Early transplant in PSC cirrhosis before variceal bleeding

What is known

• Older sources describe liver transplantation as the definitive treatment once cirrhosis and portal hypertension are recognized in PSC, and a 1992 review listed cirrhosis and portal hypertension as indications for transplantation while anticipating that transplantation might in future be indicated earlier in the disease course. ^1,2 These reports predate MELD-based allocation. In current practice, compensated cirrhosis with portal hypertension is not by itself an indication for transplantation: listing is driven by hepatic decompensation, MELD-based priority, or accepted exception criteria (for example recurrent bacterial cholangitis, intractable pruritus, or a hilar cholangiocarcinoma protocol). In the pre-bleed window this question describes, the standard of care is endoscopic screening for varices with primary prophylaxis (a non-selective beta-blocker such as carvedilol, or endoscopic band ligation) per Baveno VII and AASLD portal hypertension guidance, rather than pre-emptive transplant referral.
• In a Mayo Clinic cohort of 161 patients with PSC and no cholangiocarcinoma at entry, a history of variceal bleeding was the only independent predictor of later cholangiocarcinoma (RR 24.2, 95% CI 3.3 - 67.1). The authors note that patients with a history of variceal bleeding are typically referred for transplantation assessment irrespective of their cholangiocarcinoma risk, so this association does not help identify candidates for early transplantation. Their stated conclusion is that PSC patients who develop variceal bleeding should be urgently assessed for liver transplantation once cholangiocarcinoma has been excluded, which places the trigger at the bleed rather than before it. ³
• Before transplantation, patients with advanced cirrhotic-stage PSC should be screened for hepatocellular carcinoma and cholangiocarcinoma. ⁴

What is unknown / caveats

• No source compares transplantation performed before a first variceal bleed with transplantation performed after one, so no evidence-based threshold for pre-emptive listing in the compensated, pre-bleed window can be derived from them.

References

1. Goss JA, Shackleton CR, Farmer DG, Arnaout WS, Seu P, Markowitz JS, Martin P, Stribling RJ, Goldstein LI, Busuttil RW. Orthotopic liver transplantation for primary sclerosing cholangitis. A 12-year single center experience. Ann Surg. 1997;225:472-81; discussion 481-3. PMID: 9193175.

2. Martin FM, Braasch JW. Primary sclerosing cholangitis. Curr Probl Surg. 1992. PMID: 1587151.

3. Burak K, Angulo P, Pasha Tousif M, Egan K, Petz J, Lindor Keith D. Incidence and risk factors for cholangiocarcinoma in primary sclerosing cholangitis. Am J Gastroenterol. 2004;99:523-6. PMID: 15056096.

4. Harnois DM, Gores GJ, Ludwig J, Steers JL, LaRusso NF, Wiesner RH. Are patients with cirrhotic stage primary sclerosing cholangitis at risk for the development of hepatocellular cancer?. J Hepatol. 1997. PMID: 9314129.

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