今日の臨床サポート

蛋白漏出性胃腸症

著者: 篠村恭久 川西市医師会メディカルセンター

監修: 上村直実 国立国際医療研究センター 国府台病院

著者校正済:2022/05/11
現在監修レビュー中
患者向け説明資料

概要・推奨   

  1. Cronkhite-Canada症候群に対してステロイド治療が推奨される(推奨度1)
  1. ループス関連蛋白漏出性胃腸症に対してステロイド治療が推奨される(推奨度1)
  1. 腸リンパ管拡張症患者には、低脂肪食と中鎖脂肪酸(medium-chain triglyceride、 MCT)の補充が推奨される(推奨度2)
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  1. Ménétrier病患者にはH. pyloriの除菌が推奨される(推奨度2)
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
篠村恭久 : 特に申告事項無し[2022年]
監修:上村直実 : 未申告[2022年]

改訂のポイント:
  1. Cronkhite-Canada症候群の治療の推奨度を2から1へ変更した。
  1. ループス関連蛋白漏出性胃腸症およびFontan手術後の蛋白漏出性胃腸症の項目を追加した。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. 蛋白漏出性胃腸症とは、消化管内腔へ蛋白が異常に漏出し、低蛋白血症を来す症候群である。
  1. 蛋白漏出性胃腸症について、有病率などの疫学情報は明らかにされていない。
  1. 蛋白漏出性胃腸症を来す種々の疾患があり、漏出の機序には、リンパ系の異常、毛細血管の透過性亢進、胃腸粘膜上皮の異常などがある。蛋白漏出性胃腸症を来す代表的疾患に腸リンパ管拡張症やMénétrier病、Cronkhite-Canada症候群がある。
問診・診察のポイント  
  1. 浮腫は高頻度にみられる症状であり、浮腫がある場合は胸水や腹水による息切れや腹部膨満の有無を問診し、胸水や腹水の有無を診察で確認する。

これより先の閲覧には個人契約のトライアルまたはお申込みが必要です。

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文献 

Björn K I Meijers, Simon Schalla, Filip Eerens, Robert-J Van Suylen, Bernard Broers, Emile M Cheriex, Jan-P Smedema
Protein-losing enteropathy in association with constrictive pericarditis.
Int J Cardiovasc Imaging. 2006 Jun-Aug;22(3-4):389-92. doi: 10.1007/s10554-005-9067-2. Epub 2006 Feb 25.
Abstract/Text Although acute pericarditis is a common and usual benign disorder, sometimes evolution to constrictive pericarditis may occur. We present a case of constrictive pericarditis late after coronary bypass grafting, complicated by right sided heart failure. Edema formation was aggravated due to protein-losing enteropathy, resulting in hypoalbuminemia. Imaging of constrictive pericarditis was done by ultrasound as well as simultaneous pressure recording of the right and left ventricle. Imaging of intestinal protein loss was possible using intravenous Technetium-99m-labelled human serum albumin.

PMID 16502021
G H JEFFRIES, A CHAPMAN, M H SLEISENGER
LOW-FAT DIET IN INTESTINAL LYMPHANGIECTASIA. ITS EFFECT ON ALBUMIN METABOLISM.
N Engl J Med. 1964 Apr 9;270:761-6. doi: 10.1056/NEJM196404092701503.
Abstract/Text
PMID 14107315
Kunihiko Aoyagi, Mitsuo Iida, Takayuki Matsumoto, Shotaro Sakisaka
Enteral nutrition as a primary therapy for intestinal lymphangiectasia: value of elemental diet and polymeric diet compared with total parenteral nutrition.
Dig Dis Sci. 2005 Aug;50(8):1467-70.
Abstract/Text Intestinal lymphangiectasia (IL) is a rare disease requiring oral fat restriction. The aim of this study was to evaluate the efficacy of enteral nutrition compared to that of total parenteral nutrition (TPN). We retrospectively reviewed nine patients with IL presenting with protein-losing enteropathy. Of these, seven patients not responding to a low-fat diet were treated with elemental diet (ED), polymeric diet (PD) containing medium-chain triglycerides, or TPN. Improvement in serum total protein was observed in two of three on ED and in one of two on PD, compared with three of three on TPN. Enteric protein loss was improved in two of two on ED, one of two on PD, and two of two on TPN. Outpatients who continued to receive enteral nutrition maintained a total protein level. Enteral nutirition appears to be as effective as TPN for patients with IL, and it may provide a valid and safe alternative therapy.

