Primary Sclerosing Cholangitis

What is Primary Sclerosing Cholangitis?

Primary sclerosing cholangitis (PSC) is a chronic, progressive disease of unknown cause in which the bile ducts inside and outside the liver become inflamed and scarred. The scarring causes narrowings (strictures) that can eventually lead to biliary cirrhosis.^[1] It is a serious disease that requires care from a hepatologist, and our role is limited to supportive care.

Common symptoms

Some people have no symptoms when PSC is found. Others have itching (pruritus), fatigue, pain in the upper right abdomen, repeated bacterial infections of the bile ducts (cholangitis), or complications of portal hypertension.^[2]

• Itching, which can be severe
• Tiredness and low energy
• Upper right abdominal pain
• Yellowing of the skin and eyes (jaundice), dark urine or pale stools
• Fever and chills when a bile duct becomes infected^[2]

Causes and risk factors

The cause is unknown. PSC is regarded as an autoimmune-type liver disease because of its strong immunogenetic background, and the gut microbiome is increasingly thought to play a role.^{[2, 3]} It is more common in young men than in other groups.^[3]

PSC is strongly linked with inflammatory bowel disease, particularly ulcerative colitis. About 60 to 80 percent of people with PSC have inflammatory bowel disease.^[1] In the other direction, a pooled analysis found PSC in about 2 percent of people with inflammatory bowel disease.^[4]

How it is diagnosed and treated conventionally

Diagnosis combines blood tests showing a cholestatic pattern with imaging of the bile ducts. MRI of the bile ducts (MRCP) is now the preferred non-invasive test.^[1] People with PSC need regular surveillance, including colonoscopy in those with colitis, because the risk of colorectal cancer and of bile duct cancer (cholangiocarcinoma) is higher.^{[1, 2]}

No medical therapy has been shown to slow the disease or improve transplant-free survival.^{[1, 3]} Ursodeoxycholic acid is widely used, but a meta-analysis of eight trials with 567 patients found no significant benefit for mortality, itch, fatigue or disease progression.^[5] Narrowed ducts that cause symptoms may be treated endoscopically, and liver transplantation is effective for advanced disease, although PSC can recur in the new liver.^[3]

Why Primary Sclerosing Cholangitis calls for a whole-person approach

PSC affects the liver, the bile ducts, the bowel and day-to-day energy and mood. Because no therapy has proven to alter the course of the disease, much of ongoing care focuses on monitoring, complications and quality of life.^[6]

Supportive care can address some of the surrounding effects. It cannot treat the bile duct disease.

Itch and fatigue

Itch and fatigue are two of the most common symptoms and are listed among the main features of the disease.^[2] Itch in PSC is treated by the liver team with medicines. We are not aware of trials of acupuncture or herbs for itch caused by bile duct disease.

The bowel connection

Most people with PSC also have inflammatory bowel disease, which can bring its own pain, diarrhea and fatigue. Whether PSC and inflammatory bowel disease are one disease or two is still debated.^{[1, 7]} Bowel symptoms should be managed by your gastroenterologist.

Stress and sleep

Living with a rare chronic disease, with monitoring and uncertainty about the future, is a burden for many people. Poor sleep and stress can add to tiredness. They are therefore reasonable targets for supportive care.

Acupuncture as supportive care in Primary Sclerosing Cholangitis

What the research shows

No clinical trials of acupuncture in PSC were found in PubMed, and acupuncture has not been shown to affect the bile ducts or the course of the disease. What we can describe comes from other groups of patients and may not apply.

For fatigue, a Cochrane review of fatigue in inflammatory bowel disease found low-certainty evidence from very small electroacupuncture studies, with better fatigue scores than no treatment in 27 participants and than sham electroacupuncture in 30 participants at 8 weeks, and a smaller sham difference at 16 weeks. The review concluded that no firm conclusions could be drawn.^[8] A meta-analysis of 16 trials with 1,346 people with chronic fatigue syndrome found acupuncture favored over sham for overall response, but most studies were of low quality and the authors said no firm conclusion could be reached.^[9]

For sleep, a review of 24 trials in insomnia included a meta-analysis of 15 trials that found acupuncture improved sleep scores compared with medication after three or more weeks, with high variability between studies.^[10] For chronic pain in general, an individual patient data meta-analysis found acupuncture better than sham or no acupuncture, although it did not include liver disease.^[11]

How acupuncture may work

We are not aware of research on how acupuncture might act in PSC. In chronic pain, a meta-analysis concluded that the benefit cannot be explained by placebo effects alone, and that factors beyond needling at specific points also contribute.^[11] This does not tell us how acupuncture might act in this disease.

What treatment involves

Treatment uses fine, sterile, single-use needles placed at points on the body, usually left in place for about 20 to 40 minutes. A short course of visits is followed by reassessment of energy, sleep and stress. Please share your latest blood tests, because people with advanced liver disease may bruise or bleed more easily.

