Summerskill–Walshe–Tygstrup syndrome is a rare, inherited liver condition. People with this condition have repeated attacks of cholestasis, which means the liver cannot release bile properly. During an attack, itching (pruritus) and yellowing of the eyes and skin (jaundice) appear, often with tiredness, poor appetite, and pale stools. Each attack lasts weeks to months, then goes away completely for months or years. Between attacks, most people feel normal and tests can return to normal. The liver and bile ducts look normal on scans, and there is no blockage outside the liver. The problem is inside liver cells and relates to transporters that move bile components; the two main genetic causes are ATP8B1 (BRIC1) and ABCB11 (BRIC2). Overall outlook is good, but some people can slowly move along a spectrum toward more persistent disease (PFIC). NCBI+3Orpha+3rarediseases.info.nih.gov+3
Summerskill-Walshe-Tygstrup syndrome is a rare, inherited liver disorder. It causes repeated attacks of cholestasis, which means bile cannot flow out of the liver properly. During attacks, people develop severe itching (pruritus), jaundice (yellow skin/eyes), dark urine, pale stools, poor appetite, nausea, and weight loss. Between attacks, people usually feel normal and liver tests can go back to normal. Doctors also call it Benign Recurrent Intrahepatic Cholestasis (BRIC). “Benign” here means it usually does not cause permanent liver damage, but the intense itching can be very distressing. PubMed+1
There are two main genetic types. BRIC1 happens with changes in a gene called ATP8B1, which affects the bile canalicular “flipase” in liver cells. BRIC2 happens with changes in the ABCB11 gene, which makes the Bile Salt Export Pump (BSEP). Both problems interfere with bile salt movement and cause attacks of cholestasis and itching. The two types look very similar clinically. PMC+1
Researchers first described this condition in the 1950s, and the eponym honors Summerskill, Walshe, and Tygstrup, early contributors who reported patients with recurring intrahepatic cholestasis without duct obstruction. Today, doctors usually call it BRIC (benign recurrent intrahepatic cholestasis). MDPI+2ScienceDirect+2
Other names
This syndrome is also known as: Benign recurrent intrahepatic cholestasis (BRIC); BRIC type 1 (ATP8B1-related); BRIC type 2 (ABCB11/BSEP-related); low-GGT familial intrahepatic cholestasis (milder end of the spectrum); Summerskill’s syndrome; and intermittent intrahepatic cholestasis. accessanesthesiology.mhmedical.com+3MedlinePlus+3National Organization for Rare Disorders+3
Types
Type 1 (BRIC1) is caused by ATP8B1 gene variants. This gene affects the cell membrane’s lipid “flipase” function in hepatocytes. Patients often have normal or low GGT during attacks, with high bile acids and bilirubin. NCBI+1
Type 2 (BRIC2) is caused by ABCB11 gene variants. This gene encodes the bile salt export pump (BSEP), the main canalicular pump that exports bile salts. Like BRIC1, GGT is typically normal or only slightly raised, while bile acids and alkaline phosphatase rise. Gastro Journal+2PMC+2
Some recent reports describe BRIC-like, low-GGT recurrent cholestasis with variants in USP53 and other canalicular proteins; these remain rare and under study. turkjpediatr.org
Causes
BRIC is genetic, but attacks are often triggered by outside factors. The underlying genes make liver bile transport vulnerable; a trigger tips it into a temporary “traffic jam.” AGEB
Viral upper-respiratory infections (e.g., influenza) – most commonly reported trigger in case reviews; inflammatory cytokines and endotoxins down-regulate bile transporters, raising bile acids before bilirubin. AGEB
Bacterial infections (tonsillitis, otitis media, UTI, pneumonia) – systemic inflammation impairs transporter expression and bile flow. AGEB
Gastroenteritis – changes in gut flora and secondary bile acids can worsen cholestasis and diarrhea, precipitating attacks. AGEB
Pregnancy – hormonal shifts (estrogens/progesterone) reduce BSEP expression; many episodes start in 1st–2nd trimester. AGEB
Oral contraceptive pills – estrogen-related reduction of bile salt export can trigger attacks in susceptible women. AGEB
