Forty members and carers attended our summer meeting this year.  Numbers were down compared to previous years; but August is obviously not the best time to have this meeting, being a peak holiday month.

Simon Rushbrook, from Addenbrookes, Cambridge, began the meeting by giving us an update on the important Genome-wide PSC study to which most of us have contributed our DNA, and which, it is hoped, will eventually provide us with key genetic data on PSC. Four hundred and fifty patients have already been processed; there are to be 400 more from QEH, Birmingham; 150 from the Freeman, Newcastle and 150 from the Royal Free, London. More will be coming from the Liver Unit, King's College, London. One hundred and two UK hospitals are participating, including the Liver Centres. Dr. Chapman was asked how many PSC patients are being treated in the UK, and he said about 3,500. But that's not the same as saying that only 3,500 have PSC. The figure would be much higher.
Roger then went over some of the basics of PSC and also looked at promising new treatments coming on line. He thought that this will lead to much better treatment of PSC. (For a detailed account see his paper contained in the report of the Oslo Conference, in the current newsletter). These treatments include the use of several antibiotics, including rifampicin. Some of us are familiar with this for the treatment of itching, but it is also a powerful inducer of PXR, a receptor of central importance in the immune system. Another antibiotic, Metronidazole, has been used in a 3-year Finnish trial. Results are promising and need to be followed up. In addition Vancomycin has been used in paediatric PSC with some positive results.
Attention has been turning to the study of nuclear receptors which regulate bile acids in cholestasis (obstruction/stagnation of the flow of bile, characteristic of PSC), especially FXR which is the major regulator of bile acids.  Medicines are now available for clinical use - FXR agonists (agonists - drugs which are activators and have a stimulating effect), These are being studied in PBC (primary biliary cirrhosis), and it is hoped that this will be extended to PSC.
Especially interesting is the use of Nor-Urso, "an exciting compound," which has been used in "knockout" mice and is found to be a big improvement on Urso. Human trials are soon to go ahead in Austria.
In response to questions from the audience, Dr. Chapman explained that while ERCP and liver biopsy were used in the past as the gold standard for diagnosing PSC; MRCP, which is non-invasive, is a technology which has been greatly improved and is now the preferred imaging technique for diagnosis. About 5% of patients undergoing ERCP get cholangitis or other compications in spite of antibiotic cover. But ERCP still has to be used for therapeutic intervention, e.g. placing stents in dominant strictures or in balloon endoscopy etc.

Stents should not be left in for longer than 2 months. (Although the feeling at Oslo was only a few days, because bacteria and sludge build up behind the stent.)
The BSG guideline, for those of us with PSC + IBD, is that we should have annual colonoscopies.  If there was any doubt about that, he said we should refer doctors to these guidelines.
Is PSC an autoimmune disease similar to PBC and AIH (Autoimmune hepatitis)? This is clearly still an open question and was much discussed in Oslo. But Dr. Chapman is firmly of the view that it is an IMMUNE DYSREGULATED DISEASE, which is not the same thing. When mention was made of the video which we were shown by David Adams at our February meeting in Birmingham, where we saw lymphocytes attacking bile ducts, magnified many thousands of times, and which appeared to be direct evidence of autoimmunity, Dr. Chapman said that they could have      

been attacking for a number of reasons, including the possibility that bacteria or viruses were present. reasons, including the possibility that bacteria or viruses were present.
Once again we are grateful to Roger for giving us his time on a Sat. afternoon to explain the intricacies of our disease when he could have been doing something more useful, like playing golf!

Fortunately most people stayed on after the break for our AGM.