PMID 16110837
Y Yasunaga, Y Shinomura, S Kanayama, M Yabu, T Nakanishi, Y Miyazaki, Y Murayama, J J Bonilla-Palacios, Y Matsuzawa
Improved fold width and increased acid secretion after eradication of the organism in Helicobacter pylori associated enlarged fold gastritis.
Gut. 1994 Nov;35(11):1571-4.
Abstract/Text This study examined the effects of eradication of Helicobacter pylori (H pylori) infection on gastric mucosal morphology and acid secretion. Sixteen H pylori positive patients with enlarged gastric body folds were divided into two groups: (a) patients with moderate enlargement (fold width: 6 to 10 mm, n = 8) and (b) patients with severe enlargement (> 10 mm, n = 8). After successful treatment, gastric body fold width was reduced in both groups (p < 0.01) with an associated decrease in inflammatory infiltrates in the body mucosa (p < 0.01 and p < 0.05). Basal acid output and tetragastrin stimulated maximal acid output (mean (SEM)) in all 16 patients significantly increased from 1.1 (0.5) to 2.9 (0.9) mmol/h (p < 0.05) and from 5.4 (1.3) to 18.7 (2.3) mmol/h (p < 0.01), respectively, with a significant decrease in fasting serum gastrin concentrations, from 127.1 (16.1) to 59.6 (3.8) pg/ml (p < 0.01). The increase in acid secretion after eradication of H pylori was more noticeable in the severe group, who had shown lower acid secretion and higher serum gastrin concentrations (p < 0.05) before eradication, than the increase seen in the moderate group. The decreases in ammonia nitrogen content seen after eradication were significant in basal (from 0.91 (0.17) to 0.37 (0.08) mmol/h, p < 0.05) and stimulated gastric secretions (from 1.57 (0.19) to 0.37 (0.13) mmol/h, p < 0.01), although these changes were too small to explain the increases in basal acid output and maximal acid output. These results suggest that inflammation of the gastric body mucosa caused by H pylori infection is associated with enlarged gastric body folds and inhibition of acid secretion in H pylori positive patients with enlarged gastric body folds.

PMID 7828975
M Stolte, C H Bätz, E Bayerdörffer, S Eidt
Helicobacter pylori eradication in the treatment and differential diagnosis of giant folds in the corpus and fundus of the stomach.
Z Gastroenterol. 1995 Apr;33(4):198-201.
Abstract/Text In an earlier study, we were able to show that giant fold gastritis is probably a special form of Helicobacter pylori-associated gastritis. Proof of this contention, however--namely regression of the giant folds following eradication of the organism--in a large number of patients was not then possible. To rectify this, Helicobacter pylori (HP) eradication treatment with omeprazole and amoxicillin was applied to 47 patients with HP gastritis and giant folds (5 patients with giant folds localized in the anterior or posterior wall, 42 patients with generalised giant folds within the corpus and fundus). The results of treatment were investigated by endoscopy and histology at the earliest 4 weeks after termination of treatment. In 40 of the 47 patients (85.1%), HP eradication treatment was successful. In 7 patients in whom treatment was unsuccessful, follow-up examinations revealed no changes in the endoscopic or histological findings. In 2 out of 3 patients in whom the endoscopic findings were unchanged despite successful HP eradication, biopsy material revealed the cause of the giant folds to be a signet ring cell carcinoma; in the remaining patient the cause of giant fold persistence was unclear. In 36 patients, the endoscopic--findings normalised completely, while in one patient there was obvious regression of the giant folds. We conclude from this study that giant fold gastritis is indeed a special form of HP gastritis, and that eradicating the organism in patients with gastric giant folds may help to distinguish between inflammatory, hyperplastic and tumorous giant folds.

PMID 7793118
Abstract/Text Hypertrophic gastropathy--that is, Ménétrier's disease--was found, in a retrospective analysis, to be associated with Helicobacter pylori in more than 90% of patients. It is proposed that hypertrophic gastropathy represents a special form of H pylori gastritis in these patients. A case is described of a 28 year old woman with Ménétrier's disease associated with proved protein loss from the stomach. Treatment with cimetidine for more than three years had little benefit when colonisation by H pylori was detected. Density of H pylori colonisation and activity of gastritis, which was also present in the first biopsy specimens taken five years ago, were more pronounced in the body than in the antrum, which is in agreement with the characteristics of H pylori gastritis found in other cases with Ménétrier's disease. A 14 day antibacterial treatment course with 750 mg amoxicillin three times a day combined with 40 mg omeprazole three times a day was started in April 1991. This resulted in eradication of H pylori and the return to normal of giant folds and the mucosal histology. Serum protein concentrations returned to normal within six weeks and remained normal at two endoscopies during a two year follow up. This case report suggests that a subgroup of the patients with Ménétrier's disease may be healed by the eradication of H pylori.

PMID 8200570
Abstract/Text We herein report a case of a 46-yr-old female with Ménétrièr's disease associated with Helicobacter pylori (H. pylori) infection, in whom the appearance of enlarged gastric folds and hypoproteinemia were both successfully treated by antibacterial treatment. The patient had been treated with famotidine for 3 yr under a diagnosis of Ménétrièr's disease, which caused an increase in her serum protein concentration to a level at which she suffered no clinical problems; however, the concentration never reached a normal range, and the presence of enlarged gastric folds also remained unchanged. Because H. pylori colonization was detected in the stomach and was also retrospectively recognized in the initial histologic specimens, antibacterial treatment with omeprazole, clarithromycin, and metronidazole was thus attempted. As a result of the successful eradication of H. pylori, the enlarged gastric folds returned to normal and the serum protein concentration thus increased to a normal level after the treatment. The etiology of Ménétrièr's disease remains unknown; however, the above findings suggest that H. pylori infection may in some cases cause Ménétrièr's disease and should therefore be carefully treated in any patient with this condition.

PMID 9382064
T Kaneko, T Akamatsu, A Gotoh, K Shimodaira, T Shimizu, K Kiyosawa, T Katsuyama, A Momose
Remission of Ménétrier's disease after a prolonged period with therapeutic eradication of Helicobacter pylori.
Am J Gastroenterol. 1999 Jan;94(1):272-3. doi: 10.1111/j.1572-0241.1999.00816.x.
Abstract/Text We report here a case of Ménétrier's disease (MD) that required a prolonged period for remission after eradication therapy of Helicobacter pylori (HP). The appropriate time needed to judge the efficacy of the eradication therapy for HP infection in an MD case is discussed.

PMID 9934772
D M Bradburn, N F Redwood, C W Venables, A Gunn
Medical therapy of Ménétrier's disease with omeprazole.
Digestion. 1992;52(3-4):204-8.
Abstract/Text Medical therapy of Ménétrier's disease is often unsatisfactory and may lead to surgical treatment. Two cases, previously unresponsive to H2 antagonists, are presented showing a marked response to omeprazole.

PMID 1459354
S D Ladas, P S Tassios, H C Malamou, D P Protopapa, S A Raptis
Omeprazole induces a long-term clinical remission of protein-losing gastropathy of Ménétrier's disease.
Eur J Gastroenterol Hepatol. 1997 Aug;9(8):811-3.
Abstract/Text We report a patient with Ménétrier's disease presenting with extensive subcutaneous oedema, ascites and pleural effusion due to hypoalbuminaemia. Gastric secretory studies showed no free basal and stimulated acid secretion. The gastric juice contained significant amounts of albumin (0.2 g/dl) and immunoglobulin G (IgG) (1.11 mg/dl), corresponding to an estimated daily loss of 9.7 g and 45 mg, respectively. Protein-losing gastropathy was initially unsuccessfully treated with famotidine (80 mg/day) for 17 months, but a long-term (25 months) clinical remission was subsequently achieved with omeprazole (20 mg/day). We suggest that excellent clinical remission of Ménétrier's disease and the associated protein-losing gastropathy may be obtained with long-term omeprazole maintenance treatment, possibly due to Helicobacter pylori suppression.

PMID 9282281
M Vendelboe, J Jespersen
Hypertrophic protein-losing gastritis (Ménétrier's disease) treated with cimetidine.
Acta Med Scand. 1981;209(1-2):125-7.
Abstract/Text A man aged 46 developed Ménétrier's disease with massive hypertrophic gastritis and significantly elevated gastrointestinal protein clearance. During treatment with cimetidine the protein loss decreased markedly and the gastric mucosa normalized.

PMID 7211482
J Y Kang, K F Tang, A Goh, F X Sundram, C S Seah
Remission of Ménétrier's disease associated with ranitidine administration.
Aust N Z J Med. 1990 Oct;20(5):716-7.
Abstract/Text We describe a patient with Ménétrier's disease in whom acute administration of ranitidine reduced gastric protein loss more effectively than cimetidine or propantheline. This patient went into remission following a course of ranitidine. We reviewed the literature on remissions in Ménétrier's disease occurring without surgery. More detailed studies of various anti-secretory agents on individual patients are required to determine whether or not they are truly efficacious.

PMID 2285389
M Kondo, M Ikezaki, H Kato, M Masuda
Anti-fibrinolytic therapy of giant hypertrophic gastritis (Menetrier's disease).
Scand J Gastroenterol. 1978;13(7):851-6.
Abstract/Text In five cases of giant hypertrophic gastritis (Menetrier's disease) biopsied gastric mucosa was examined for fibrinolytic activity; in all cases there was marked elevation of the activity due mainly to tissue plasminogen activator. The patients were given antifibrinolytic therapy with oral tranexamic acid (trans-4-aminomethyl cyclohexane carboxylic acid; trans-AMCHA), and four of the patients showed marked improvement of their hypoproteinemia as well as their mucosal disorders. One patient, who showed moderate increase of serum protein level but no reduction of the mucosal disorder, finally received gastrectomy. It was concluded that antifibrinolytic therapy seemed to block the vicious circle of 'membrane disorders', 'increased tissue fibrinolysis', 'increased vascular permeability' and 'hypoproteinemia' in Menetrier's disease.

PMID 364627
G E Davis, M C O'Rourke, J R Metz, W V Kindig, J G Sweeney, K N Kane
Hypertrophic gastropathy symptoms responsive to prednisone. A case report and a review of the literature.
J Clin Gastroenterol. 1991 Aug;13(4):436-41.
Abstract/Text We describe a 33-year-old man with giant hypertrophic gastropathy (Menetrier's disease), which, on operative biopsy of the stomach, showed a significant inflammatory component. The patient was treated with prednisone and quickly responded. Although a review of the literature revealed only two cases similarly treated, with equivocal results, spontaneous remission, does occur. Symptoms associated with this disease may respond to corticosteroids, which may offer effective therapy while awaiting involution of the gastropathy. Thus total gastrectomy may be avoided.

PMID 1918850
M Geist, A Fich, P Mogle
Menetrier's disease: evolution of disease under histamine-2 receptor antagonists.
Am J Gastroenterol. 1992 May;87(5):648-50.
Abstract/Text A 33-yr-old man, with known peptic disease, developed giant thickening of the gastric mucosa and hypoproteinemia. Serial endoscopic and x-ray examinations of the upper gastrointestinal tract were available before and after the development of Menetrier's disease. In a 1-yr interval, erosive gastritis developed in a normal gastric mucosa, which was followed a few months later by hypoproteinemia. The patient developed the disease while being treated with histamine-2 receptor antagonists.

PMID 1595656
H W Scott, H J Shull, D H Law, H Burko, D L Page
Surgical management of Menetrier's disease with protein-losing gastropathy.
Ann Surg. 1975 May;181(5):765-77.
Abstract/Text Three patients with Menetrier's disease and protein-losing gastropathy who were studied during a 12 year period have been presented. The characteristic findings which differentiate them from patients with hypertrophic hypersecretory gastropathy, including the Zollinger-Ellison syndrome, are: 1) hypertrophy of gastric mucosa with giant rugal folds involving the fundus, cardia and body of the stomach but sparing the antrum; 2) muscosal hypertrophy consisting of gastric mjcus-secreting cells while parietal cells and chief cells are diminished in number and may be absent from many microscopic sections; 3) gastric secretion of large volume containing excess mucus, low to absent hydrochloric acid and protein concentration 5 or 6 times normal (1.7 mg/ml); 4) hypoalbuminemia and hypoglobulinemia due to loss of serum proteins fron gastric mucosa into the gastric lumen; 5) rare association with gastric ulcer. Unlike the Zollinger-Ellison syndrome none of our patients had duodenal ucler or multiple endocrine adenomatosis or a family history of these conditions. We have found no authenticated reports in the literature which document a relationship of Menetrier's disease ( as defined above) with multiple endocrine adenomatosis. Menetrier's disease with protein-losing gastropathy is a potentially lethal disorder of unknown cause with no specific treatment. Resection of the site of gastric protein losses as first done by Waugh is logical and effective. One of our three patients died in hospital before gastrectomy was done. Two others have done well for 11 months and 12 years, respectively, after total gastrectomy with Roux-en-Y esophagojejunostomy and Hunt-Lawrence jejunal pouch.

PMID 1130890
T M Sundt, C C Compton, R A Malt
Ménétrier's disease. A trivalent gastropathy.
Ann Surg. 1988 Dec;208(6):694-701.
Abstract/Text Current conceptions of Ménétrier's disease only obliquely resemble those originally described. Bona-fide cases are so uncommon that, of 125 cases diagnosed as Ménétier's disease, hypertrophic gastritis, or protein-losing gastropathy treated at the Massachusetts General Hospital during the 26-year period of 1962-1987, only six cases merited an unequivocal anatomic diagnosis. Two other cases previously described proved on review to be nondiagnostic in one instance and Campylobacter pylori gastritis in the other. Because abnormalities in the secretion of gastric acid and in the loss of protein from the stomach may coexist, a representation of each case in semiquantitative terms can be described on triaxial coordinates. Three patients had a hypercoagulable state, one in association with gastric carcinoma. One other case of gastric carcinoma and one of esophageal carcinoma coexistant with Ménétrier's disease were identified. Administration of subcutaneous heparin during the perioperative period to patients with Ménétrier's disease is appropriate regardless of whether or not hypercoagulation or carcinoma is manifest. If treatment with anticholinergic drugs and inhibitors of gastric acid secretion fails, total gastrectomy is the best solution, because it stops protein loss, eliminates hyperchlorhydria, prevents development of gastric carcinoma, and permits anastomotic reconstruction between normal esophagus and normal small bowel.

PMID 3264139
J S Burdick, E Chung, G Tanner, M Sun, J E Paciga, J Q Cheng, K Washington, J R Goldenring, R J Coffey
Treatment of Ménétrier's disease with a monoclonal antibody against the epidermal growth factor receptor.
N Engl J Med. 2000 Dec 7;343(23):1697-701. doi: 10.1056/NEJM200012073432305.
Abstract/Text
PMID 11106719
Stephen H Settle, Kay Washington, Christopher Lind, Scott Itzkowitz, W Haley Fiske, J Steven Burdick, W Gray Jerome, Margaret Ray, Wilfred Weinstein, Robert J Coffey
Chronic treatment of Ménétrier's disease with Erbitux: clinical efficacy and insight into pathophysiology.
Clin Gastroenterol Hepatol. 2005 Jul;3(7):654-9.
Abstract/Text BACKGROUND & AIMS: Ménétrier's disease is a rare premalignant hypertrophic gastropathy characterized by large rugal folds, foveolar hyperplasia with glandular atrophy, hypochlorhydria, and hypoalbuminemia. Patients with severe disease often exhibit refractory nausea and vomiting and require gastrectomy. Evidence from both mice and human beings suggests a critical role for epidermal growth factor receptor (EGFR) signaling in the pathogenesis of this disease. We previously reported significant clinical and biochemical improvement of a single patient treated for 1 month with Erbitux, a monoclonal antibody that blocks ligand binding to EGFR.
METHODS/RESULTS: We describe 2 patients who were given longer-term treatment with Erbitux as an alternative to gastrectomy. The first patient presented with nausea, hypoalbuminemia, and peripheral edema that required total parenteral nutrition (TPN) and infusions of albumin. On institution of Erbitux, there was rapid improvement in nausea and vomiting and stabilization of serum albumin with discontinuation of TPN and albumin infusions. Serum albumin remained stable during a 1-year course of Erbitux without supplemental protein. Application before and after Erbitux of the radiopaque dye ruthenium red to biopsies of the gastric oxyntic gland mucosa demonstrated prompt and persistent closure of tight junctions by electron microscopy. The second patient presented with chronic gastric bleeding that required bimonthly blood transfusions. During a 4-month course of Erbitux, his hematocrit stabilized, and transfusion requirements were eliminated.
CONCLUSIONS: The present report demonstrates the efficacy of prolonged Erbitux therapy in patients with different presentations of severe Ménétrier's disease and also provides insight into the pathophysiology of the protein-losing gastropathy.

PMID 16206497
D M Russell, P S Bhathal, D J St John
Complete remission in Cronkhite-Canada syndrome.
Gastroenterology. 1983 Jul;85(1):180-5.
Abstract/Text A case of Cronkhite-Canada syndrome, where there was complete remission confirmed by radiology, endoscopy, and histology, is reported. On presentation, there was generalized gastrointestinal polyposis, hypoalbuminemia, skin pigmentation, onycho-dystrophy, and alopecia. Enteral nutrition alone was administered for 10 wk, with resolution of the ectodermal features and disappearance of the polyps within 4 mo of commencement of the treatment. This report of complete remission supports the generally accepted nonneoplastic and essentially inflammatory nature of the polyps in Cronkhite-Canada syndrome. Consideration needs to be given to nutritional deficiency as a cause of the syndrome.

PMID 6406289
Rajagopal Chadalavada, D Kaine Brown, Anna N Walker, Shahriar Sedghi
Cronkhite-Canada syndrome: sustained remission after corticosteroid treatment.
Am J Gastroenterol. 2003 Jun;98(6):1444-6. doi: 10.1111/j.1572-0241.2003.07509.x.
Abstract/Text
PMID 12818298
Chikako Watanabe, Shunsuke Komoto, Kengo Tomita, Ryota Hokari, Masanori Tanaka, Ichiro Hirata, Toshifumi Hibi, Jonathan D Kaunitz, Soichiro Miura
Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite-Canada syndrome: a Japanese nationwide survey.
J Gastroenterol. 2016 Apr;51(4):327-36. doi: 10.1007/s00535-015-1107-7. Epub 2015 Jul 28.
Abstract/Text BACKGROUND: First reported in 1955, Cronkhite-Canada syndrome (CCS), a rare syndrome characterized by ectodermal abnormalities and inflammatory changes of the gastrointestinal tract mucosa, has been associated with a poor prognosis and life-threatening malignant complications. In a large population survey, we endeavored to characterize the course and treatment outcome of CCS through clinical and endoscopic assessment, and to explore its optimal treatment and surveillance strategy.
METHODS: A retrospective analysis of 210 patients with CCS was conducted via a questionnaire-based nationwide survey of 983 teaching hospitals located throughout Japan. We assessed clinical features, endoscopic findings, treatments used, and short- and long-term outcomes.
RESULTS: The average age at diagnosis was 63.5 years. In all cases, upper or lower gastrointestinal tract polyposis was confirmed, accompanied by characteristic ectodermal abnormalities. Of the treatments used, oral corticosteroids (30-49 mg/day) were the most effective treatment for active disease, with adjunctive nutritional support considered beneficial. With corticosteroid treatment, abdominal symptoms were relieved within a few months, whereas polyp regression often required more than 6 months. Maintenance of endoscopic remission with or without steroids for 3 years significantly lowered the development of CCS-related cancer, compared with relapsers or nonresponders, underscoring the importance of sustained endoscopic remission for cancer prevention.
CONCLUSIONS: The prognosis of CCS has greatly improved through the use of improved medical treatment. Although CCS continues to be relentlessly progressive, carrying a high cancer risk, a sufficient dose and duration of corticosteroid therapy accompanied by nutritional support and periodic endoscopic surveillance appears to improve its natural history.