Certain antibiotics (e.g., amoxicillin-clavulanate, erythromycin, sulfonamides, cefuroxime) – some drugs inhibit BSEP or alter transporter regulation. AGEB
Tetracyclines – repeatedly linked to cholestasis in case series; can impair canalicular transport. AGEB
Acetaminophen (paracetamol) – rare cholestatic patterns reported; probably via transporter dysregulation in predisposed patients. AGEB
Seasonal factors (winter/autumn predominance) – likely reflects higher infection burden and indoor exposures. AGEB
Vaccinations – rarely reported temporal associations; mechanism uncertain but immunologic signaling may transiently down-regulate transport. AGEB
Hyperthyroidism – isolated reports suggest thyroid hormones may alter bile acid handling. AGEB
Physiologic stress/illness – broad inflammatory states and cytokines reduce transporter activity (animal data support this). AGEB
Potential drug–transporter interactions (general) – BSEP inhibition by lipophilic drugs is a known path; genetics raise susceptibility. AGEB
Fasting/malnutrition – may change bile acid pool and hepatic energy balance, worsening transporter function (inferred mechanism). Gastro Journal
Alcohol binges – can impair hepatocyte membranes and transporter expression (rarely documented but biologically plausible). Gastro Journal
Surgery/anesthesia – peri-operative stress, drugs, and fasting sometimes precede attacks. Gastro Journal
Adolescent growth/hormonal changes – many first attacks occur in teens/early 20s, aligning with hormonal modulation of transporters. rarediseases.info.nih.gov
Genetic load (family history) – autosomal recessive inheritance sets the stage; triggers reveal the phenotype. NCBI
Environmental toxins – theoretical risk via transporter inhibition/oxidative stress; evidence limited. AGEB
Idiopathic (no clear trigger) – in many attacks, no cause is found even after careful review. AGEB
Symptoms
Itching (pruritus) – often severe, worse at night; due to bile acids and pruritogens in blood. AGEB
Jaundice – yellow eyes/skin from conjugated bilirubin buildup. Orpha
Dark urine – conjugated bilirubin spills into urine. Orpha
Pale/greasy stools – fat malabsorption when bile doesn’t reach the gut. AGEB
Fatigue and malaise – common during attacks, improves when attack resolves. rarediseases.info.nih.gov
Nausea and vomiting – especially during intense cholestasis. rarediseases.info.nih.gov
Loss of appetite and weight loss – due to nausea and fat malabsorption. AGEB
Abdominal discomfort – vague right-upper-quadrant heaviness is possible. Orpha
Steatorrhea (oily stools) – undigested fat because bile acids are low in the intestine. AGEB
Bruising/bleeding tendency – from vitamin K deficiency during prolonged attacks. AGEB
Irritability/sleep disturbance – intense itching disrupts sleep and mood. rarediseases.info.nih.gov
Dry skin/excoriations – due to scratching. Orpha
Hearing problems (rare) – extrahepatic features reported with ATP8B1 defects. AGEB
Pancreatitis (rare) – sporadic association, especially with ATP8B1 disease. AGEB
Gallstones (some ABCB11-related cases) – due to altered bile composition. AGEB
Diagnostic tests
Doctors diagnose BRIC by combining history of repeated attacks, typical lab patterns, normal bile ducts on imaging, liver biopsy during an attack, and genetic testing. A classic set of six criteria by Luketic & Shiffman (1999) is still widely used. PMC+2PMC+2
Physical examination
Skin and eyes check for jaundice & scratch marks – confirms visible cholestasis and pruritus damage. Orpha
Abdominal exam – liver size is usually normal; marked tenderness or big spleen suggests other diseases. jcehepatology.com
Nutritional status – weight loss or muscle wasting can appear after long attacks. AGEB
Stool color inspection – pale stools support reduced bile in the gut during attacks. AGEB
Manual/bedside tests
Pruritus severity scales – simple itch scales track improvement or worsening; helpful to judge treatment response. (Supportive measure; attacks are self-limited.) jcehepatology.com
Stool fat (qualitative spot) check – oily stain suggests fat malabsorption during attacks. AGEB
