the large did not; however, my last MRCP showed > > that I was begining to develop narrowing of the large ducts. I have no > > external duct involvement as of my last MRCP almost 2 years ago. When > > I look at my MRCP's you can clearly see the differences between them > > and and I could even see where some of the small ducts were no longer > > visable on the last one compared to some of the previous ones. > > > > I do agree this is a fasinating discussion > > > > Dawn > > > > > > Dawn, > > I just saw my new hepatologist last week, and he wanted to do an ERCP just to " get a > baseline look " at my disease. I had the ERCP which diagnosed my disease in Oct of 2007, > after an MRCP which did not show anything. I've had another, subsequent MRCP which > also showed nothing, and my previous hepatologist indicated to me that he was fine with > using the MRCP to monitor me, even though they are not seeing the damage that they > know is there in the small ducts. I asked him, " What if year after year, the MRCP continues > to show nothing? " He just smiled and said, " That will be a good thing. " > > My new hepatologist said that you can't see the small ducts on the MRCP & that's why he > wants to do another ERCP. Maybe I misunderstood him, and maybe he just meant you > can't see them as well. But it was interesting to me that your MRCP showed beading in > your small ducts. > > I don't want another ERCP, unless it's really necessary. I'm not having any symptoms or > problems right now. For now, my new hepatologist has agreed to put the ERCP off if I get > images of my first ERCP to him. But I don't know if he is going to be willing to rely on > MRCPs like my previous hepatologist was. > > Debbie > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 29, 2009 Report Share Posted January 29, 2009 Debbie, It is possible that I misunderstood my doctors, it has been a few years now. What I understood was that as the small ducts became scarred they showed up on the MRCP and then those would kind of fade away as they became obliterated. WHen you look at my MRCP pictures you can see the white beeds going the whole way through at some points and then some areas there is absolutely nothing but sponge like material (not sure howw to really describe this) In some of my previous MRCP's I had white bead like lines where there is now just the sponge like stuff. They did tell me that it was invading my large ducts so maybe I misunderstood. I can not have ERCP's any more so I don't have anything to go by there. I cholongiogram last year and they siad the large ducts look pretty good on it, but I did not see those films. I am beginning to think that every doctor has a different opinion about this disease!! Dawn > > > > > > I am by no means an expert, But from what I was told, at least for me, > > the MRCP shows both the small and large and my small ducts had the > > beaded appearance but the large did not; however, my last MRCP showed > > that I was begining to develop narrowing of the large ducts. I have no > > external duct involvement as of my last MRCP almost 2 years ago. When > > I look at my MRCP's you can clearly see the differences between them > > and and I could even see where some of the small ducts were no longer > > visable on the last one compared to some of the previous ones. > > > > I do agree this is a fasinating discussion > > > > Dawn > > > > > > Dawn, > > I just saw my new hepatologist last week, and he wanted to do an ERCP just to " get a > baseline look " at my disease. I had the ERCP which diagnosed my disease in Oct of 2007, > after an MRCP which did not show anything. I've had another, subsequent MRCP which > also showed nothing, and my previous hepatologist indicated to me that he was fine with > using the MRCP to monitor me, even though they are not seeing the damage that they > know is there in the small ducts. I asked him, " What if year after year, the MRCP continues > to show nothing? " He just smiled and said, " That will be a good thing. " > > My new hepatologist said that you can't see the small ducts on the MRCP & that's why he > wants to do another ERCP. Maybe I misunderstood him, and maybe he just meant you > can't see them as well. But it was interesting to me that your MRCP showed beading in > your small ducts. > > I don't want another ERCP, unless it's really necessary. I'm not having any symptoms or > problems right now. For now, my new hepatologist has agreed to put the ERCP off if I get > images of my first ERCP to him. But I don't know if he is going to be willing to rely on > MRCPs like my previous hepatologist was. > > Debbie > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 29, 2009 Report Share Posted January 29, 2009 Debbie, It is possible that I misunderstood my doctors, it has been a few years now. What I understood was that as the small ducts became scarred they showed up on the MRCP and then those would kind of fade away as they became obliterated. WHen you look at my MRCP pictures you can see the white beeds going the whole way through at some points and then some areas there is absolutely nothing but sponge like material (not sure howw to really describe this) In some of my previous MRCP's I had white bead like lines where there is now just the sponge like stuff. They did tell me that it was invading my large ducts so maybe I misunderstood. I can not have ERCP's any more so I don't have anything to go by there. I cholongiogram last year and they siad the large ducts look pretty good on it, but I did not see those films. I am beginning to think that every doctor has a different opinion about this disease!! Dawn > > > > > > I am by no means an expert, But from what I was told, at least for me, > > the MRCP shows both the small and large and my small ducts had the > > beaded appearance but the large did not; however, my last MRCP showed > > that I was begining to develop narrowing of the large ducts. I have no > > external duct involvement as of my last MRCP almost 2 years ago. When > > I look at my MRCP's you can clearly see the differences between them > > and and I could even see where some of the small ducts were no longer > > visable on the last one compared to some of the previous ones. > > > > I do agree this is a fasinating discussion > > > > Dawn > > > > > > Dawn, > > I just saw my new hepatologist last week, and he wanted to do an ERCP just to " get a > baseline look " at my disease. I had the ERCP which diagnosed my disease in Oct of 2007, > after an MRCP which did not show anything. I've had another, subsequent MRCP which > also showed nothing, and my previous hepatologist indicated to me that he was fine with > using the MRCP to monitor me, even though they are not seeing the damage that they > know is there in the small ducts. I asked him, " What if year after year, the MRCP continues > to show nothing? " He just smiled and said, " That will be a good thing. " > > My new hepatologist said that you can't see the small ducts on the MRCP & that's why he > wants to do another ERCP. Maybe I misunderstood him, and maybe he just meant you > can't see them as well. But it was interesting to me that your MRCP showed beading in > your small ducts. > > I don't want another ERCP, unless it's really necessary. I'm not having any symptoms or > problems right now. For now, my new hepatologist has agreed to put the ERCP off if I get > images of my first ERCP to him. But I don't know if he is going to be willing to rely on > MRCPs like my previous hepatologist was. > > Debbie > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 29, 2009 Report Share Posted January 29, 2009 Hi Don; I think that the general rule is that if the classic signs of PSC (i.e. beaded appearance of bile ducts) are seen on either ERCP or MRCP, then it is " classic " or " typical " PSC. The small-duct variant of PSC is where they can't see any beading of the ducts on ERCP or MRCP, so that the cholangiogram looks normal. So, in your case, if they saw the classic beading it is likely " classic " or " typical " PSC. But within " classic " PSC there is probably substantial variability .... some patients may show strictures in the really large ducts both within and outside the liver. Others may show strictures or beading in only the medium and somewhat finer branches within the liver, without involvement of the really large ducts. It's the very tiny branches at the tips of the biliary tree that seem to be affected in the small-duct PSC variant, and it's these that are really difficult to see in ERCP. Perhaps newer MRCP procedures (using contrast agents) can better visualize these tiny branches these days? The following text is from: Saich R, Chapman R 2008 Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease. World J. Gastroenterol. 14: 331-337. http://www.wjgnet.com/1007-9327/14/331.asp _________________________ SMALL DUCT PSC " A variant of the disease called `small duct PSC' is applied to the small percentage of patients with characteristic clinical findings but who have a normal cholangiogram. The absence of macroscopic abnormalities necessitates liver biopsy for the diagnosis of small- duct PSC. Characteristic features of PSC on liver biopsy are concentric rings of connective tissue around bile ducts, the so- called `onion ring' appearance[77]. Other features which are less specific include enlargement of the portal triads, periportal inflammation with mononuclear and polymorphonuclear cells, oedema and bile duct proliferation. Extension into the hepatic parenchyma with bridging fibrosis and ultimately cirrhosis represents advanced disease. Small duct PSC appears to have a more favourable prognosis with a median survival 29.5 years compared to 17 years for classical PSC[78- 81], although occasionally patients with small duct PSC progress to large duct disease. " _________________________ I guess what they mean here by " large duct disease " is that the cholangiogram now begins to show the " classic " or " typical " PSC pattern. I don't think that their use of the term " large duct disease " means that it has to involve the main duct coming out of the liver ... I think it just means that it is now visible in ERCP or MRCP. Best regards, Dave (father of (23); PSC 07/03; UC 08/03) > > > > Hi Don; > > > > As Debbie has already mentioned, it is pretty challenging to diagnose > > small-duct PSC. > > Hi again Dave, > I've had few ERCP's,compared to most of the group. The one that made a > big impression on me was when I had my one big episode of cholangitis, > or whatever it was. Chills and fever for 3 days. I refused to go into > the ER, as is my Norwegian MO, until I was really a mess, and they told > me at work I was turning yellow. My own DR (not my present GI)was not > around and his partner decided to remove my gall bladder. They did an > ERCP first, and that's the only X-ray of my liver I've seen. It showed > all the ducts with the classic beaded appearance. > > Were these small ducts or medium or large? Is the large duct just the > main bile duct, outside the liver? > > This discussion is fascinating, but perhaps only idle curiousity on my > part. > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 29, 2009 Report Share Posted January 29, 2009 Hi Don; I think that the general rule is that if the classic signs of PSC (i.e. beaded appearance of bile ducts) are seen on either ERCP or MRCP, then it is " classic " or " typical " PSC. The small-duct variant of PSC is where they can't see any beading of the ducts on ERCP or MRCP, so that the cholangiogram looks normal. So, in your case, if they saw the classic beading it is likely " classic " or " typical " PSC. But within " classic " PSC there is probably substantial variability .... some patients may show strictures in the really large ducts both within and outside the liver. Others may show strictures or beading in only the medium and somewhat finer branches within the liver, without involvement of the really large ducts. It's the very tiny branches at the tips of the biliary tree that seem to be affected in the small-duct PSC variant, and it's these that are really difficult to see in ERCP. Perhaps newer MRCP procedures (using contrast agents) can better visualize these tiny branches these days? The following text is from: Saich R, Chapman R 2008 Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease. World J. Gastroenterol. 