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Cave DR, Hanscom M, Bledsoe J, Madahian S, Hyatt B, Marya N, Baptista V, Schneider D, Reed G, Albo B, Min M, Barton B. Chronic Stenosing Enteritis: A Variant of Chronic Non-specific Stenosing Ulceration (CNSU) that Is Distinct from Crohn's Disease. Dig Dis Sci 2025; 70:1449-1456. [PMID: 39985738 DOI: 10.1007/s10620-024-08815-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/07/2024] [Accepted: 12/18/2024] [Indexed: 02/24/2025]
Abstract
OBJECTIVE Chronic non-specific stenosing ulcers (CNSU) of the small intestine is an under-recognized syndrome characterized by iron-deficiency anemia, superficial ulcerations, and stenoses of the small intestine. Despite the recent identification of a gene mutation SLCO2A1 in some Japanese patients that plays an etiological role, much remains uncertain about the etiology and pathogenesis of CNSU in the Western Hemisphere. We report a similar pattern of non-specific ulceration that is nontransmural and often associated with small intestinal stenosis and iron deficiency but not hypoalbuminemia, and that appears to be distinct from Crohn's disease, and compare the demographic, clinic, and histopathologic features. METHODS This was a retrospective, single-center study performed at a tertiary care hospital between 2007 and 2019. Forty patients were included, of whom 20 were diagnosed with CNSU and 20 with small intestinal CD. Demographic, clinical, and histopathologic data were collected and compared. RESULTS Patients with CNSU were significantly older than patients with CD (56.9-years vs. 33.6-years, p < 0.0001), and had significantly lower rates of diarrhea (10% vs 90%; p < 0.01) and weight loss (5% vs 40%; p = 0.005) and greater rates of blood transfusions (50% vs 10%, p = 0.005) and iron infusions (35% vs. 0%, p = 0.001). In addition, qualitative descriptions of endoscopic findings and histopathologic features differed between the two groups. CONCLUSION CNSU is an uncommon small intestinal disease with clinical and pathologic features that distinguish it from CD. However, the immunology of both conditions is similar, suggesting a generic immune response. Further research is needed to better define the pathogenesis and prognosis of the disease.
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Affiliation(s)
- David R Cave
- Department of Medicine, Brigham & Women's Hospital, Boston, MA, USA
| | - Mark Hanscom
- Department of Medicine, Weill Cornell Medicine, 1283 York Ave., 9Th Floor, New York, NY, 10065, USA.
| | - Jacob Bledsoe
- Department of Pathology, Boston Children's Hospital, Boston, MA, USA
| | - Sepideh Madahian
- Department of Pathology, UMass Memorial Medical Center, Worcester, MA, USA
| | | | - Neil Marya
- Department of Medicine, UMass Memorial Medical Center, Worcester, MA, USA
| | - Veronica Baptista
- Department of Medicine, UMass Memorial Medical Center, Worcester, MA, USA
| | - Douglas Schneider
- Department of Pathology, Steward St. Elizabeth's Hospital, Brighton, MA, USA
| | - George Reed
- Department of Medicine, UMass Memorial Medical Center, Worcester, MA, USA
| | - Betty Albo
- Department of Medicine, UMass Memorial Medical Center, Worcester, MA, USA
| | - May Min
- Department of Medicine, Brown University Rhode Island Hospital, Providence, RI, USA
| | - Bruce Barton
- Department of Quantitative Health Sciences, UMass Memorial Medical Center, Worcester, MA, USA
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Singh A. Cryptogenic Multifocal Ulcerating Stenosing Enteropathy(CMUSE) and/or Chronic Non-specific Multiple Ulcers of the Small Intestine(CNSU) and Non-granulomatous Ulcerating Jejunoileitis (NGUJI). Curr Gastroenterol Rep 2019; 21:53. [PMID: 31501950 DOI: 10.1007/s11894-019-0721-6] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/10/2023]
Abstract
PURPOSE OF REVIEW The purpose is to make aware of the existence of the rare and exclusive small intestine (SI) diseases, namely cryptogenic multifocal ulcerating stenosing enteropathy (CMUSE) or chronic non-specific multiple ulcers of the small intestine (CNSU) and non-granulomatous ulcerating jejunoileitis (NGUJI). The article will elucidate their epidemiology, pathogenesis, clinical features, diagnosis, differentiating features and management. RECENT FINDINGS Recent papers have published the clinical features and diagnostic criteria of CMUSE/CNSU and NGUJI. CNSU/CMUSE is caused by gene mutations involved in the prostaglandin pathways. Although capsule endoscopy can detect these lesions, it carries a risk of retention. TNF antagonists and azathioprine have shown response in few cases. CMUSE/CNSU and NGUJI are uncommon diseases that cause relapsing SI obstruction and bleed due to short-segment strictures and multiple shallow ulcers. This article focuses on current knowledge and novel insights regarding their pathogenesis, genetics, clinical features, diagnostic criteria and management. Multicentric clinical and genetic studies are the need of the hour.