Vitamin deficiency screening (clinical) – bruising points to low vitamin K; night blindness hints at low vitamin A. AGEB
Family history & trigger diary – careful history often reveals infection, pregnancy, or drugs before attacks. AGEB
Laboratory and pathological tests
Liver panel during attack – conjugated (direct) bilirubin and alkaline phosphatase rise; **GGT is often normal or only slightly high, which is a hallmark in BRIC. jcimcr.org+1
Serum bile acids – rise early in an attack and typically precede the bilirubin rise; useful for timing and pathophysiology. AGEB
Prothrombin time/INR – may prolong if vitamin K is low; corrects with supplementation. AGEB
Viral hepatitis and autoimmune panels – help rule out other liver diseases; BRIC is a diagnosis of exclusion plus genetics. PMC
Genetic testing (ATP8B1, ABCB11; ± others) – confirms BRIC1 or BRIC2; explains mechanism (flipase defect vs. BSEP defect). NCBI+1
Fat-soluble vitamin levels (A, D, E, K) – can fall during prolonged attacks; guides supplementation. AGEB
Liver biopsy (during an attack) – shows centrilobular/canalicular cholestasis with little inflammation or duct damage. Between attacks it may be near-normal. PMC
Bile acid fractionation (specialized) – research/tertiary centers may analyze patterns supporting cholestasis and transporter dysfunction. Gastro Journal
Electrodiagnostic tests
**Electrodiagnostic studies (nerve tests) are not part of standard BRIC diagnosis. They may be used only if severe itch leads to neuropathic complaints or to rule out unrelated neuromuscular problems. Core BRIC criteria rely on labs, imaging, histology, and genetics. PMC
ECG (heart tracing) when using certain therapies – if drugs like sertraline or others are used for itch, clinicians may monitor heart rhythm as a safety step, not for diagnosis of BRIC itself. PMC
Imaging tests
Ultrasound – checks liver and gallbladder; usually normal bile ducts; rules out stones and extrahepatic obstruction. PMC
MRCP (magnetic resonance cholangiopancreatography) – normal intra- and extra-hepatic ducts are a key criterion; confirms no blockage. ERCP is rarely needed. PMC
Non-pharmacological treatments (therapies & others)
1) Gentle skin care & emollients
Description: Use fragrance-free moisturizers multiple times daily, avoid hot showers, use mild soaps, and pat dry. Keep nails short to reduce skin injury from scratching. Loose cotton clothing and cool rooms help. Purpose: Reduce skin dryness and nerve irritation that worsen itching. Mechanism: Emollients restore the skin barrier and reduce transepidermal water loss, which lowers itch signaling from dry, inflamed skin. AASLD
2) Tepid baths & menthol/cooling gels
Description: Bathe with lukewarm water; apply small amounts of menthol or cooling gels to localized areas. Purpose: Provide short-term itch relief without systemic effects. Mechanism: Cooling activates TRPM8 receptors and competes with itch signals; tepid water prevents heat-induced vasodilation that can intensify itch. AASLD
3) Sleep hygiene for nocturnal itch
Description: Fixed sleep schedule, dark cool room, short pre-bed bath, and mindfulness or relaxation audio. Purpose: Improve sleep quality, which is often disturbed by cholestatic itch. Mechanism: Reduces arousal and scratching cycles; cooler temperature lowers peripheral nerve firing that drives itch. AASLD
4) Trigger review & avoidance
Description: Review recent drugs (e.g., estrogen-containing contraceptives, some antibiotics), illnesses, alcohol, and herbal products; stop likely triggers in consultation with a clinician. Purpose: Reduce attack frequency and severity. Mechanism: Removing cholestasis-provoking exposures lowers bile acid accumulation. (General cholestasis guidance.) AASLD
5) Nutritional support during attacks
Description: Small frequent meals; consider medium-chain triglycerides; ensure calories to prevent weight loss. Purpose: Maintain energy and prevent malnutrition when appetite is poor. Mechanism: Easily absorbed fats and fractionated meals minimize fat-induced bile flow burden while meeting needs. MDPI
6) Sun/heat moderation
Description: Limit prolonged heat exposure; use fans and cool packs. Purpose: Reduce vasodilation and heat-aggravated itch. Mechanism: Cooler skin dampens peripheral sensory neuron activation. AASLD