PMID 26216651
Kenta Okamoto, Hajime Isomoto, Saburo Shikuwa, Hitoshi Nishiyama, Masahiro Ito, Shigeru Kohno
A case of Cronkhite-Canada syndrome: remission after treatment with anti-Helicobacter pylori regimen.
Digestion. 2008;78(2-3):82-7. doi: 10.1159/000165354. Epub 2008 Oct 24.
Abstract/Text A 67-year-old man with nausea, appetite loss, frequent diarrhea and severe weight loss presented with alopecia, skin hyperpigmentation and onychodystrophy. Laboratory investigations showed mild anemia, hypoproteinemia and hypoalbuminemia. Colonoscopy identified the numerous, hyperemic and sessile polyps with mucous exudation of various sizes throughout the colorectum. The ileocecal valve was substantially swollen. Magnified chromoendoscopy revealed sparsely distributed crypt openings with widening of the preicryptal space without destruction in the affected lesions. Upper gastrointestinal endoscopy revealed multiple small, reddish, and sessile polyps in the duodenum and Helicobacter pylori-associated gastritis. Histopathological examination of the colonic polyps revealed cystic dilatation and elongation of scattered glands with epithelial hyperplasia and stromal edema and inflammatory cell infiltrates. Thus, a diagnosis of Cronkhite-Canada syndrome was made. The patient was given clarithromycin, amoxicillin and lansoprazole, resulting in negative (13)C-urea breath tests. Three months later, his clinical symptoms and edema of the legs resolved with normalization of serum total protein and albumin levels and return to his previous body. The ectodermal abnormalities were resolved 8 months later. On repeat colonoscopic examinations, there was progressive remission of the duodenal and colorectal polyposis, leaving scattered pedunculated polyps in the transverse and ascending colon and on the almost normal-appearing ileocecal valve. At the follow-up magnifying endoscopic examination 8 months later, small round or round-oval pits were densely and regularly distributed.

Copyright 2008 S. Karger AG, Basel.
PMID 18948692
Renan Bazuco Frittoli, Jéssica Fernandes Vivaldo, Lilian Tereza Lavras Costallat, Simone Appenzeller
Gastrointestinal involvement in systemic lupus erythematosus: A systematic review.
J Transl Autoimmun. 2021;4:100106. doi: 10.1016/j.jtauto.2021.100106. Epub 2021 Jun 10.
Abstract/Text Introduction: Gastrointestinal involvement is a common complain observed in 40-60% of systemic lupus erythematosus (SLE) patients. We performed a systematic review of clinically severe and potential life-threatening gastrointestinal manifestations and discuss clinical presentation, pathogenesis and treatment.
Methods: We performed a literature search in English literature using PubMed and Embase from 2000 to December 2020. The following MeSH terms: systemic lupus erythematosus, protein-losing enteropathy, ascites, pancreatitis, vasculitis, intestinal vasculitis, enteritis and diarrhea published in the English literature.
Results: We identified 141 studies (case reports, case series and cohort studies). The most frequent presenting symptoms are acute abdominal pain, nausea, and vomiting. Many of the manifestations were associated with disease activity. Histological features are rarely available, but both vasculitis and thrombosis have been described. There is no treatment guideline. The majority of patients were treated with corticosteroids and the most common immunososupressant were azathioprine, cyclophosphamide and mycophenolate.
Conclusion: Vasculitis and thrombosis may be responsible for severe life-threatening manifestations such as pancreatitis, protein loosing gastroenteritis, acalculous cholecistyitis and enteritis.

