Chronic Enteropathy Associated with SLCO2A1 Gene (CEAS)

Chronic enteropathy associated with SLCO2A1 gene (CEAS) is a rare inherited bowel disease in which many long-lasting small ulcers form in the small intestine, especially in the ileum, and do not heal well. These ulcers slowly bleed and leak protein, so patients often have long-term anemia (low red blood cells) and low blood protein, which can cause swelling of the legs and face.

Chronic enteropathy associated with SLCO2A1 gene (often shortened to CEAS) is a very rare genetic bowel disease. It happens when both copies of the SLCO2A1 gene have harmful changes (autosomal recessive inheritance). This gene makes a “prostaglandin transporter” protein that helps move prostaglandins (hormone-like molecules) into cells so they can be broken down.[1] When the transporter does not work, prostaglandins build up in the gut wall and drive long-lasting inflammation.[2] This leads to many small, shallow ulcers in the small intestine, especially the ileum, which may look like ulcers caused by long-term NSAID use, even though patients usually do not take these pain medicines.[3] The disease often causes chronic blood loss and protein loss, so people may develop anemia, low blood protein, leg swelling, tiredness, and abdominal pain from childhood or young adulthood.[1][4] Because CEAS is rare and looks similar to Crohn’s disease, it is often misdiagnosed, and diagnosis needs a mix of endoscopy, imaging, and genetic testing for SLCO2A1 mutations.[1][5]

This disease is caused by harmful changes (mutations) in a gene called SLCO2A1, which gives the body instructions to make a “prostaglandin transporter” protein. Prostaglandins are small chemical messengers that protect the gut lining, control blood flow, and help ulcers heal. When the SLCO2A1 protein does not work properly, the balance of prostaglandins around the intestine becomes abnormal, and the bowel wall becomes fragile and more likely to form chronic ulcers.

CEAS is usually inherited in an autosomal recessive way. This means a person becomes sick when they receive one faulty copy of the gene from each parent, who are usually healthy carriers. The condition often starts in childhood, the teenage years, or young adulthood and can look like Crohn’s disease, but it is a different disease with a different genetic cause and different response to medicines.

Other names

This condition has been described in the past with several different names. The oldest name is “chronic nonspecific multiple ulcers of the small intestine (CNSU)”, used before the gene cause was known. Later, it has also been called “SLCO2A1-related chronic enteropathy,” “SLCO2A1-related small intestinal ulcers,” or simply “CEAS.” All of these names describe the same disease: long-lasting small-bowel ulcers caused by mutations in the SLCO2A1 prostaglandin transporter gene.

Some patients with SLCO2A1 mutations also have a bone and skin condition called primary hypertrophic osteoarthropathy (PHO), with thickened skin on the scalp and face, finger clubbing, and bone changes. In these people, the disease may be described as “SLCO2A1-related enteropathy with hypertrophic osteoarthropathy.”

Types

1. Classic small-intestine–predominant CEAS
   This type mainly affects the ileum and other parts of the small intestine. Patients have many shallow but long ulcers, often arranged in a line around the bowel. They usually have iron-deficiency anemia and low albumin but may have only mild abdominal pain.

2. Crohn-like CEAS with colonic involvement
   Some patients show strictures and ulcers in the small intestine that look similar to Crohn’s disease, and sometimes the colon can also be affected. However, genetic testing reveals SLCO2A1 mutations, and usual Crohn’s medicines like strong immunosuppressants or biologics often do not work well.

3. Pediatric very-early onset CEAS
   In some children, symptoms start very early in life, even in primary school age. These children can have poor growth, anemia, and low protein and may be misdiagnosed as having early-onset inflammatory bowel disease until genetic testing confirms SLCO2A1-related enteropathy.

4. Adult-onset CEAS
   Other patients develop symptoms in their 20s, 30s, or later. They may have a long history of mild anemia and vague abdominal discomfort before the correct diagnosis is made, sometimes after many years of repeated tests and even bowel surgery.

5. CEAS with primary hypertrophic osteoarthropathy (PHO) overlap
   A group of patients with SLCO2A1 mutations show both bowel ulcers and bone/skin changes such as clubbing, thickened skin folds, and painful swelling of long bones. In these people, the same gene defect seems to cause both the gut disease and the bone-joint syndrome.

6. Mild or subclinical CEAS
   Some individuals with SLCO2A1 mutations may have few symptoms, such as mild anemia only, and small ulcers may be found only when capsule endoscopy is done for another reason. These milder cases show that the disease has a wide range of severity.

Causes

The only known direct cause of CEAS is harmful mutations in the SLCO2A1 gene. The other points below describe how this gene problem and related factors may influence when the disease starts and how severe it becomes.

1. Biallelic SLCO2A1 loss-of-function mutations
   CEAS happens when both copies of the SLCO2A1 gene carry damaging changes (mutations) that stop or strongly reduce the function of the prostaglandin transporter protein. Without a working transporter, prostaglandin handling in the gut wall is disturbed and chronic ulcers form.

2. Compound heterozygous variants
   Many patients inherit two different faulty variants in SLCO2A1, one from each parent (compound heterozygosity). Each variant alone may be mild, but together they reduce transporter function enough to cause disease.

3. Autosomal recessive inheritance and carrier parents
   Parents usually carry one faulty copy and one normal copy of SLCO2A1 and are healthy. When two carriers have a child, there is a one in four chance that the child will inherit both faulty copies and develop CEAS. Consanguineous (related) parents increase this risk.

