celiac disease, igg, igg4 pouchitis is a general term that refers to a wide spectrum of diseases and conditions that cause inflammation of the pouch. it is a common complication after ipaa/rpc. people report many symptoms including abdominal pain or cramps, increased bowel frequency, urgency of movements, strong evacuation urges, daytime incontinence, nocturnal seepage, and/or rectal bleeding. studies show pouchitis occurs more often in people who got their pouch because of ulcerative colitis rather than familial adenomatous polyposis (fap) which suggests that the pathogenic (microbial) background of uc may contribute to the development of pouchitis in some pouches. diagnosis of pouchitis: pouchitis is diagnosed based on the presence of symptoms together with endoscopic and histological evidence of pouch inflammation. for example, biopsies may be taken during a pouchoscopy (a camera exam like a colonoscopy but for the pouch) to rule out infection from clostridium difficile infection (c diff) or cytomegalovirus (cmv). treatment of infections usually begins with antibiotics and may also include multi-strain probiotics. after exams and tests, pouchitis is divided into two categories based on findings: idiopathic or secondary. in idiopathic pouchitis the cause of inflammation is still unclear. with secondary pouchitis there is an association with a specific causative or pathogenetic factor. secondary pouchitis can be classified into subgroups. it is possible to have one or more causes of pouch inflammation at the same time. antibiotic therapy for pouchitis: standard treatment of pouchitis when first reported (acute pouchitis) without any other obvious cause identified such as infection or anal join leak (fistula) is oral antibiotics for two weeks, typically ciprofloxacin 500 mg every 12 hours. alternatives to ciprofloxacin for initial therapy include metronidazole 500 mg (twice daily) or tinidazole 500 mg (twice daily). for pouches with acute idiopathic pouchitis, response to antibiotic therapy is typically examined clinically by asking the patient if they have experienced an improvement with their symptoms plus looking endoscopically using a pouchoscopy after completing antibiotic therapy. while endoscopic mucosal healing may lag behind symptomatic improvement, mucosal healing is a treatment target for patients with pouchitis. probiotics for pouchitis: if pouchitis responded to antibiotic therapy, some people may be prescribed probiotics, depending on individual circumstances, to help maintain remission and heal the pouch's mucus layer. the most clinically researched multi-strain probiotic for pouchitis is the de simone formulation (formulation name) invented my medical doctor professor claudio de simone. the probiotic formulation was clinically researched for inflammatory bowel diseases including pouchitis under the brand name vsl#3 until 2016 when the makers of vsl#3 changed their formulation. the original de simone formulation is no longer in the probiotic named vsl#3, however, it continues to be sold under a variety of brand names globally including visbiome and vivomixx. in august 2019 the american gastroenterology association (aga) issued a correction for its official pouchitis guidance stating that the probiotic formulation in vsl#3 had changed. other bodies and journals such european crohn's and colitis organisation (ecco) have also issued clarifications. as has the cochrane library with a clarification regarding the formulations for probiotics using the de simone formulation and the new vsl#3. research since 2016 is published under the formulation's name "de simone formulation" or an individual regional brand names. the new post-2016 formulation of vsl#3 is also a multi-strain probiotic that some use for the treatment of various gastrointestinal disorders. research on the new formulation starts from 2016. cuffitis: cuffitis is inflammation of the retained rectal 'cuff' usually in a stapled pouch-anal anastomosis or the spot where the intestinal pouch was attached to the anus to restore anal evacuation. symptoms are typically similar to ulcerative proctosis for ulcerative colitis pouches including burning in the anal canal, a change in bowel movements, and sometimes rectal bleeding. cuffitis diagnosis: most expert pouch centers plus national gastroenterology society guidelines worldwide recommend a cuff be no longer than 2 cm with the aim to staple the pouch at the level of the anorectal junction, leaving about 1 cm of rectum or mucus layer behind for the staple to attach the pouch to the anus. this retained 1 to 2 cm of rectum can therefore, sometimes retain ulcerative colitis in uc pouches. in contrast, hand sewn pouch-anal anastomosis' typically do not retain any mucus layer but sometimes a cuff is also used and it might cause discomfort for some hand sewn pouches. cuffitis is clinically diagnosed by symptoms plus endoscopically (pouchoscopy). treatment of cuffitis: first-line therapy for acute cuffitis due to retained rectal mucosal layer is usually similar to the treatment for ulcerative proctosis that ulcerative colitis patients would have likely used before pouch surgery. mesalazine suppositories or enemas are sometimes prescribed first (brand names include asacol, canasa, and pentasa). if those do not provide enough relief then treatment might be escalated to corticosteroid suppositories or enemas such as budesonide. biological therapy may be prescribed if all other medical therapies fail to manage cuffitis and the person either isn't suitable for or does not want to undergo a revision operation to remove the retained cuff and hand sew the pouch on the level of the dentate line (spot where anal skin changes to rectal mucus layer). crohn's disease of the pouch: some people undergoing ipaa/rpc may be later diagnosed with underlying crohn's disease because the disease likely had not fully expressed itself at the time of surgery. crohn's disease of the pouch is associated with high failure rates. pouches may experience fistula leaks that cause pelvic sepsis and other complications. treatment of crohn's disease of the pouch: if a person later diagnosed with crohn's disease of the pouch wishes to keep their pouch operational, in some circumstances medical management might be possible. typically this is done with the prescription of biologics. it may take more than one drug prescription to find a suitable biologic that encourages the desired anti-inflammatory response. celiac disease: celiac disease is an autoimmune condition that causes inflammation in the small intestine after gluten is eaten. it can cause pouchitis symptoms and discomfort. some pouches that are celiac initially get misdiagnosed with crohn's of the pouch. celiac disease is typically diagnosed by biopsy of the pouch. treatment of celiac disease: typically, the first therapy approach to manage celiac disease in person with an ileum pouch is dietary modification to reduce and/or eliminate gluten. other inflammatory conditions including igg and igg4: immunoglobulin related diseases can also cause problems for a pouch. the two most common that are biopsied for are igg and igg4. igg molecules can initiate inflammatory reactions, both good and bad. when the autoimmune reaction is inappropriate, a pouch might have problems with igg. igg4 is a subclass of igg. igg4 disease is a chronic immune-mediated fibroinflammatory disorder that can manifest with painless enlargement of organs or tumor-like masses. pouch infections examples: clostridium difficile infection (c diff) or cytomegalovirus (cmv) biopsies of the pouch should confirm if an infection is the root cause of pouch inflammation. if an infection from clostridium difficile (c diff) or cytomegalovirus (cmv) is found, initial therapy is usually antibiotics. certain infections such as a clostridium difficile (c diff) might also be treated with probiotics just as a c diff infection in a person who still has their colon would be treated. probiotics that are prescribed for pouchitis (specifically pouchitis diagnosed as being caused by dysbiosis) are often also prescribed for c diff. surgery related complications examples: anastomotic leaks, fistulas, sinus, pelvic sepsis the pouch surgery itself can be the reason for some complications. anastomotic leaks: anastomotic leaks occur on the lines where the pouch was sutured or stapled. they usually occur close to the time of surgery but can appear months or years later. when an anastomotic leak occurs, it can form a fistula or tract of fluid. fistulas: most fistulas will connect from an anastomotic leak to another area of the body such as a pouch-vagina fistula, perianal fistula, or presacral fistula with pelvic collection. in some circumstances fistulas develop years after pouch creation due to the development of crohn's disease. fistula's caused by crohn's disease are often treated with biological therapy while a fistula from an anastomotic leak requires different therapies as biologics rarely help close a leak in a surgical suture or staple line of the pouch. different potential treatment options for an anastomotic fistula depend on the leak's location and include but are not limited to endo-vac sponge, needle-knife therapy, draining seton, or cutting seton. pelvic collection/sinus: a pelvic collection or collection of fluid anywhere from a leak is called a sinus. when an anastomotic leak isn't treated promptly or doesn't heal it can cause pelvic sepsis. pelvic sepsis: pelvic sepsis also called peri pouch sepsis is a main cause of pouch failure and it creates conditions that make major revisionary surgery difficult. mechanical disorders examples: large or small pouch, u-bends, twists, prolapse, stricture, weak sphincters large or small pouch: standard guidelines for j-pouch construction is to use two loops of 15–20 cm ileum. if the pouch is too small, the pouch will have a small volume. this will increase the frequency of moments and could also be a cause of pouch failure. pouch prolapse: a pouch can either tilt or twist when stapled onto the anus causing prolapse. pouch prolapse is also sometimes referred to as a floppy pouch. pouch prolapse from a pouch being tilted or twisted can also cause pouch fistulas to develop, especially pouch-vagina fistulas. prolapse can also happen after the pouch is already in use. with symptoms for pouch prolapse, a person typically has evacuation difficulties, seepage, pain, nausea, abdominal pain, or overt external prolapse of tissue. strictures or stenosis narrowing of the anal canal under the pouch can cause evacuation difficulties. anal stricture can be a cause of pouch failure if not managed properly. anal stricture is a common complication of pouch surgery. it is treated with dilatation or stretching under anesthesia. some people are also prescribed home dilatation routine using a hegar dilator to manage chronic stenosis that keeps returning after dilatation. u-bend: this happens when the surgeon fires the linear stapler and it malfunctions. usually the flaw is not noticed until after surgery is complete during first pouchoscopy. the pouch is called a "u" bend because instead of stapling the two full sections of ileum together into a "j", the stapler doesn't create the 'j' but instead retains the shape of a 'u'. functional disorders examples: irritable pouch syndrome, pelvic floor dysfunction, evacuation difficulties, coexisting psychiatric diagnosis pelvic floor dysfunction and evacuation difficulties pelvic floor dysfunction is a common complication of pelvic surgery. the mayo clinic believes it is an under reported complication of ipaa/rpc surgery with up to 75% of pouch patients experiencing non-relaxing pelvic floor dysfunction. biofeedback therapy is the main treatment for pelvic floor dysfunction. dysplasia or neoplasia examples: adenomas, cancers cancer is a rare event after a pouch is created. however, retained rectal mucosa can develop dysplasia over time especially if cuffitis is an ongoing complication. cancer can also develop in the pouch when there has been long-term pouchitis (inflammation of the pouch for any reason). people who got their pouch as a result of bowel cancer may also experience cancer of the pouch. systemic or metabolic disorders examples: malnutrition, anemia, vitamin b12 and vitamin d deficiency, low potassium removal of the entire large intestine (colon) and amounts of the terminal ileum at the end of the small intestine leads to fluid and nutritional absorption issues for all pouches. the colon absorbs water, salts, and some key nutrients. dehydration can occur if a person does not get enough fluids. the importance of ileum is that it absorbs key vitamins and minerals including b12, potassium, and magnesium. in medical terms, low potassium is called hypokalemia. when a pouch is constructed small amounts of ileum are lost to the stomas and any stoma revisions. the pouch itself is also made from ileum. if a pouch is defunctioned (also explained as disconnected and/or pouch failure) the person also loses this extra amount of ileum when the pouch is disconnected from the digestive tract. people with a pouch are at a further health risk if they experience excessive potassium loss due to things like vomiting, diarrhea, and prescription medications that increase urination. this is because potassium is a key electrolyte that helps regulate the heart and if levels become too low,