The author is not medically trained. The described research takes place in the Netherlands, and therefore numbers, results stated, conclusions and medical protocols might vary according to each country. Please contact your own physician with any queries you have regarding your own health.

The Netherlands has approx. 17 million habitants and is 41.520 km2 / 16,031 sq. m..

The Dutch health care system is mandatory and the premium is not related to health situation, age  or income. It has a dual-level system: primary and curative care is paid by the private mandatory insurance (e.g. GP visits) , while long term care is covered by social insurance (funded via earmarked taxation). Hospitals are mostly privately run and not-for-profit.

In the first week of 2014, I conducted an interview with Kirsten Boonstra, PhD researcher at the Academic Medical Center ( AMC ), Amsterdam (the Netherlands)  regarding her recent  publication in Hepatology 1 (’The monthly liver journal’)  concerning a unique study on Primary Sclerosing Cholangitis ( PSC ) in the Netherlands. The AMC PSC research group specializes in studying the progression of the disease. What conclusions can we draw from this groundbreaking research project? And are there consequences for PSCers ? The following information comes from her interview.

First, the research design is quite unique as it consists of a partnership between 44 Dutch hospitals, with all doctors & nurses involved, and the three liver transplant centers in the Netherlands ( LUMC , UMCG and Erasmus MC ) . Geographically,  this covers roughly half of the Netherlands . All records of patients with confirmed or suspected PSC were examined individually by the researchers Boonstra and Ponsioen, to confirm the diagnosis of PSC . This resulted in  590 PSC patients. Each year their medical records have been and will continue to be updated, so researchers will continue to have insight into the course of their disease . It is the first time a rare disease like PSC will be examined in a defined geographical area amongst an entire population. After 6 years of research, the AMC team has covered roughly half the population and upon completion of the project in 6 years time, they will not only have all PSCers in the Netherlands registered, but they already have valuable up-to-date information on a personal level available of a 1-to-12 year timeframe.

Not only the research design is groundbreaking, the  research results are also very interesting in many ways. For instance, the number of Dutch PSC’ers are lower than ever reported: six in 100,000 , in absolute terms .

In addition, the time from diagnosis to ‘end point’ ( transplantation or death)  was not 13.2 years, as reported in the transplant centers, but  just over 21 years throughout the Dutch PSC population. That is partly due to the fact the first number was based only on patients in transplant centers , who are generally referred to the transplant centers as  they are already quite ill . In this study however, they looked at patients from the various academic hospitals as well as regional hospitals  and therefore there are  fewer sick PSCers seen with a more favorable disease course than previously thought.


Cholangiocarcinoma ( CCA ) , is very rare among the Dutch population, with most cases of Dutch CCA appearing amongst PSC’ers . The risk of CCA is nearly 400 times greater for PSC patients than the healthy population of the same age . It is worth mentioning that CCA hardly occurs with ‘small duct PSC ‘ ( That is, PSC in the small bile ducts ) . About 9 % of the surveyed PSC patients have small duct PSC .


Colorectal Cancer
Global research shows that the risk of colorectal cancer ( CRC ) , which is ten times greater among PSC patients , occurs on average in a relatively young age , namely 39  (compared to IBD controls (59yo)). In general,  the risk of death from colorectal cancer is much lower than from CCA ,  especially if the cancer is detected in time. For the newly PSC diagnosed patient, the current Dutch procedure is e.g.  to undergo a colonoscopy  at the so-called “diagnostic time”, which is the time of diagnosis of PSC. If there is no inflammation seen, then there is no need to have any more endoscopies. However, if inflammation is observed in the intestines , it is advisable to have annual colonoscopies.

Research shows the number of deaths from CRC is significantly lower in the group of patients who have annual colonoscopies than in the group that did not  have annual colonoscopies or did not have them often enough. ” This highlights the importance of an annual endoscopy if there is an inflammation. And please note it justifies to have this screening at a relative young age, due  to the young age of onset of colorectal cancer: 39 years”,  researcher Boonstra states .

Ursodeoxycholic acid

In half of all patients the use of ursodeoxycholic acid ( among users often known as ‘ urso ‘ ) has a positive effect on laboratory values, and almost all users say they feel better taking it. However, the drug has no proven effect on the further development of PSC . Often, the use of ursodeoxycholic acid will inhibit the inflammation.