4. Abnormal prostaglandin E2 (PGE₂) transport
   SLCO2A1 belongs to the organic anion transporting polypeptide (OATP) family and normally brings prostaglandins such as PGE₂ into cells for breakdown. When this transporter is defective, prostaglandin levels and location in the bowel wall change, which may reduce local mucosal protection and healing.

5. Impaired mucosal defense and ulcer healing
   Prostaglandins help keep the intestinal lining moist, maintain blood flow, and support repair after injury. If their handling is disturbed due to SLCO2A1 mutations, the small-bowel lining becomes more vulnerable, so tiny injuries turn into persistent ulcers instead of healing normally.

6. Chronic protein-losing enteropathy
   Ongoing leakage of protein through the damaged bowel wall leads to low albumin and low total protein in the blood. This in turn causes swelling (edema), fatigue, and poor wound healing and can worsen the disease cycle by weakening the body’s ability to repair the gut.

7. Long-term iron loss from slow bleeding ulcers
   Small but constant blood loss from chronic small-bowel ulcers causes iron-deficiency anemia. The body’s low iron stores make patients tired and short of breath and can further reduce tissue oxygen and healing in the gut wall.

8. Female predominance in many series
   Some patient series show more affected females than males, suggesting that sex-related biological or hormonal factors may influence disease expression, even though the basic inheritance is autosomal recessive.

9. Ethnic and geographic background
   CEAS was first described in Japan and has been reported most often in East Asian populations, though cases are now reported from Europe and South Asia. This pattern suggests that certain SLCO2A1 mutations are more common in some ethnic groups.

10. Overlap with primary hypertrophic osteoarthropathy (PHO)
    The same SLCO2A1 gene mutations can cause PHO, and some people with CEAS also have PHO features. These bone and skin changes may reflect systemic effects of abnormal prostaglandin handling and can coexist with or even precede the bowel disease.

11. Possible epigenetic regulation of SLCO2A1 expression
    Studies in inflammatory bowel disease suggest that DNA methylation and other epigenetic changes can reduce SLCO2A1 expression in intestinal tissue. Similar mechanisms may change the severity of CEAS in people with mutations, although this is still being studied.

12. Environmental or infectious triggers of mucosal injury
    Everyday infections or non-specific intestinal inflammation may damage the gut lining. In healthy people, these injuries heal, but in someone with SLCO2A1 mutations and poor prostaglandin-mediated protection, they may start a chain of chronic ulcer formation.

13. Use of non-steroidal anti-inflammatory drugs (NSAIDs) as a worsening factor
    By definition, classic CEAS is not caused by NSAIDs, but NSAID use can further block prostaglandin production and may worsen existing ulcers or confuse the diagnosis because NSAID-enteropathy can look similar on endoscopy.

14. Nutritional deficiency and low protein intake
    Poor appetite, nausea, and diarrhea may reduce food intake, while protein loss through the gut increases needs. Malnutrition and low protein can delay healing of the ulcers and contribute to a vicious circle of ongoing disease.

15. Microbiome changes in the small intestine (dysbiosis)
    Although research is still limited, changes in the types and balance of bacteria in the small intestine may influence inflammation and healing in CEAS, as in other chronic intestinal diseases. This is an area of active study.

16. Repeated mechanical stress and strictures
    Narrowed segments (strictures) can increase pressure and mechanical stress on the bowel wall upstream, making new ulcers more likely to form and older ones harder to heal.

17. Delayed diagnosis and inappropriate treatments
    Because CEAS can look like Crohn’s disease, patients may receive immunosuppressant or biologic medicines that do not fix the basic gene problem. Delayed recognition allows ulcers and strictures to slowly worsen and may lead to avoidable surgeries.

18. Coexisting inflammatory conditions
    Some patients may also have other inflammatory or autoimmune disorders. While these are not direct causes of CEAS, they can add extra inflammation and make symptoms more severe.

19. Other genetic modifiers
    Variants in genes that control inflammation, barrier function, or prostaglandin metabolism may change the age at onset or the severity of disease in people who already have SLCO2A1 mutations, though these modifiers are not fully understood.

20. Low blood flow or oxygen to the gut wall
    Severe anemia, dehydration, or low blood pressure can reduce blood flow to the intestine, which can further damage already fragile mucosa and slow ulcer healing in CEAS patients.

Symptoms

1. Chronic iron-deficiency anemia
   The most common symptom is long-term anemia caused by ongoing slow blood loss from small-bowel ulcers. Patients often feel tired, weak, dizzy, or short of breath, and lab tests show low hemoglobin and low iron stores.

2. Black or dark stools and hidden blood in stool
   Some patients notice dark, tarry stools (melena), but many have no visible bleeding. Tests often show hidden (occult) blood in the stool, reflecting chronic bleeding from small-intestinal ulcers.

3. Low blood protein and swelling (edema)
   Loss of protein from the damaged bowel wall causes low blood albumin. This leads to swelling of the legs, feet, hands, or face and sometimes fluid in the abdomen (ascites). Patients may look puffy even if they are underweight.

4. Abdominal pain and discomfort
   Many patients have vague, cramping pain around the belly button or lower abdomen. The pain may worsen after meals or during flares when ulcers are active or when strictures slow the passage of food.

5. Bloating and abdominal distension
   Gas build-up and partial obstruction at strictured segments can cause bloating and a visibly swollen abdomen. Patients may feel full or uncomfortable even after small meals.

6. Diarrhea or loose stools
   Some patients pass loose stools because inflamed, ulcerated small intestine cannot absorb fluid and nutrients properly. In others, stools may be normal, so diarrhea is not always present.

7. Weight loss and poor appetite
   Long-term pain, nausea, and fear of eating, together with poor absorption and protein loss, lead to weight loss. Children may fall off their growth curve, and adults may lose weight slowly over months or years.

8. Growth delay in children and teenagers
   In pediatric-onset CEAS, chronic anemia, malnutrition, and inflammation may delay height gain and puberty. This is an important clue that the intestinal disease is serious and long-lasting.

9. Nausea, vomiting, and early fullness
   When strictures narrow the small intestine, food may not pass easily, causing nausea, vomiting, and a feeling of being full very quickly. These symptoms can worsen weight loss and malnutrition.

10. Acute bowel obstruction episodes
    In severe cases, a tight stricture can cause a sudden blockage of the small intestine. Patients may have strong cramping pain, no gas or stool, and vomiting, and may need urgent hospital care or surgery.

11. Fatigue and reduced exercise tolerance
    Chronic anemia, low protein, and poor nutrition make patients feel very tired and unable to keep up with normal activities or sports, which strongly affects quality of life.

12. Digital clubbing (bulb-shaped fingertips)
    Some patients develop widening and rounding of the fingertips and toenails, known as clubbing. This is often linked to associated primary hypertrophic osteoarthropathy due to SLCO2A1 mutations.

13. Bone and joint pain
    In overlap cases, painful swelling of long bones, joint discomfort, and thickened skin folds (pachydermia) may occur, reflecting extra-intestinal effects of the same gene defect.

14. Recurrent need for transfusions or iron therapy
    Because anemia tends to come back, many patients require repeated iron infusions or blood transfusions. This pattern of recurrent anemia despite treatment should prompt doctors to search for CEAS or other chronic enteropathies.

15. Psychological stress and reduced quality of life
    Long-term illness, repeated hospital visits, and fear of flare-ups can lead to anxiety, low mood, and difficulty with school, work, or social life, especially in young people.

Diagnostic tests

Doctors use many tests together to diagnose CEAS and to tell it apart from Crohn’s disease and other causes of small-bowel ulcers. These tests can be grouped into physical exam, manual tests, lab/pathological tests, electrodiagnostic tests, and imaging tests.

Physical exam tests

1. General physical examination
   The doctor looks at the patient’s overall appearance, checking for pale skin (anemia), swelling of the legs or face (edema), weight loss, and signs of poor nutrition. These findings suggest chronic blood and protein loss from the intestine.

2. Abdominal examination
   The abdomen is carefully inspected, listened to with a stethoscope, and gently pressed to look for tenderness, masses, and swelling. Mild or moderate tenderness and sometimes distension may hint at chronic small-bowel disease or partial obstruction.

3. Growth and development assessment in children
   In children and teenagers, the doctor measures height, weight, and puberty stage and plots them on growth charts. Falling off the normal curve is a strong sign of chronic illness such as CEAS or other long-standing intestinal disorders.

Manual tests

4. Edema “pitting” test
   The doctor presses a finger gently on the shin or ankle for several seconds and then releases it. If a dent (pit) remains, it suggests fluid build-up due to low blood protein from protein-losing enteropathy.

5. Digital rectal examination with stool guiac card
   A gloved finger is used to feel the rectum and obtain a small stool sample, which is then placed on a card to test for hidden blood. Positivity suggests ongoing bleeding somewhere in the gastrointestinal tract, including possibly from small-bowel ulcers.

6. Manual examination of fingers and joints
   The doctor checks the fingers and toes for clubbing and palpates long bones and joints for tenderness and thickening, which may indicate associated hypertrophic osteoarthropathy in SLCO2A1-related disease.

Lab and pathological tests

7. Complete blood count (CBC)
   A CBC measures hemoglobin, red blood cells, white blood cells, and platelets. In CEAS, it usually shows microcytic iron-deficiency anemia and may show other changes depending on bleeding and nutrition.

8. Iron studies and ferritin
   Blood tests for serum iron, ferritin, and transferrin saturation confirm iron deficiency and help guide iron replacement. Very low ferritin is typical in long-term bleeding from small-intestinal ulcers.

9. Serum albumin, total protein, and liver function tests
   These tests show low albumin and sometimes low total protein in CEAS, reflecting protein-losing enteropathy. Liver tests help to exclude liver disease as another cause of low albumin.

10. Inflammatory markers (CRP, ESR) and related tests
    Blood tests such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) assess inflammation. They may be normal or mildly raised in CEAS, which can help distinguish it from strongly inflammatory diseases like Crohn’s disease.

11. Stool tests for occult blood and protein loss
    Repeated stool tests for hidden blood document ongoing bleeding. Measurement of stool alpha-1 antitrypsin or other markers can show protein-losing enteropathy, supporting the diagnosis of a chronic small-bowel disease.

12. Fecal calprotectin
    This stool marker reflects intestinal inflammation. Levels may be raised in CEAS but are not specific, so they are mainly useful to show that the gut is inflamed and to decide on further imaging and endoscopy.

13. Genetic testing for SLCO2A1 mutations
    Sequencing of the SLCO2A1 gene, either alone or as part of a panel for monogenic intestinal diseases, can find biallelic loss-of-function variants. A clear genetic result, combined with the clinical picture, confirms the diagnosis of CEAS.

14. Small-intestinal biopsies with histology
    During enteroscopy, small tissue samples are taken from the ulcer edges. Under the microscope, they usually show non-specific chronic inflammation without the granulomas seen in Crohn’s disease, helping to distinguish CEAS from other causes of ulcers.

15. Research tests for prostaglandin pathways
    In research settings, scientists may measure prostaglandin levels or study SLCO2A1 expression in tissue to better understand how the transporter defect leads to disease. These tests are not routine but support the proposed mechanism.

Electrodiagnostic tests

16. Electrocardiogram (ECG)
    An ECG records the heart’s electrical activity. In CEAS it is used not to diagnose the bowel disease itself, but to check for heart strain or rhythm problems caused by severe, long-lasting anemia and to ensure the patient is safe for anesthesia or surgery.

17. Nerve conduction studies in severe malnutrition
    If a patient has signs of nerve damage, such as numbness or weakness, nerve conduction tests can be done. These studies check electrical signals in nerves and may show damage from vitamin deficiencies related to chronic malabsorption.

Imaging tests

18. Capsule endoscopy
    The patient swallows a small camera capsule that takes thousands of pictures as it travels through the small intestine. In CEAS it shows multiple small, shallow ulcers and sometimes strictures, which are very typical findings for this disease.

19. Double-balloon enteroscopy
    This special endoscopy uses balloons to pull the intestine over the scope so the doctor can see deep into the small bowel, take biopsies, and sometimes treat strictures. It confirms the presence and pattern of ulcers and allows tissue sampling.

20. CT or MR enterography
    CT enterography and MR enterography use contrast images to visualize the small intestine. In CEAS they may show segmental wall thickening, ulcers, and strictures and help assess how long the affected segments are, plan treatment, and monitor for complications like obstruction.

Non-pharmacological treatments (therapies and others)

1. Individualized bowel-friendly diet – A soft, low-fat, low-fiber diet reduces friction and irritation on inflamed small-bowel ulcers.[1] The aim is to keep nutrition good while avoiding foods that worsen pain, bloating, or diarrhea. This supportive approach can lessen symptoms and help healing together with medical treatment.[1]

2. Exclusive or partial enteral nutrition – Some patients benefit from liquid nutritional formulas taken as the main or partial food source.[2] These drinks are easy to absorb, give balanced calories, vitamins, and minerals, and can reduce mechanical and chemical stress on the ulcers, similar to enteral nutrition used in Crohn’s disease.[2]

3. Correction of iron-deficiency anemia – CEAS often causes chronic blood loss, so intravenous or oral iron and nutritional support are important non-drug strategies.[3] Correcting anemia improves fatigue, exercise tolerance, and quality of life while disease-specific treatment works in the background.[3]

4. Protein-rich nutrition for hypoproteinemia – Because protein leaks from the injured intestine, high-protein meals, oral protein supplements, or, in severe cases, intravenous albumin help restore normal blood protein levels.[4] This reduces leg and facial swelling and supports wound and ulcer healing in the gut.[4]

5. Careful fluid and electrolyte management – Chronic diarrhea and protein loss can disturb salt and fluid balance.[1] Oral rehydration solutions or hospital intravenous fluids keep blood pressure stable, protect kidney function, and reduce dizziness and weakness during disease flares.[1]

6. Avoidance of NSAIDs and gut-toxic drugs – Since CEAS ulcers resemble NSAID-induced enteropathy, doctors usually advise strict avoidance of NSAIDs and other medicines known to injure the small bowel.[3] This prevention step helps stop further ulcer formation and bleeding on top of the genetic problem.[3]

7. Smoking cessation and alcohol moderation – Smoking and heavy alcohol use can worsen intestinal inflammation and delay mucosal healing.[2] Stopping smoking and limiting alcohol are simple but powerful lifestyle changes that support better long-term bowel health in CEAS.[2]

8. Stress management and psychological support – Chronic abdominal pain, anemia, and repeated hospital visits can cause anxiety, low mood, or school/work problems.[4] Counseling, stress-reduction techniques, and sometimes formal psychotherapy help patients cope better and stay engaged with long-term care plans.[4]

9. Gentle physical activity – Light exercise like walking or stretching can improve energy, muscle strength, and mood.[2] Activity plans should be adjusted to the patient’s anemia and nutritional status, avoiding over-exertion but still keeping the body moving during stable phases.[2]

10. Vaccination and infection prevention – Because many CEAS patients later receive immune-modifying drugs, completing age-appropriate vaccines (for example influenza, pneumococcal, hepatitis B) is important.[5] This reduces infection risk when the immune system is deliberately suppressed by treatment.[5]

11. Regular gastroenterology follow-up – Scheduled visits allow monitoring of weight, blood counts, proteins, and imaging or endoscopy when needed.[1] Early detection of new strictures, severe bleeding, or malnutrition lets the team react quickly with diet changes, medicines, or surgery.[1]

12. Family genetic counseling – CEAS is autosomal recessive, so brothers and sisters may also carry or have the disease.[1][5] Genetic counseling explains inheritance, testing options, and future pregnancy risks in very simple language for the whole family.[1][5]

13. School and workplace accommodations – Young patients often need flexible schedules, toilet access, or reduced physical activity at school or work.[4] Written plans and clear communication lower stress and help them continue education or employment despite chronic symptoms.[4]

14. Nutritional counseling by a dietitian – A dietitian experienced in inflammatory bowel diseases can build a personal plan that respects culture, taste, and budget.[2] They help choose safe foods, plan small frequent meals, and avoid nutrient gaps while protecting the small intestine.[2]

15. Oral or parenteral vitamin supplementation – Chronic small-bowel disease can lead to low levels of vitamin B12, folate, fat-soluble vitamins, and others.[3] Replacing these with oral or injectable vitamins supports blood formation, nerve health, and immunity.[3]

16. Bone-health support (calcium and lifestyle) – Long-term inflammation and later steroid use can weaken bones.[5] Adequate calcium and vitamin D intake, weight-bearing exercise when possible, and sunlight exposure (if safe) help protect bone density in growing children and adults.[5]

17. Symptom diaries and self-monitoring – Keeping a simple diary of pain, stool frequency, weight, and diet helps patients notice patterns and share clear information with doctors.[4] This can guide treatment adjustments and make clinic visits more effective.[4]

18. Social and peer support groups – Because CEAS is rare, meeting others with similar chronic bowel diseases (online or in person) can reduce loneliness.[4] Shared experiences often provide practical tips about diet, travel, and coping with long-term illness.[4]

19. Oral rehydration and anti-dehydration planning for flares – Patients are taught how to use oral rehydration solutions early during diarrhea flares.[1] This simple home measure may prevent hospital admissions and keep circulation stable until medical care is accessed.[1]

20. Multidisciplinary care team – Ideal care involves gastroenterologists, surgeons, dietitians, nurses, and mental-health professionals.[1][5] Working together, they decide the right mix of diet, monitoring, medicines, and surgery for each stage of CEAS.[1][5]

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Drug treatments

Important: No medicine is currently approved specifically for CEAS. Treatments are adapted from inflammatory bowel disease and case reports. Doses below are general examples from FDA-approved labels for similar conditions; the exact drug and dose must always be chosen by a specialist doctor for each patient.[1][2]

1. Budesonide (e.g., ENTOCORT EC, UCERIS) – Budesonide is a steroid that mainly acts inside the gut with less whole-body exposure.[3] It is often used for mild-to-moderate ileal and right-colon inflammation once daily (for example 9 mg/day in Crohn’s disease). It reduces immune activity in the bowel wall and may help ulcers heal, but long-term use can still cause steroid side effects like weight gain or bone thinning.[3]

2. Systemic corticosteroids (prednisone / prednisolone) – Oral corticosteroids are strong anti-inflammatory drugs often used for short periods during severe flares.[4] They broadly suppress immune responses and reduce swelling in ulcerated segments. Usual IBD induction doses might be around 0.5–1 mg/kg/day prednisolone, tapering as symptoms improve. Long use can cause high blood sugar, infection risk, mood change, and bone loss.[4]

3. Azathioprine (IMURAN) – Azathioprine is an immunosuppressive purine analog that reduces over-active T-cell and B-cell responses.[5] Case reports show CEAS improvement with azathioprine, especially for anemia and protein loss.[1] Typical maintenance doses for IBD are about 1.5–2.5 mg/kg/day orally. Side effects include bone-marrow suppression, liver injury, and increased infection or lymphoma risk, so regular blood tests are essential.[5]

4. 6-mercaptopurine – 6-MP is closely related to azathioprine and works by blocking DNA synthesis in immune cells.[5] It may be used instead of azathioprine when better tolerated, at weight-based daily doses under close lab monitoring. Risks include low white blood cells, liver enzyme rise, nausea, and, rarely, serious infections or malignancy.[5]

5. Infliximab (REMICADE and biosimilars) – Infliximab is an antibody that blocks tumor necrosis factor-alpha (TNF-α), a key inflammatory signal.[6] For Crohn’s disease or ulcerative colitis it is given as intravenous infusions (e.g., 5 mg/kg at weeks 0, 2, 6 then every 8 weeks). It may help CEAS-like inflammation when conventional therapy fails, but can cause serious infections, infusion reactions, and rare demyelinating disease or heart failure worsening.[6]

6. Other infliximab biosimilars (INFLECTRA, RENFLEXIS, ZYMFENTRA) – Biosimilars provide TNF-α blockade similar to brand infliximab and are used with the same dosing principles in IBD.[7] They may be chosen for cost or availability reasons. Safety issues are similar: increased infection risk, possible reactivation of TB or hepatitis B, and infusion or injection reactions.[7]

7. Vedolizumab (ENTYVIO) – Vedolizumab is a gut-selective antibody that blocks α4β7 integrin on lymphocytes, preventing them from entering the intestinal wall.[8] It is given as IV or SC therapy at regular intervals for moderate-to-severe IBD and could be considered off-label in CEAS. It mainly increases infection risk in the gut and liver, but is relatively gut-targeted compared to systemic biologics.[8]

8. Ustekinumab – Ustekinumab blocks interleukin-12 and interleukin-23, two cytokines involved in chronic gut inflammation.[2] It is approved for Crohn’s disease and ulcerative colitis, given first intravenously then as periodic injections. It may help some CEAS-like cases, but data are limited. Side effects include infection, headache, and rare serious allergic reactions.[2]