© 2021 The Authors. Published by Elsevier B.V.
PMID 34179742
Xin-Ping Tian, Xuan Zhang
Gastrointestinal involvement in systemic lupus erythematosus: insight into pathogenesis, diagnosis and treatment.
World J Gastroenterol. 2010 Jun 28;16(24):2971-7. doi: 10.3748/wjg.v16.i24.2971.
Abstract/Text Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by SLE. Gastrointestinal symptoms are common in SLE patients, and more than half of them are caused by adverse reactions to medications and viral or bacterial infections. Though not as common as lupus nephritis, SLE-related gastrointestinal involvement is clinically important because most cases can be life-threatening if not treated promptly. Lupus mesenteric vasculitis is the most common cause, followed by protein-losing enteropathy, intestinal pseudo-obstruction, acute pancreatitis and other rare complications such as celiac disease, inflammatory bowel diseases, etc. No specific autoantibody is identified as being associated with SLE-related gastroenteropathy. Imaging studies, particularly abdominal computed tomography scans, are helpful in diagnosing some SLE-related gastroenteropathies. Most of these complications have good therapeutic responses to corticosteroids and immunosuppressive agents. Supportive measures such as bowel rest, nutritional support, antibiotics and prokinetic medications are helpful in facilitating functional recovery and improving the outcome.

PMID 20572299
A Amiot
[Protein-losing enteropathy].
Rev Med Interne. 2015 Jul;36(7):467-73. doi: 10.1016/j.revmed.2014.12.001. Epub 2015 Jan 21.
Abstract/Text Protein-losing enteropathy is a rare syndrome of gastrointestinal protein loss. The primary causes can be classified into lymphatic leakage due to increased interstitial pressure and increased leakage of protein-rich fluids due to erosive or non-erosive gastrointestinal disorders. The diagnosis of protein-losing enteropathy should be considered in patients with chronic diarrhea and peripheral oedema. The diagnosis of protein-losing enteropathy is most commonly based on the determination of fecal alpha-1 antitrypsin clearance. Most protein-losing enteropathy cases are the result of either lymphatic obstruction or a variety of gastrointestinal disorders and cardiac diseases, while primary intestinal lymphangiectasia (Waldmann's disease) is less common. Treatment of protein-losing enteropathy targets the underlying disease but also includes dietary modification, such as high-protein and low-fat diet along with medium-chain triglyceride supplementation.

Copyright © 2014 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.
PMID 25618488
Maxim Itkin, David A Piccoli, Gregory Nadolski, Jack Rychik, Aaron DeWitt, Erin Pinto, Jonathan Rome, Yoav Dori
Protein-Losing Enteropathy in Patients With Congenital Heart Disease.
J Am Coll Cardiol. 2017 Jun 20;69(24):2929-2937. doi: 10.1016/j.jacc.2017.04.023.
Abstract/Text BACKGROUND: Protein-losing enteropathy (PLE), characterized by loss of proteins in the intestine, is a devastating complication in patients with congenital heart disease. The cause of PLE is unknown, but lymphatic involvement has been suspected.
OBJECTIVES: The authors evaluated the use of lymphangiographic imaging and liver lymphatic embolization as a treatment for PLE.
METHODS: This was a single-center, retrospective review of imaging and interventions used in 8 consecutive patients with liver lymphatic embolization and congenital heart disease with elevated central venous pressure complicated by PLE.
RESULTS: Liver lymphangiography was performed in 8 patients (5 males, 3 females; median age, 21 years), 7 of whom demonstrated leakage of liver lymph into the duodenum through abnormal hepatoduodenal lymphatic communications. This was confirmed by duodenoscopy with simultaneous injection of isosulfan blue dye into the liver lymphatics in 6 of 7 patients. Liver lymphatic embolization with ethiodized oil in 2 patients resulted in a temporary increase in albumin blood level and symptom improvement in 1 patient, but was complicated by duodenal bleeding in both patients. Of the remaining 6 patients, liver lymphatic embolization with n-butyl cyanoacrylate glue resulted in sustained improvement of the serum albumin level and symptoms in 3 patients, temporary improvement in 2 patients, and no change in 1 patient with median follow-up of 135 days (range, 84 to 1,005 days).
CONCLUSIONS: The authors demonstrated liver lymph leakage as a cause of PLE in patients with congenital heart disease and elevated central venous pressure. Lymphatic embolization led to improved albumin levels and relief of symptoms. Further experience with the technique is needed to determine long-term outcome of this procedure.

Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
PMID 28619193

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