NSAIDs are Non – Steroidal Anti – Inflammatory Drugs. Examples of commonly used NSAIDs include ibuprofen , naproxen and diclofenac. There appears to be evidence that the occasional use of NSAIDs  has a protective effect on the progression of the disease. However, Boonstra indicates that at present, one can not make  firm conclusions yet , but it is certainly  worth further research , also in relation to the possible harmful effects in many related diseases like Crohn’s disease or Ulcerative Colitis.

Transplant recipients

Although strictly speaking, transplant recipients aren’t a part of this research,  former PSC’ers who have undergone a transplant during the research period are tracked by the researchers in order to get a better view on this population. For instance it is possible that a PSCer returns to the hospital after transplant, or that  the transplant is damaged , for example, at the bile junction. It is interesting material for researchers and doctors , who thanks to  this group of patients may be able to better treat patients in the future.


And what we can do as PSC patients ourselves ?
You can play an active role, including keeping track of whether or not you have had your annual endoscopy and biennial Dexa scan ( for bone density ). There is a PSC protocol in the Netherlands, but this study shows that it is not always followed up appropriately. It is possible that a  doctor sometimes forgets to ask . You can always mention it to your doctor!

With an annual colonoscopy , even with little or no gastrointestinal symptoms , the mortality rate drops significantly . For PSC patients with a confirmed healthy gut (without inflammation ) such annual examinations are not necessary .

In addition , it is essential that patients with this rare disease participate in various surveys and registries, and they give permission for the use of their information as research material and research results on a national and international level . The more researchers and doctors know about this disease ,  the faster a possible treatment and / or medication for PSC will be available.


Especially for a rare disease , it is important that research takes place with a large population cohort , so that good and reliable data and details of the disease emerge. As a result of this study, the numbers for the incidence and prevalence rates of the disease ( 6 per 100,000 ) and survival years after diagnosis internationally have become the new standard ( 21 years to transplantation or death) .

The research has yet another positive side effect : the PSC specialists in AMC increasingly receive phone calls from specialists from one of the 44 hospitals involved ,  to discuss a patient or to get other PSC – related questions answered. Definitely an increase of peer consultation , and it is fair to say the  PSC patients  can only benefit from such  collaboration.

What’s next ?

PhD Liesbeth de Vries has recently joined the AMC PSC research team and she will further expand and explore the Dutch PSC’ers in the coming years. Through this research, we will have a unique situation in the Netherlands : all PSC’ers will be mapped and thanks to annual updates, the course of PSC can be closely monitored .

The whole research project is currently being stored in a large database in the AMC , accessible only to AMC researchers. Within the research team they hope to further develop a web-based version, where every doctor involved can login and add relevant information on his patients.

This is the largest population-based cohort of PSC patients worldwide . This group of patients is being followed prospectively at this time. It will be very interesting for future researchers to do further PSC research as the research  population is  already known and identified . This will save valuable time and money . Moreover, it will be interesting to see once all PSC’ers are registered, whether there are regional differences  or whether more generic clinical developments can be discovered .

* Drs. Boonstra hopes to defend her. PhD thesis in October 2014.  She would then like to pursue her career as a gastrointestinal hepatologist  physician and will apply for an apprenticeship in the coming year.


Cohort: A group of people with a common characteristic, used in a study

Colonoscopy: Endoscopic examination of the colon

DEXA scan: Dual Energy X-ray Absorptiometry. This measures the bone density to determine if there is ‘bone loss’ (osteoporosis)

Endoscopy: Visual examination of the interior of a hollow body organ by use of an endoscope (a flexible tube)

Prospective study: Where a group PSC patients are followed over the further course of the disease which is not primarily caused by another disease

Sclerosing: Hardening of tissue

Cholangitis:  Inflammation of the bile ducts


1 . Boonstra K , Weersma R Erpecum Of KJ , et al Population -Based Epidemiology, Malignancy Risk and Outcome of Primary Sclerosing Cholangitis. Hepatology 2013 , 58:2045-2055 .

Author: Marleen K

Editor: Valmae


Link to article featured in interview: