Guest Commentary: Distinguishing between PSC and PBC after a name change

In this guest commentary, Ricky Safer, CEO, PSC Partners Seeking a Cure , and Martine Walmsley, Chair of Trustees, PSC Support, address the recent name change for primary biliary cholangitis and the potential for confusion with primary sclerosing cholangitis. Safer and Walmsley, in conjunction with medical advisors, hope to bring increased awareness of the PBC/PSC discussion to the medical community. PSC Partners Seeking a Cure (U.S.) and PSC Support (UK) are nonprofit patient advocacy organizations collaborating for education and support of PSC patients and their caregivers and work toward a cure for PSC.

Primary biliary cholangitis and primary sclerosing cholangitis, with almost-identical names, give us substantial reason to consider our two diseases to be closely related. However, with similar names also comes the significant risk for confusion between the two diseases, which can lead to dangerous misdiagnoses.

While the two rare autoimmune bile duct diseases often share the same symptoms of fatigue and pruritus and might therefore appear to be the same disease, they are markedly different in the site of disease involvement, co-morbidities, monitoring requirements, method of diagnosis, and treatment options. An erroneous diagnosis that results in neglect of regular treatment and monitoring could have disastrous results for PSC and PBC patients alike.

What’s in a Name?

A single word might signify the difference between receiving life-saving cancer surveillance or not being monitored at all. In our PSC community and in PSC Partners’ patient-driven registry of over 1,000 PSC patients, we have discovered several instances of misdiagnosis that have occurred in various locations worldwide. Each incorrect diagnosis of a PBC patient indicates a loss of precious treatment time and unnecessary cancer surveillance, whereas each incorrect diagnosis of a PSC patient means an absence of critical cancer monitoring. As PSC patient organizations, we seek to provide easy access to accurate information so that PSC and PBC patients alike will receive appropriate diagnoses and the screening and treatment their conditions require.

What’s Not in a Name?

PSC coexists with inflammatory bowel disease in about 80% of its population while IBD remains rare in PBC. Only PSC comes with a seriously increased risk for colorectal cancer and cholangiocarcinoma. Furthermore, PBC has a 90% predominance of women, whereas PSC affects two men for every woman. PBC can be diagnosed through a combination of blood tests and liver biopsy, while PSC is most often diagnosed by MRCP. There are no effective medical treatments for PSC other than a liver transplant, after which PSC can recur. In a large number of cases, PBC responds to ursodeoxycholic acid and now to obeticholic acid, which for many, will slow down disease progression.* We celebrate the new treatment option for the PBC community.

The last black box

Despite significant advances in our understanding of PSC in the last decade, PSC remains “the last black box of hepatology.” The progress made in finding effective treatments for PBC serves as a source of hope for our PSC community members. It is an exciting time for PSC and PBC, with several new potential therapies in the pipeline for both diseases. But none of these advances matter if patients are not accurately diagnosed from the start.

PSC Partners Seeking a Cure and PSC Support are committed to disseminating information about the similarities and differences of these two almost identically-named diseases. It is particularly important that these distinguishing characteristics are understood by physicians and clinics that have little exposure to patients with PSC or PBC.

We seek your assistance in this campaign. Please share with your colleagues and students the simple PSC and PBC Differentiation Chart that we have created with the help of PSC and PBC experts.

Ricky Safer can be reached at contactus@pscpartners.org.

References:

Bowlus C. Hepat Med. 2016;doi:10.2147/HMER.S91709.

Hirschfield G, Trivedi P. PSC Support website. www.pscsupport.org.uk/psc-pbc. November 6, 2016. Accessed March 17, 2017.

Disclosures: Safer reports that PSC Partners received an educational grant from Intercept Pharmaceuticals through their U.S. Practice to Policy Health Awards Program. Walmsley reports no relevant financial disclosures.