7) Behavioral strategies (habit reversal/mindfulness)
Description: Short daily sessions to notice urge-to-scratch and substitute gentle pressure/tapping; mindfulness to reduce distress. Purpose: Lower skin injury and break itch-scratch cycles. Mechanism: Top-down cognitive control dampens central itch amplification. AASLD
8) Psychological support
Description: Brief cognitive-behavioral support when repeated attacks cause anxiety or low mood. Purpose: Improve coping and adherence. Mechanism: Treats the central component of chronic itch perception and sleep loss. AASLD
9) Photoprotection of fragile skin
Description: Use SPF and protective clothing if scratching has left excoriations. Purpose: Prevent secondary hyperpigmentation/infection. Mechanism: Minimizes UV-induced inflammation on damaged skin. AASLD
10) Antiseptic wound care for excoriations
Description: Clean minor wounds, apply simple non-fragranced antiseptic if needed, and cover. Purpose: Prevent infection. Mechanism: Reduces bacterial load and irritation. AASLD
11) Structured itch diary
Description: Record triggers, foods, drugs, stress, sleep, and severity. Purpose: Inform step-wise treatment. Mechanism: Identifies patterns that guide personalized therapy. AASLD
12) Vaccinations (hepatitis A & B, routine)
Description: Keep routine immunizations up to date, including hepatitis A/B. Purpose: Reduce risk of superimposed liver insults that might precipitate attacks. Mechanism: Prevents additional hepatic injury that could worsen cholestasis. AASLD
13) Avoid alcohol during attacks
Description: Stop alcohol when jaundiced/itching. Purpose: Protect liver function. Mechanism: Alcohol is hepatotoxic and can worsen cholestasis. AASLD
14) Review estrogen exposure
Description: Discuss alternatives to estrogen-containing contraceptives if attacks correlate with use. Purpose: Lower cholestasis risk. Mechanism: Estrogens impair bile secretion in predisposed individuals. AASLD
15) Endoscopic nasobiliary drainage (NBD)
Description: Short ERCP procedure placing a temporary small tube from the bile duct to the nose to drain bile externally for days. Purpose: Rapidly stops unbearable itch in refractory attacks. Mechanism: Physically diverts bile acids, lowering serum bile salts and itch within 24 hours in BRIC. PubMed+2Lippincott Journals+2
16) Molecular Adsorbents Recirculating System (MARS)
Description: Specialized albumin dialysis for severe, resistant pruritus; done in hospital. Purpose: Rapid symptom control when medicines fail. Mechanism: Removes protein-bound toxins (including bile acids) from blood, decreasing itch; effects can be temporary. PubMed+1
17) Short inpatient supportive care
Description: IV fluids for dehydration, anti-nausea support, and monitoring if oral intake is poor. Purpose: Stabilize during severe attacks. Mechanism: Restorative care prevents complications and allows stepped therapy. AASLD
18) Specialist dietetic review
Description: Individualized plan for calories, fat type, and fat-soluble vitamin intake. Purpose: Prevent deficiencies during long attacks. Mechanism: Optimizes absorption strategies and supplement timing around bile-acid binders. AASLD
19) Family genetic counseling
Description: Offer counseling and, where appropriate, genetic testing for ATP8B1/ABCB11 variants. Purpose: Inform recurrence risks and life planning. Mechanism: Clarifies inheritance (usually autosomal recessive). PMC
20) Structured, step-wise treatment plan
Description: Start with skin care and bile-acid sequestrant, then escalate to rifampin, opioid antagonists, SSRIs, IBAT inhibitors (specialist) or procedures when needed. Purpose: Efficient, evidence-based control of itch. Mechanism: Targets bile acids first, then central/peripheral itch pathways. AASLD+1
Drug treatments
(Important safety notes: Many of these are off-label for BRIC but widely used for cholestatic pruritus under specialist care. Doses below are typical adult starting points—always individualize, check interactions, and adjust for liver function. FDA labels are cited from accessdata.fda.gov to verify drug properties and safety.)