9. JAK inhibitor (e.g., upadacitinib – RINVOQ) – Upadacitinib is an oral Janus kinase inhibitor that blocks multiple inflammatory signals inside immune cells.[9] It is used for moderate-to-severe ulcerative colitis at once-daily doses. Because it can increase risk of serious infections, blood clots, and changes in lipids, careful specialist supervision is required, and use in CEAS would be off-label and cautious.[9]

10. Proton pump inhibitors (PPIs, e.g., omeprazole) – PPIs reduce stomach acid and can lessen upper-GI irritation and pain.[3] They do not directly treat CEAS ulcers in the small intestine, but can protect the stomach and duodenum when steroids or other irritating drugs are used. Side effects include headache, diarrhea, and, with long use, possible nutrient deficiencies.[3]

11. H2-receptor blockers (e.g., famotidine) – These medicines also reduce acid but are generally milder than PPIs.[3] They may be chosen for night-time symptoms or when PPIs are not tolerated. Potential side effects include headache or dizziness, and severe reactions are rare.[3]

12. Sucralfate – Sucralfate coats damaged mucosa with a protective layer and may soothe ulcers in the upper gut.[3] It is usually taken several times daily on an empty stomach. It does not change inflammation directly but protects the surface from acid and bile. Constipation and interference with absorption of other medicines are possible side effects.[3]

13. Oral iron supplements (e.g., ferrous sulfate) – When iron-deficiency anemia is mild and absorption is adequate, oral iron tablets or liquids can refill body iron stores.[3] Typical doses are divided through the day and adjusted to tolerance. Nausea, constipation, or dark stools are common, so dosing rules may be modified by the doctor.[3]

14. Intravenous iron (e.g., iron sucrose, ferric carboxymaltose) – In CEAS, oral iron may absorb poorly because of small-bowel ulcers.[3] IV iron gives a controlled dose directly into the bloodstream in hospital or clinic. This method quickly raises hemoglobin but must be given under supervision because of rare allergic reactions or low blood pressure.[3]

15. Parenteral nutrition (total or partial) – In very severe malnutrition or after major bowel surgery, nutrition can be given directly into a vein.[4] This is not a first-line treatment but a temporary rescue to stabilize weight, protein, and micronutrients. It carries risks like infection, liver stress, and line complications, so is carefully monitored.[4]

16. Antidiarrheal agents (e.g., loperamide) – These slow bowel movements and can reduce urgency in milder phases.[3] They must be used carefully and usually avoided in severe flares or when obstruction is suspected, because they might worsen toxic megacolon or ileus. Side effects include constipation and abdominal cramps.[3]

17. Bile-acid binders (e.g., cholestyramine) – If portions of the ileum are diseased or removed, bile acids may enter the colon and cause watery diarrhea.[3] Cholestyramine powder binds bile acids in the intestine and can reduce this symptom. It may interfere with absorption of other drugs and vitamins and can cause bloating or constipation.[3]

18. Antibiotics (e.g., metronidazole, ciprofloxacin) – Short courses of antibiotics might be used for bacterial overgrowth, abscesses, or postoperative infections but are not routine long-term CEAS therapy.[2] Overuse may cause resistance, gut microbiome disturbance, or side effects like nausea, tendon problems, or neuropathy.[2]

19. Albumin infusions – For very low blood protein with edema, intravenous albumin can temporarily restore oncotic pressure and reduce swelling.[4] It is usually combined with diuretics and better control of intestinal protein loss. Risks include fluid overload and cost, so it is used selectively in hospital settings.[4]

20. Pain-control strategies (paracetamol, cautious opioids) – Pain is usually managed first with paracetamol (acetaminophen), because NSAIDs are avoided in CEAS.[3] In very severe cases, short-term opioid use under strict specialist supervision may be required, but long-term opioids can slow the gut and cause dependence.[3]

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Dietary molecular supplements

These are supportive ideas sometimes discussed in chronic intestinal disease. Evidence in CEAS specifically is limited, and any supplement should be checked with the treating doctor.

1. Vitamin D – Helps bone health and modulates immune responses in the gut; low levels are common in chronic enteropathy.[1] Supplement doses depend on blood levels and are often taken once daily or weekly as advised.

2. Calcium – Supports bones, especially if steroids are used or activity is low.[1] It is usually taken in divided oral doses with food, but high doses can cause constipation or kidney stones, so total intake is guided by a clinician.

3. Vitamin B12 – The terminal ileum absorbs B12; CEAS can damage this area or require resections.[2] In deficiency, intramuscular injections or high-dose oral B12 support red-blood-cell and nerve function.

4. Folate – Folate is important for blood formation and may be low due to malabsorption or certain drugs like azathioprine.[5] Oral low-dose folic acid is often used, but doses and need are checked with blood tests.

5. Iron (as supplement) – Oral or IV iron are “molecular” replacements of the missing mineral needed for hemoglobin.[3] Choice of route and dose depends on anemia severity, tolerance, and gut absorption.

6. Zinc – Diarrhea and protein loss can reduce zinc stores, which are important for immune function and wound healing.[2] Oral zinc supplements may help recovery but can cause nausea or interact with copper absorption if over-used.