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Affiliation(s)
- Ayaskanta Singh
- Department of Gastroenterology and Hepatobiliary Sciences, IMS and SUM Hospital, Siksha 'O' Anusandhan, deemed to be University, Kalinga Nagar, Bhubaneswar, Orissa, 751003, India.
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3
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Gao X, Wang ZJ. Idiopathic chronic ulcerative enteritis with perforation and recurrent bleeding: A case report. World J Gastroenterol 2013; 19:4836-4840. [PMID: 23922486 PMCID: PMC3732861 DOI: 10.3748/wjg.v19.i29.4836] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/31/2013] [Revised: 06/04/2013] [Accepted: 07/18/2013] [Indexed: 02/06/2023] Open
Abstract
Idiopathic chronic ulcerative enteritis (ICUE) is a distinct entity without a defined etiology and is rarely seen in the clinic. Patients with ICUE mainly present with insidious abdominal symptoms such as chronic abdominal pain and intermittent gastrointestinal hemorrhage and symptoms of malnourishment in the early stages of the disease. ICUE is always difficult to diagnose. However, as the disease progresses, patients have a variety of acute abdominal complications such as hemorrhage, perforation, or ileus. Surgical intervention is always needed, and the condition can recur and require repeat laparotomy. When diffuse ulceration of the small bowel is present in the absence of recognizable causes, it is classified as nonspecific or idiopathic. The histological examination always demonstrates an acute, chronic inflammatory infiltration without giant cells, granulomas, or villous atrophy. The etiology of ICUE has not been identified, and its pathogenesis is poorly understood; therefore, radical surgical resection is considered the best available treatment. Here, we report a case of ICUE characterized by nonspecific, multiple, small intestinal ulcers resulting in perforation and recurrent bleeding. The differential diagnosis and the treatment are also discussed.
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Matsumoto T, Kubokura N, Matsui T, Iida M, Yao T. Chronic nonspecific multiple ulcer of the small intestine segregates in offspring from consanguinity. J Crohns Colitis 2011; 5:559-65. [PMID: 22115375 DOI: 10.1016/j.crohns.2011.05.008] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/06/2011] [Revised: 05/20/2011] [Accepted: 05/21/2011] [Indexed: 02/08/2023]
Abstract
BACKGROUND AND AIMS Chronic nonspecific multiple ulcer of the small intestine is a recently proposed enteropathy characterized by persistent blood and protein loss from the small-bowel. We examined possible segregation of the disease in family pedigrees. METHODS All cases of the disease diagnosed at our institution were reviewed with respect to particular focuses on the presence of close consanguinity in the families, the enteroscopic findings and the long-term clinical course. The diagnosis was based on persistent occult gastrointestinal bleeding and hypoproteinemia for more than 5 years, and irregularly shaped shallow ulcers in the ileum. RESULTS During a 45-year-period, 13 patients were diagnosed as having the disease. There were 11 females and 2 males, with ages ranging from 8 to 37 years at the time of the initial presentation and with those from 13 to 38 years at the diagnosis. Enteroscopy performed in 11 patients with a time duration ranging from 0.5 to 44 years after the diagnosis revealed active ileal ulcers in 10 patients. Parents' consanguineous marriage was verified in 6 patients, two of whom also had siblings with the enteropathy. Another patient without consanguinity had a sibling with protein-losing enteropathy. CONCLUSION Chronic nonspecific multiple ulcer of the small intestine seems to segregate in offspring from consanguineous marriage.
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Affiliation(s)
- Takayuki Matsumoto
- Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
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5
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Wang W, Wang Z, Yang Y, Linghu E, Lu Z. Long-term follow-up of nonspecific small bowel ulcers with a benign course and no requirement for surgery: is this a distinct group? BMC Gastroenterol 2011; 11:51. [PMID: 21569268 PMCID: PMC3103450 DOI: 10.1186/1471-230x-11-51] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/01/2010] [Accepted: 05/10/2011] [Indexed: 12/17/2022] Open
Abstract
BACKGROUND Nonspecific small bowel ulcers are rare and surgical intervention is often believed to be elective. Since the extensive investigation of the small bowel in the 1990s, there have been limited reports of these ulcers and the updates have been unsatisfactory. The aim of this study was to explore the clinical features and natural histories of nonspecific small bowel ulcers through prospective observational study. METHODS We reviewed the medical records of all patients who had undergone ileocolonoscopy or enteroscopy between 2000 and 2005 in a tertiary referral hospital. Seven patients with small bowel ulcers of unknown cause were identified. These patients were prospectively followed in a prolonged observation until March 2010. RESULTS All seven patients (mean age: 54.7 years) presented with mild gastrointestinal symptoms, including chronic diarrhea and/or abdominal pain/discomfort, except for one who was asymptomatic when surveyed for colon polyps. Most patients were suspected of having functional bowel disorders for a long time (4.4 years) before small bowel ulcers were demonstrated on ileoscopy. The ulcers were characteristically multiple, superficial, and small (3-6 mm), locating at the terminal ileum and/or ileocecal valve. Various empirical treatments were applied, and most patients felt partly improved, even relieved. However the gastrointestinal symptoms did not always correlate with the presence of ulcers, and the ulcers tended to be either persistent (4 patients) or recurrent (1 patient). Ileocolonoscopy was repeated 4.1 times during follow-up, even after the lesions had healed. The characteristics of the ulcers, if still present during follow-up, were similar to their earlier characteristics. No patient experienced exacerbation or complications, such as intestinal obstruction, perforation, or bleeding. All patient survived and no surgical intervention was involved during the prolonged follow-up (7.0 years). CONCLUSION The reported patients with nonspecific small bowel ulcers experienced benign courses, inconsistent with previous reports. Without extensive investigation, this disease can be confused with functional bowel disorders.