1) Cholestyramine (bile-acid sequestrant)
Class: Anion-exchange resin. Dose/Time: 4 g powder once daily, titrate to 4 g 2–4×/day; take separate from other medicines by ≥4 hours. Purpose: First-line for cholestatic itch. Mechanism: Binds bile acids in the gut so fewer are reabsorbed; lowers serum bile acids and itch. Side effects: Constipation, bloating; may bind other drugs and fat-soluble vitamins. Label includes pruritus from partial biliary obstruction; BRIC use is off-label. FDA Access Data+1
2) Colestipol (bile-acid sequestrant)
Class: Anion-exchange resin. Dose/Time: 2 g once or twice daily; titrate. Purpose: Alternative if cholestyramine not tolerated. Mechanism: Similar bile-acid binding to reduce enterohepatic recirculation. Side effects: Constipation, GI discomfort; may reduce absorption of some drugs/vitamins—separate dosing. FDA Access Data+1
3) Colesevelam (bile-acid sequestrant)
Class: Polymer resin (tablets or powder). Dose/Time: Typical 3.75 g/day (split dosing). Purpose: Another option when others not tolerated. Mechanism: Binds bile acids; may be easier to take. Side effects: Dyspepsia, constipation; can reduce oral contraceptive effectiveness—separate dosing. FDA Access Data+1
4) Ursodeoxycholic acid / Ursodiol
Class: Hydrophilic bile acid. Dose/Time: 13–15 mg/kg/day in divided doses with food (standard PBC dosing; BRIC is off-label). Purpose: Improve bile flow and cholestasis; sometimes shortens attacks. Mechanism: Replaces hydrophobic bile acids; cytoprotective, choleretic. Side effects: Diarrhea, rare stones. FDA Access Data+1
5) Rifampin (rifampicin)
Class: Antibiotic; PXR agonist. Dose/Time: Start 150 mg daily; increase to 300 mg twice daily if needed. Purpose: Second-line for pruritus not controlled by sequestrants. Mechanism: Induces liver enzymes and transporters that metabolize/bypass pruritogens (including bile acids). Side effects: Hepatotoxicity, orange secretions, many drug interactions (warfarin, OCPs, opioids). Monitor LFTs. FDA Access Data+1
6) Naltrexone (oral)
Class: Opioid receptor antagonist. Dose/Time: Begin 12.5–25 mg daily; titrate to 50 mg daily. Purpose: Third-line for cholestatic itch when others fail. Mechanism: Counters endogenous opioid tone that amplifies itch signaling in cholestasis. Side effects: Can precipitate withdrawal in opioid-dependent patients; nausea, headache; check LFTs. FDA Access Data+1
7) Naloxone (IV, inpatient use)
Class: Opioid antagonist. Dose/Time: Short IV infusions in monitored settings for acute severe itch (specialist use). Purpose: Diagnostic/temporary antipruritic in refractory cases. Mechanism: Rapidly blocks opioid receptors; effect is brief. Side effects: Can trigger opioid withdrawal; monitor. FDA Access Data+1
8) Sertraline
Class: SSRI antidepressant. Dose/Time: Start 25–50 mg daily; titrate. Purpose: Adjunct when mood/sleep are affected and itch persists. Mechanism: Modulates central itch perception and comorbid anxiety/depression. Side effects: GI upset, sexual dysfunction; adjust dose in liver impairment. FDA Access Data+1
9) Hydroxyzine
Class: Antihistamine/anxiolytic. Dose/Time: 25 mg at night or 25 mg 3–4×/day if needed. Purpose: Sedative relief of nighttime scratching; modest benefit for cholestatic itch. Mechanism: Antihistamine with central sedation reduces scratching behavior. Side effects: Drowsiness, dry mouth; caution in elderly. FDA Access Data+1
10) Doxepin 5% cream (Zonalon)