7. Omega-3 fatty acids – Omega-3s from fish oil may have anti-inflammatory effects in some intestinal diseases.[2] Typical doses are several grams per day of EPA/DHA, but evidence in CEAS is limited and bleeding risk must be considered.

8. Probiotics – Certain probiotic strains might help balance gut bacteria and reduce symptoms in some chronic bowel conditions.[2] Evidence in CEAS is not strong, so they are usually considered optional, low-risk additions rather than core therapy.

9. Multivitamin with trace elements – A complete multivitamin can cover multiple small deficiencies (vitamins A, E, K, selenium, copper) that are hard to track individually in a malabsorptive state.[3] Doses follow the product label and doctor advice.

10. Oral amino-acid or peptide formulas – These products give protein in forms that are easier to absorb in damaged small bowel.[4] They may support protein status and healing when whole-protein foods cause bloating or discomfort.

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Immune-booster / regenerative / stem-cell-related therapies

For CEAS, true “stem cell” or regenerative treatments are experimental. They are usually done only in clinical trials or specialized centers, never as self-treatment.

1. Good basic nutrition as the first “immune booster” – Adequate calories, protein, and vitamins are the safest and strongest way to support immunity and mucosal healing.[1] There is no single magic pill; balanced diet is the base.

2. Thiopurines (azathioprine / 6-MP) as immune modulators – These drugs do not “boost” the immune system; they re-balance it by calming harmful inflammation.[5] When inflammation is under control, the gut lining can regenerate more effectively.

3. Biologic agents (infliximab, vedolizumab, ustekinumab) – By precisely blocking key inflammatory signals, biologics give the bowel a chance to heal.[2][6] Mucosal healing itself is a kind of regeneration, even though the medicines suppress parts of the immune system.

4. Hematopoietic stem cell transplantation (HSCT) – HSCT has been tried in severe, refractory inflammatory bowel disease with mixed results.[2] It aims to “reset” the immune system, but risks are very high (serious infections, death), so it is not standard for CEAS.

5. Mesenchymal stromal cell therapy – Experimental trials in IBD use mesenchymal cells to release anti-inflammatory and tissue-healing factors.[2] For CEAS this approach is still theoretical and should only be considered in formal research studies.

6. Future SLCO2A1-targeted or gene-based therapy – As we better understand how SLCO2A1 controls prostaglandin transport, gene therapy or small-molecule modulators may appear in the future.[2][7] These are still research ideas, not current treatment options.

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Surgeries – Procedures and why they are done

1. Segmental small-bowel resection – When a bowel segment has deep ulcers, severe bleeding, or tight strictures that block passage, surgeons may remove that limited piece of intestine and reconnect the healthy ends.[1] This can relieve obstruction and reduce protein loss, but too many resections risk short-bowel problems.[3]

2. Strictureplasty – In some strictures, instead of cutting out the bowel, surgeons can open and widen the narrowed area and then close it in a way that keeps length.[3] This preserves more intestine and can be helpful for patients with several strictures along the small bowel.

3. Endoscopic balloon dilation – For short strictures, an endoscope can be used to inflate a balloon inside the narrowed area to gently stretch it.[3] This minimally invasive option may delay or avoid open surgery, but sometimes needs to be repeated.

4. Creation of a temporary stoma – In very severe disease or after complex surgery, an opening (stoma) on the abdominal wall may divert stool.[3] This allows the lower bowel to rest and heal, with the possibility of later re-connection when inflammation is controlled.

5. Emergency surgery for perforation or massive bleeding – Rarely, ulcers may perforate or bleed heavily, causing life-threatening situations.[1][3] Emergency surgery removes the damaged part and controls bleeding, saving the patient’s life but also increasing future surgical complexity.

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Preventions

Because CEAS is genetic, it cannot be fully prevented, but complications can often be reduced:

1. Avoid NSAIDs and other gut-toxic drugs whenever possible.[3]

2. Do not smoke; avoid heavy alcohol use.[2]

3. Keep regular follow-up with a gastroenterologist, even when feeling well.[1]

4. Treat anemia and malnutrition early to protect growth and organs.[1]

5. Stay up-to-date with routine and IBD-related vaccines before strong immune suppression.[5]

6. Seek quick care for new severe pain, vomiting, or blood in stool to catch complications early.[3]

7. Follow agreed diet plans, including enteral nutrition when prescribed.[2]

8. Share a written emergency plan with family and local doctors for flares.[4]

9. Manage stress and mental health, which can worsen symptom perception and adherence.[4]

10. Offer family genetic counseling for siblings and future pregnancies.[1][5]

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When to see doctors

People with chronic enteropathy associated with SLCO2A1 gene should stay in regular contact with a gastroenterologist and a pediatric or adult internist. They should seek urgent or emergency care if they notice severe new abdominal pain, vomiting with inability to drink, black or bloody stools, fainting, fever with chills, or sudden swelling and shortness of breath.[1][3] These signs can point to complications such as perforation, massive bleeding, severe infection, or blood clots, which need fast hospital treatment. Worsening tiredness, weight loss, leg swelling, or poor school/work performance are also reasons to book a clinic visit soon so that anemia, protein loss, or treatment side effects can be checked and corrected.[4] Because CEAS is rare and complex, management should be shared by experienced specialists rather than self-medication at home.[1][5]

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What to eat and what to avoid

Diet must always be personalized with a dietitian and doctor, especially in children.

