Fibrosing colonopathy explained

Synonyms:	Abdominal pain, abdominal swelling, vomiting, constipation
Causes:	High doses of pancreatic enzyme supplements
Risks:	Young age, prior surgery of the intestines, certain medications including corticosteroids and H2 blockers
Frequency:	>60 cases reported

Fibrosing colonopathy is a disease that arises in people with cystic fibrosis treated with high doses of pancreatic enzyme supplements.^[1] Symptoms are non-specific with abdominal pain, abdominal swelling, vomiting, and constipation.^[1]

Risk factors include being young, prior surgery of the intestines, and the use of certain medications including corticosteroids and H2 blockers.^[2] It may appear similar to distal intestinal obstruction syndrome or inflammatory colitis such as Crohn's disease.

A maximum dose of 10,000 IU of lipase per kilogram per day is recommended for pancreatic enzyme supplementation to prevent this condition.^[3] More than 60 cases have been described as of 1999.^[2] The disease was suggested to be caused by methacrylic acid copolymer which is used as coating for delayed release of enzymes but there is no reliable evidence for that.^[4]

Signs and symptoms

Clinical symptoms of fibrosing colonopathy can be similar to those of intestinal obstruction and include constipation or diarrhea, abdominal pain, hematochezia,^[5] vomiting, nausea, and poor weight gain.^[6]

Causes

The precise etiopathogenesis is still unknown. Young age (2–13 years), gastrointestinal history (DIOS, meconium ileus), abdominal surgery in the past, HDPE, and use of histamine H2-receptor blockers, corticosteroids, or recombinant human deoxyribonuclease (DNase) are risk factors.^[7] Fibrosing colonopathy and cystic fibrosis have a strong correlation.^[6]

Diagnosis

The afflicted colon's histologic findings include thickening of the muscularis propria, submucosal fibrosis, a cobblestone-like appearance, and persistent mucosal inflammation.^[8] With relative rectal sparing, imaging can demonstrate diffuse narrowing, shortening, and loss of haustration of the colonic lumen.^[9]

See also

• Cystic fibrosis
• Pancreatic enzymes (medication)

Further reading

• DODGE . J A . Fibrosing colonopathy . Gut . BMJ . 46 . 2 . February 1, 2000 . 0017-5749 . 10.1136/gut.46.2.152 . 152–153 . 10644305 . 1727820 . none.
• Bansi . D S . Fibrosing colonopathy in an adult owing to over use of pancreatic enzyme supplements . Gut . BMJ . 46 . 2 . February 1, 2000 . 0017-5749 . 10.1136/gut.46.2.283 . 283–285 . 10644326 . 1727802 . none.

External links

• Fibrosing Colonopathy in Cystic Fibrosis
• Cystic Fibrosis Foundation - Pancreatic Enzymes Clinical Care Guidelines

Notes and References

1. Smyth RL . Fibrosing colonopathy in cystic fibrosis . . 74 . 5 . 464–8 . May 1996 . 8669970 . 1511547 . 10.1136/adc.74.5.464.
2. Lloyd-Still . JD . Beno . DW . Kimura . RM . Cystic fibrosis colonopathy. . Current Gastroenterology Reports . June 1999 . 1 . 3 . 231–7 . 10980955. 10.1007/s11894-999-0040-4 . 37595322 .
3. Schibli . S . Durie . PR . Tullis . ED . Proper usage of pancreatic enzymes. . Current Opinion in Pulmonary Medicine . November 2002 . 8 . 6 . 542–6 . 12394164. 10.1097/00063198-200211000-00010 .
4. Lack of evidence for fibrosing colonopathy by 5-ASA in humans. Digestive Diseases and Sciences. 1999. 44. 7. 1494. 10.1023/a:1026676407838. 10489937. Borum. M. L.. Ginsberg. A. L.. 34629882.
5. Terheggen . G . Dieninghoff . D . Rietschel . E . Drebber . U . Kruis . W . Leifeld . L . Successful Non-Invasive treatment of stricturing fibrosing colonopathy in an adult patient . European Journal of Medical Research . Springer Science and Business Media LLC . 16 . 9 . 2011 . 411–414 . 2047-783X . 10.1186/2047-783x-16-9-411 . 22024442 . 3352147 . free .
6. Fiore . Dominic . Sohini . Vidhur . Mileti . Elizabeth . Fiore . Nicholas . Fibrosing Colonopathy Presenting in a Patient with Cystinosis . JPGN Reports . Wiley . 4 . 3 . June 9, 2023 . 2691-171X . 10.1097/pg9.0000000000000321 . e321. 37600607 . 10435035 .
7. FitzSimmons . Stacey C. . Burkhart . Greg A. . Borowitz . Drucy . Grand . Richard J. . Hammerstrom . Thomas . Durie . Peter R. . Lloyd-Still . John D. . Lowenfels . Albert B. . High-Dose Pancreatic-Enzyme Supplements and Fibrosing Colonopathy in Children with Cystic Fibrosis . New England Journal of Medicine . Massachusetts Medical Society . 336 . 18 . 1997 . 0028-4793 . 10.1056/nejm199705013361803 . 1283–1289. free .
8. Pawel . Bruce R . de Chadarévian . Jean-Pierre . Franco . Maria E . The pathology of fibrosing colonopathy of cystic fibrosis: A study of 12 cases and review of the literature . Human Pathology . Elsevier BV . 28 . 4 . 1997 . 0046-8177 . 10.1016/s0046-8177(97)90025-3 . 395–399. 9104936 .
9. Schwarzenberg . S.J. . Wielinski . C.L. . Shamieh . I. . Carpenter . B.L.M. . Jessurun . J. . Weisdorf . S.A. . Warwick . W.J. . Sharp . H.L. . Cystic fibrosis–associated colitis and fibrosing colonopathy . The Journal of Pediatrics . Elsevier BV . 127 . 4 . 1995 . 0022-3476 . 10.1016/s0022-3476(95)70113-3 . 565–570. 7562277 .