Class: Topical tricyclic with antihistamine effects. Dose/Time: Thin layer to itchy areas up to 4×/day for short courses. Purpose: Localized adjunct for focal itch. Mechanism: Local antihistaminic/anticholinergic effects reduce itch signaling in skin. Side effects: Local irritation, drowsiness if large areas used. FDA Access Data+1
11) Gabapentin
Class: Neuromodulator. Dose/Time: 100–300 mg at night; titrate. Purpose: Off-label for chronic itch with neuropathic features and poor sleep. Mechanism: Reduces neuronal excitability in central itch pathways. Side effects: Dizziness, somnolence; dose-adjust in renal impairment. FDA Access Data
12) Pregabalin
Class: Neuromodulator. Dose/Time: 25–75 mg at night; titrate. Purpose: Alternative to gabapentin for refractory nocturnal itch and anxiety. Mechanism: Similar central neuromodulation. Side effects: Drowsiness, edema; taper to stop. FDA Access Data+1
13) Ondansetron (for nausea during attacks)
Class: 5-HT3 antagonist. Dose/Time: 4–8 mg every 8–12 h as needed. Purpose: Control nausea/vomiting to maintain nutrition and meds. Mechanism: Blocks serotonin receptors in gut/brain. Side effects: Headache, constipation; dose caution in severe hepatic impairment. FDA Access Data+1
14) Vitamin K1 (phytonadione) (if prolonged jaundice with coagulopathy)
Class: Fat-soluble vitamin (Rx). Dose/Time: Individualized oral/IV dosing per INR. Purpose: Corrects prolonged prothrombin time due to fat-soluble vitamin malabsorption. Mechanism: Restores clotting factor carboxylation. Side effects: Over-correction can increase thrombosis risk; monitor INR. FDA Access Data+1
15) Ursodiol (Actigall brand example)
Class: Hydrophilic bile acid (repeat for labeling variety). Note: Same mechanisms/dose as #4; different brand/formulations exist. Purpose/Mechanism: Choleretic and cytoprotective effects; sometimes used continuously in recurrent disease. Safety: See label. FDA Access Data
16) Odevixibat (BYLVAY) — IBAT inhibitor
Class: Ileal bile acid transporter inhibitor. Dose/Time: Per label for PFIC/ALGS (e.g., oral pellets/capsules; pediatric dosing). Purpose: In specialist centers, may be considered off-label for similar bile-acid–driven itch in BRIC. Mechanism: Blocks bile acid re-uptake in ileum → lowers serum bile acids and itch. Side effects: Diarrhea, abdominal pain; monitor growth in children. FDA Access Data+1
17) Maralixibat (LIVMARLI) — IBAT inhibitor
Class: Ileal bile acid transporter inhibitor. Dose/Time: Per label for ALGS and PFIC; age/weight-based titration. Purpose: Specialist consideration off-label for BRIC-like pruritus. Mechanism: Reduces enterohepatic bile acid circulation. Side effects: Diarrhea, abdominal pain; time doses away from bile-acid binders. FDA Access Data+1
18) Sertraline (capsule formulation label)
Class: SSRI. Note: Re-emphasizing hepatic dose caution and psychiatric benefits for persistent itch-distress. Purpose: Adjunct for quality of life. Mechanism/Side effects: As above. FDA Access Data
19) Hydroxyzine (Vistaril label)
Class: Antihistamine. Note: Night-time symptomatic aid; reinforce anticholinergic cautions. Mechanism/Side effects: As above. FDA Access Data
20) Rifampin (alternative label references)
Class: Enzyme inducer antibiotic. Note: Emphasize strong drug–drug interactions (warfarin, OCPs, many others) and LFT monitoring. Mechanism/Side effects: As above. FDA Access Data
Dietary molecular supplements
(Use with clinician guidance, especially if you’re also taking bile-acid binders that can block absorption. Space supplements 4+ hours away from cholestyramine/colesevelam/colestipol.)