1. Eat soft, well-cooked rice, pasta, or potatoes that are easy to digest; avoid very spicy, fried, or greasy fast foods that can increase cramps and diarrhea.[2]

2. Eat tender, lean proteins like chicken, fish, eggs, tofu, or well-cooked lentils if tolerated; avoid large fatty meat portions and tough, stringy cuts.[2]

3. Eat peeled, well-cooked vegetables and low-fiber fruits (like banana); avoid raw salads, seeds, popcorn, and very fibrous skins during flares.[2][4]

4. Use lactose-free or low-lactose dairy if milk worsens symptoms; avoid heavy cream, rich cheeses, and sugary milkshakes, which can cause bloating.[3]

5. Drink plenty of water and oral rehydration solutions; avoid large amounts of very sweet juices and fizzy drinks that may pull more water into the gut.[1]

6. Choose small, frequent meals through the day; avoid very large meals that stretch and stress the inflamed bowel.[2]

7. Include healthy fats in small amounts, such as olive oil or small portions of nut butters if tolerated; avoid deep-fried foods.[2]

8. Limit caffeine (strong tea, coffee, energy drinks), which can speed up bowel movements; avoid energy drinks that also add sugar and stimulants.[3]

9. Be careful with “health” herbs and supplements that are not discussed with the doctor; some can interact with medicines or irritate the gut.[4]

10. Adjust diet during flares (more liquid and very soft foods) and slowly expand during quiet times, guided by symptoms and professional advice.[2]

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FAQs

1. Is chronic enteropathy associated with SLCO2A1 gene the same as Crohn’s disease?
No. CEAS looks similar to Crohn’s disease on endoscopy, but it is a monogenic, autosomal-recessive disease caused by SLCO2A1 mutations, while Crohn’s is a complex, multi-gene inflammatory disease. Treatment ideas overlap, but diagnosis and genetics are different.[1]

2. How is CEAS diagnosed?
Doctors combine history, blood tests, capsule or balloon endoscopy, imaging, and sometimes biopsy stains for SLCO2A1.[1][5] Finally, they confirm the disease by genetic testing showing harmful mutations in both copies of the SLCO2A1 gene.[1]

3. What symptoms usually appear first?
Common early signs are long-term abdominal pain, iron-deficiency anemia, tiredness, swelling from low protein, and sometimes watery or bloody stools.[3] Many patients have repeated hospital visits for anemia or low albumin before the rare diagnosis is found.[3]

4. At what age does CEAS usually start?
Many patients develop symptoms from late childhood to early adulthood, but diagnosis can be delayed into later life.[4] Reports describe pediatric and adult cases, showing that CEAS is a life-long condition once present.[4]

5. Is there a cure?
Right now there is no genetic cure for CEAS.[2] Treatment focuses on controlling inflammation, healing ulcers, correcting anemia and protein loss, and treating complications like strictures or perforation with surgery when needed.[3]

6. Can CEAS go into remission?
Yes. With proper diet, medicines, and sometimes surgery, many patients experience periods of fewer symptoms and better blood tests.[1][3] However, long-term follow-up is needed because flares can return.

7. Will all patients need surgery?
Not all, but about half of reported patients eventually need some form of intestinal resection or stricture treatment because of repeated ulcers or strictures.[3][4] Decisions depend on symptom severity, response to medicines, and imaging findings.

8. Is CEAS life-threatening?
Severe untreated disease can cause life-threatening bleeding, perforation, or extreme malnutrition.[3] With modern supportive care, careful monitoring, and appropriate surgery, many patients can live for many years, though they may have ongoing health challenges.[1]

9. Can family members be tested?
Yes. Because CEAS is autosomal recessive, brothers, sisters, and parents may be carriers or affected.[1] Genetic counseling explains who should be tested, what results mean, and options in future pregnancies.[5]

10. Is pregnancy possible for someone with CEAS?
Many women with chronic intestinal diseases can have safe pregnancies with planning.[2] They should work closely with gastroenterologists and obstetricians before conception to adjust medicines, optimize nutrition, and plan monitoring. Some drugs are not safe in pregnancy and must be changed beforehand.

11. Do biologic drugs mean the immune system is “destroyed”?
No. Biologics modify specific immune pathways rather than destroying immunity completely.[6] Infection risk rises, but with vaccines, screening for TB and hepatitis, and regular follow-up, many patients use these medicines safely for years.[6]

12. Can diet alone treat CEAS?
Diet is very important but usually not enough alone.[2] Because the root problem is a genetic prostaglandin transporter defect, medical and sometimes surgical treatments are normally needed together with careful nutrition.

13. Are probiotics always good for CEAS?
Not always. Some people feel better, others notice no change, and some feel worse.[2] Evidence is limited, so probiotics should be treated as optional and discussed with the doctor, especially in severely ill or immunosuppressed patients.

14. How often should follow-up visits happen?
In stable periods, visits every 3–6 months may be enough; during flares or after starting new medicines, appointments may be much more frequent.[1] Blood tests and imaging schedules depend on symptoms and chosen treatments.

15. Where can patients and families find more information?
Reliable sources include rare-disease registries, peer-reviewed case reports and reviews, and patient organizations for inflammatory bowel disease.[1][4] Because CEAS is rare, information online should always be checked with the treating gastroenterologist before any changes are made.

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: January 23, 2026.