14: 331-337. http://www.wjgnet.com/1007-9327/14/331.asp _________________________ SMALL DUCT PSC " A variant of the disease called `small duct PSC' is applied to the small percentage of patients with characteristic clinical findings but who have a normal cholangiogram. The absence of macroscopic abnormalities necessitates liver biopsy for the diagnosis of small- duct PSC. Characteristic features of PSC on liver biopsy are concentric rings of connective tissue around bile ducts, the so- called `onion ring' appearance[77]. Other features which are less specific include enlargement of the portal triads, periportal inflammation with mononuclear and polymorphonuclear cells, oedema and bile duct proliferation. Extension into the hepatic parenchyma with bridging fibrosis and ultimately cirrhosis represents advanced disease. Small duct PSC appears to have a more favourable prognosis with a median survival 29.5 years compared to 17 years for classical PSC[78- 81], although occasionally patients with small duct PSC progress to large duct disease. " _________________________ I guess what they mean here by " large duct disease " is that the cholangiogram now begins to show the " classic " or " typical " PSC pattern. I don't think that their use of the term " large duct disease " means that it has to involve the main duct coming out of the liver ... I think it just means that it is now visible in ERCP or MRCP. Best regards, Dave (father of (23); PSC 07/03; UC 08/03) > > > > Hi Don; > > > > As Debbie has already mentioned, it is pretty challenging to diagnose > > small-duct PSC. > > Hi again Dave, > I've had few ERCP's,compared to most of the group. The one that made a > big impression on me was when I had my one big episode of cholangitis, > or whatever it was. Chills and fever for 3 days. I refused to go into > the ER, as is my Norwegian MO, until I was really a mess, and they told > me at work I was turning yellow. My own DR (not my present GI)was not > around and his partner decided to remove my gall bladder. They did an > ERCP first, and that's the only X-ray of my liver I've seen. It showed > all the ducts with the classic beaded appearance. > > Were these small ducts or medium or large? Is the large duct just the > main bile duct, outside the liver? > > This discussion is fascinating, but perhaps only idle curiousity on my > part. > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 29, 2009 Report Share Posted January 29, 2009 Hi Don; I think that the general rule is that if the classic signs of PSC (i.e. beaded appearance of bile ducts) are seen on either ERCP or MRCP, then it is " classic " or " typical " PSC. The small-duct variant of PSC is where they can't see any beading of the ducts on ERCP or MRCP, so that the cholangiogram looks normal. So, in your case, if they saw the classic beading it is likely " classic " or " typical " PSC. But within " classic " PSC there is probably substantial variability .... some patients may show strictures in the really large ducts both within and outside the liver. Others may show strictures or beading in only the medium and somewhat finer branches within the liver, without involvement of the really large ducts. It's the very tiny branches at the tips of the biliary tree that seem to be affected in the small-duct PSC variant, and it's these that are really difficult to see in ERCP. Perhaps newer MRCP procedures (using contrast agents) can better visualize these tiny branches these days? The following text is from: Saich R, Chapman R 2008 Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease. World J. Gastroenterol. 14: 331-337. http://www.wjgnet.com/1007-9327/14/331.asp _________________________ SMALL DUCT PSC " A variant of the disease called `small duct PSC' is applied to the small percentage of patients with characteristic clinical findings but who have a normal cholangiogram. The absence of macroscopic abnormalities necessitates liver biopsy for the diagnosis of small- duct PSC. Characteristic features of PSC on liver biopsy are concentric rings of connective tissue around bile ducts, the so- called `onion ring' appearance[77]. Other features which are less specific include enlargement of the portal triads, periportal inflammation with mononuclear and polymorphonuclear cells, oedema and bile duct proliferation. Extension into the hepatic parenchyma with bridging fibrosis and ultimately cirrhosis represents advanced disease. Small duct PSC appears to have a more favourable prognosis with a median survival 29.5 years compared to 17 years for classical PSC[78- 81], although occasionally patients with small duct PSC progress to large duct disease. " _________________________ I guess what they mean here by " large duct disease " is that the cholangiogram now begins to show the " classic " or " typical " PSC pattern. I don't think that their use of the term " large duct disease " means that it has to involve the main duct coming out of the liver ... I think it just means that it is now visible in ERCP or MRCP. Best regards, Dave (father of (23); PSC 07/03; UC 08/03) > > > > Hi Don; > > > > As Debbie has already mentioned, it is pretty challenging to diagnose > > small-duct PSC. > > Hi again Dave, > I've had few ERCP's,compared to most of the group. The one that made a > big impression on me was when I had my one big episode of cholangitis, > or whatever it was. Chills and fever for 3 days. I refused to go into > the ER, as is my Norwegian MO, until I was really a mess, and they told > me at work I was turning yellow. My own DR (not my present GI)was not > around and his partner decided to remove my gall bladder. They did an > ERCP first, and that's the only X-ray of my liver I've seen. It showed > all the ducts with the classic beaded appearance. > > Were these small ducts or medium or large? Is the large duct just the > main bile duct, outside the liver? > > This discussion is fascinating, but perhaps only idle curiousity on my > part. > Quote Link to comment Share on other sites More sharing options...
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