1) Vitamin A (retinol/β-carotene)
Description (≈150 words): In prolonged cholestasis, fat-soluble vitamins may be poorly absorbed, causing night blindness and dry eyes/skin. Supplementation corrects deficiency and supports epithelial health. Dosing is individualized; avoid high doses without testing because vitamin A can be toxic and accumulate in cholestasis. Mechanism: Restores retinoid-dependent vision and epithelial integrity; deficiency is common when bile acids in the gut are low. AASLD
2) Vitamin D3 (cholecalciferol)
Description: Cholestasis impairs vitamin D absorption, risking bone loss. Supplementation (often higher doses with monitoring) helps maintain calcium balance and bone strength. Mechanism: Restores vitamin D endocrine function for calcium absorption and bone mineralization. AASLD
3) Vitamin E (α-tocopherol)
Description: Antioxidant vitamin E can be low in chronic cholestasis, causing neuropathy or myopathy in severe cases. Water-miscible preparations (e.g., TPGS) improve absorption. Mechanism: Replenishes antioxidant defenses and neuronal membrane protection. AASLD
4) Vitamin K1 (phytonadione) (nutritional oral forms)
Description: Oral vitamin K supports clotting factor activation when INR is borderline due to fat-soluble vitamin loss. Use labs to guide dose and avoid over-replacement. Mechanism: Restores γ-carboxylation of clotting factors II, VII, IX, X. FDA Access Data
5) Medium-chain triglyceride (MCT) oil
Description: Provides energy that does not depend on bile for absorption. Add small amounts to meals or shakes during attacks. Mechanism: MCTs are absorbed directly via the portal vein, bypassing bile-dependent micelles. MDPI
6) Phosphatidylcholine (lecithin)
Description: A membrane phospholipid sometimes used to support bile composition; evidence is limited but it’s generally safe in food amounts. Mechanism: May protect canalicular membranes and support micelle formation. PMC
7) S-adenosyl-L-methionine (SAMe)
Description: A methyl donor with cholestasis literature mainly in other liver diseases; may support hepatocellular antioxidant capacity. Mechanism: Replenishes hepatic glutathione via transmethylation/transsulfuration. PMC
8) Omega-3 fatty acids (EPA/DHA)
Description: Can support anti-inflammatory balance and triglyceride control; choose purified products. Mechanism: Compete with arachidonic acid pathways and may reduce pruritogen sensitization. PMC
9) Water-miscible multivitamin for cholestasis
Description: Specialized formulations (fat-soluble vitamins in absorbable forms) used in cholestatic disorders to prevent deficiencies. Mechanism: Improves bioavailability despite low bile. AASLD
10) Probiotic foods (e.g., yogurt with live cultures)
Description: Maintain gut barrier function and regularity during binder use; direct antipruritic effect is unproven. Mechanism: Modulates microbiome and bile acid deconjugation pathways. PMC
Immunity-booster / regenerative / stem-cell drugs
There are no FDA-approved “immunity-booster,” regenerative, or stem-cell drugs for BRIC. Management remains symptomatic (itch control, nutrition, vitamins) and, when needed, procedures like NBD or albumin dialysis. Experimental cell or gene therapies are not standard of care in BRIC as of October 22, 2025. The most “disease-adjacent” advances are IBAT inhibitors (maralixibat, odevixibat) for PFIC/ALGS, which share bile-acid pathophysiology—these may be considered off-label in highly selected BRIC cases by specialists. Always discuss clinical trials with a hepatology center. FDA Access Data+1
(If your goal is immune protection, focus on vaccination, nutrition, and trigger avoidance rather than unproven “immune boosters.”) AASLD
Procedures/surgeries
1) Endoscopic nasobiliary drainage (NBD)
Procedure: ERCP places a soft tube from bile duct to nose for temporary external drainage. Why: For rapid relief of unbearable cholestatic itch; often works within 24 hours in BRIC. PubMed+1
2) Temporary biliary stenting (endoscopic)
Procedure: A small plastic stent is left in the common bile duct for days–weeks. Why: Alternative to NBD in specialized centers to enhance bile drainage and relieve pruritus. PMC
3) Molecular Adsorbents Recirculating System (MARS) albumin dialysis
Procedure: Extracorporeal circuit that cleans protein-bound toxins. Why: Short-term rescue for refractory pruritus when medications fail. PubMed+1
4) Therapeutic plasma exchange (plasmapheresis)
Procedure: Removes plasma containing pruritogens and replaces with albumin/FFP. Why: Bridge therapy for severe refractory itch where available. (Supportive in cholestatic pruritus literature.) PMC