Serious problems from acupuncture are rare. In prospective studies, about 9 in 100 patients had a minor reaction such as brief bleeding, soreness or redness at a needle site, and serious events occurred in about 1 in 10,000 patients.^[12] Tell the practitioner first if you take a blood thinner, have a bleeding disorder or low platelet count, have fluid in the abdomen, are pregnant, have a pacemaker (relevant to electroacupuncture), or take immune-suppressing medicines. Also tell the practitioner if you have an active infection or fever.

Ayurvedic medicine as supportive care in Primary Sclerosing Cholangitis

The Ayurvedic view

Classical Ayurveda does not describe PSC. A practitioner might relate bile-duct disease with jaundice and itch to disturbed Pitta, the principle linked with heat and bile, and to the traditional category of liver and bile disorders. This is a traditional framework for individualizing supportive care, not a medical explanation of the disease.

Therapies that may be used

Because PSC is a serious liver disease, Ayurvedic care here is conservative:

• Dietary guidance such as regular, simple, freshly prepared meals, working with the diet advice from your liver team
• Regular sleep and meal times and stress-reducing practices such as breathing exercises and gentle yoga within your limits
• Gentle warm oil massage on intact skin

Panchakarma procedures, including purgation and enema therapies, are not appropriate for people with PSC. They are not appropriate during pregnancy, acute illness or frailty, and we would not use them in cirrhosis or with bile duct infection, which PSC can cause.^[2] We also do not use traditional metal-containing (rasa shastra) preparations, which had a higher rate of lead and mercury in a study of online products.^[13] Oral herbal products are covered in the next section.

What the research shows

No clinical studies of Ayurvedic treatment for PSC were found in PubMed. There is no evidence that any Ayurvedic therapy improves bile duct disease. Diet and routines are offered for general well-being and sleep, not as treatment for the liver.

Herbal medicine as supportive care in Primary Sclerosing Cholangitis

Herbs and formulas that have been studied

No herb or formula has been tested in PSC, and none has been shown to help it. Because the bile ducts and liver are already damaged, we do not start oral herbs unless your hepatologist agrees, and we generally avoid them.

What the research shows

There is no research on herbal medicine for this disease. We do not cite any herbal evidence for PSC and make no claim that any herb helps.

Safety and herb-drug interactions

Herbs are not harmless to the liver. Herbal and dietary supplements account for about 20 percent of drug-induced liver injury cases in the United States, and the culprit in multi-ingredient products is often unknown.^[14] A systematic review of liver injury from Chinese herbal medicine named He-Shou-Wu as the most commonly reported herb and found that Dang Gui was a common ingredient in implicated patent medicines and decoctions.^[15]

Ashwagandha, often marketed for stress, has been linked to liver injury that is frequently cholestatic and presents with jaundice and itch, which can look like worsening PSC. A review of case reports advised that people with advanced chronic liver disease avoid it.^[16] For that reason we do not recommend it to people with PSC.

Herbs can also interact with prescribed drugs, including immune-suppressing medicines, and many of the reported interactions come from case reports.^[17] About one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[13] Do not start any herb or supplement without telling your hepatologist and us.

Translating traditional medicine into modern biological language

This section is interpretation, not equivalence. Chinese medicine may describe PSC-like jaundice and right-sided pain through patterns such as liver qi stagnation or damp-heat in the liver and gallbladder. Ayurveda may describe aggravated Pitta. Researchers instead study immune activity around the bile ducts, the gut microbiome and the way bile flow is impaired.^{[2, 3]}

No study has shown that a traditional pattern matches a biological process in PSC. For the symptoms we can support, such as fatigue and sleep, the research simply asks whether symptoms improve, without claiming a traditional pattern explains them.^[8]

How this care fits with your medical care

PSC requires ongoing specialist care. For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care. Your team may include a hepatologist and a gastroenterologist, and a transplant center if you are listed.

Please bring your diagnosis, recent liver blood tests and imaging reports, details of colonoscopy surveillance, a full list of medicines and supplements, and any transplant plans. We do not diagnose, monitor or treat the disease, and we do not change prescribed treatment, including ursodeoxycholic acid. Do not stop any prescribed medicine without your prescriber.

When to seek urgent medical care

PSC can cause serious complications, including bile duct infection and bleeding from portal hypertension.^[2]

• Fever with chills, especially with jaundice or abdominal pain, which can signal a bile duct infection
• Vomiting blood or passing black, tarry stools
• New confusion, extreme sleepiness or unusual behavior
• Rapidly deepening jaundice or severe new abdominal pain
• A swollen abdomen or leg swelling that is getting worse
• Unplanned weight loss with persistent abdominal pain

If you have any of these emergencies, call 911 or go to the nearest emergency department.