5) Partial external biliary diversion (rare)
Procedure: Surgical diversion of bile from enterohepatic circulation (used in PFIC). Why: Very rarely considered in extreme, refractory, BRIC-like cases in expert centers; not routine. PMC
Preventions
Keep skin moisturized and cool daily to lower baseline itch. AASLD
Avoid alcohol, especially during attacks. AASLD
Review medicines with clinicians; avoid likely cholestatic triggers when possible (e.g., estrogens). AASLD
Keep hepatitis A/B vaccinations current. AASLD
Maintain healthy weight and balanced diet with adequate protein and vitamins. MDPI
At first sign of relapse, start skin care and contact your doctor early to escalate therapy. AASLD
Space medicines from bile-acid binders by ≥4 hours to avoid absorption problems. FDA Access Data
Limit heat exposure; choose breathable clothing. AASLD
Plan pregnancy/contraception with hepatology/OBGYN if you have estrogen-related flares. AASLD
Use an itch diary to learn personal triggers and treatment responses. AASLD
When to see doctors (red flags)
See a doctor immediately if you have sudden severe jaundice, intense itch with sleep loss, fever/chills, confusion, easy bruising/bleeding, very dark urine with pale stools, new abdominal pain, or you’re pregnant and develop cholestatic symptoms. These signs need urgent tests to exclude obstruction, infection, or other liver diseases and to start therapies such as bile-acid binders, rifampin, or procedures like NBD if needed. AASLD+1
What to eat and what to avoid
Eat small, frequent meals; heavy meals can worsen nausea. MDPI
Prefer lean proteins (fish, poultry, legumes) to maintain strength. MDPI
Use MCT oil in moderation for calories during attacks. MDPI
Choose whole grains, fruits, vegetables for fiber and micronutrients. MDPI
If prescribed, take fat-soluble vitamins in absorbable forms; time them away from bile-acid binders. AASLD+1
Limit very fatty, fried foods during severe cholestasis; they may aggravate symptoms. MDPI
Avoid alcohol during and shortly after attacks. AASLD
Stay hydrated; sip fluids when nauseated. MDPI
Be cautious with herbal supplements (e.g., kava, comfrey) that may harm the liver. AASLD
If weight loss persists, ask for a dietitian-guided plan or temporary oral nutrition supplements. AASLD
Frequently asked questions
1) Is Summerskill-Walshe-Tygstrup syndrome the same as BRIC?
Yes. It’s another name for Benign Recurrent Intrahepatic Cholestasis. PubMed
2) What causes it?
Inherited changes in ATP8B1 (BRIC1) or ABCB11 (BRIC2) that disrupt bile movement in liver cells. PMC
3) How is it diagnosed?
Pattern of recurrent cholestasis, no duct blockage on imaging, high bile acids, and often gene testing. PubMed
4) Will it damage my liver long-term?
Usually not, but attacks can be very distressing; rare cases can be complicated. Follow-up is important. PubMed
5) What is the main symptom?
Severe itching and jaundice during attacks. MDPI
6) What can I do at home first?
Gentle skin care, cool environment, moisturizers, tepid baths, and early contact with your clinician. AASLD
7) Which medicine is usually started first?
A bile-acid sequestrant like cholestyramine; space it from other meds by ≥4 hours. FDA Access Data
8) What if cholestyramine is not enough?
Doctors may add rifampin or naltrexone, and sometimes sertraline for distress/sleep. FDA Access Data+2FDA Access Data+2
9) Are there quick procedures if medicines fail?
Yes—nasobiliary drainage or albumin dialysis (MARS) can give rapid relief. PubMed+1
10) Are new drugs available?
IBAT inhibitors (maralixibat, odevixibat) are FDA-approved for ALGS/PFIC itch and are being used in specialized centers; BRIC use is off-label. FDA Access Data+1
11) Can I take these drugs if I’m on many other medicines?
Some (like rifampin) have major interactions. Always review your full list with your clinician/pharmacist. FDA Access Data
12) Do I need vitamins?
Often yes, especially A, D, E, K, overseen by your clinician, because cholestasis reduces absorption. AASLD
13) What about pregnancy or birth control?
Discuss non-estrogen options if your symptoms worsen with estrogens. Close obstetric-hepatology care is needed. AASLD
14) When should I go to the hospital?
If you have severe itch with no sleep, fever, confusion, bleeding, or new severe pain, seek urgent care. AASLD
15) Can this be cured?
There’s no permanent cure yet, but most people do well with episodic treatment and expert follow-up. PubMed
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The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members
Last Updated: October 21, 2025.