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Affiliation(s)
- Weifeng Wang
- Department of Gastroenterology and Hepatology, Chinese PLA General Hospital & Chinese PLA Postgraduate Medical School, Beijing 100853, China
| | - Zhanbo Wang
- Department of Pathology, Chinese PLA General Hospital, Beijing 100853, China
| | - Yunsheng Yang
- Department of Gastroenterology and Hepatology, Chinese PLA General Hospital & Chinese PLA Postgraduate Medical School, Beijing 100853, China
| | - Enqiang Linghu
- Department of Gastroenterology and Hepatology, Chinese PLA General Hospital & Chinese PLA Postgraduate Medical School, Beijing 100853, China
| | - Zhongsheng Lu
- Department of Gastroenterology and Hepatology, Chinese PLA General Hospital & Chinese PLA Postgraduate Medical School, Beijing 100853, China
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Matsumoto T, Iida M, Matsui T, Yao T, Watanabe H, Yao T, Okabe H. Non-specific multiple ulcers of the small intestine unrelated to non-steroidal anti-inflammatory drugs. J Clin Pathol 2004; 57:1145-50. [PMID: 15509673 PMCID: PMC1770471 DOI: 10.1136/jcp.2003.015735] [Citation(s) in RCA: 61] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
AIM Non-steroidal anti-inflammatory drug (NSAID) enteropathy is a cause of chronic blood and protein loss from the intestine. The aim of this investigation was to compare NSAID enteropathy with enteropathy not related to chronic NSAID use. PATIENTS/METHODS During the period 1967-97, 12 cases of non-specific small intestinal multiple ulcers were laparotomised because of chronic and persistent blood loss for a prolonged period. The clinical features before and after surgery, and the small intestinal lesions, were compared between NSAID users and non-users. RESULTS Three patients were NSAID users and the other nine were not. Initial clinical manifestations, haemoglobin concentrations, serum protein values, and inflammatory reactions were similar in the two groups. In both groups there were multicentric stenoses in the small intestine. In those not using NSAIDs, the stenoses were accompanied by multiple, sharply demarcated small ulcers with minimal and non-specific chronic inflammatory infiltrates. The small intestinal ulcers in NSAID users showed linear and circumferential alignment. NSAID users were free from medication during the subsequent period of observation, whereas repeated laparotomy and medication were required in eight of the nine patients not using NSAIDs. CONCLUSION There appears to be a chronic enteropathy, not related to the use of NSAIDs, in which non-specific multiple ulcers are found. This enteropathy may be a distinct entity that causes persistent blood and protein loss from the intestine.
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Affiliation(s)
- T Matsumoto
- Department of Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan.
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Cervoni JP, Brisset F, Larvol L, Levecq H, Damade R. [Ascites and emaciation]. Rev Med Interne 2000; 21 Suppl 3:350s-355s. [PMID: 10916852 DOI: 10.1016/s0248-8663(00)89266-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/17/2022]
Affiliation(s)
- J P Cervoni
- Service de gastroentérologie, hôpital Fontenoy, Chartres
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Besnard M, Peuchmaur M, Mougenot JF, Aigrain Y, Faure C, Navarro J, Cezard JP. Extensive hyperplastic and ulcerative mucosal enteritis in a child requiring total enterectomy. J Pediatr Gastroenterol Nutr 1999; 28:222-6. [PMID: 9932863 DOI: 10.1097/00005176-199902000-00028] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Affiliation(s)
- M Besnard
- Pediatric Gastroenterology Department, Hôpital Robert Debré, Paris, France
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Abstract
Coeliac disease (CD) is a disorder of the small intestine, characterized by villous atrophy, due to an intolerance to dietary gluten in genetically susceptible individuals, which responds to gluten withdrawal. The underlying immunological mechanisms causing the disorder are still being worked out. In recent years a wide range of clinical presentations has become increasingly apparent, as has a lengthening list of associated conditions. Severe malabsorption with steatorrhoea and profound weight loss is seen infrequently, perhaps as a result of earlier diagnosis and the recognition of 'silent' and 'latent' disease. The prevalence of CD as judged by population screening with, in particular, anti-endomysial antibodies, appears to be much higher than that found with clinically apparent cases. There are a variety of well recognized complications, the commonest probably being osteopenia and osteoporosis. The marked increased risk of lymphoma can be avoided by a strict gluten-free diet. Follow-up of patients needs to be lifelong with prompt investigation of new symptoms and blood test abnormalities.
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Affiliation(s)
- N D Parnell
- Department of Gastroenterology, The Rayne Institute, St Thomas' Hospital, London, UK
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10
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Koberstein B, Wedell J, Balzer K. [Acute abdomen in endemic sprue--a rare complication]. MEDIZINISCHE KLINIK (MUNICH, GERMANY : 1983) 1998; 93:43-6. [PMID: 9505079 DOI: 10.1007/bf03045040] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
BACKGROUND The celiac sprue, a small intestinal disease usually becoming apparent by a malabsorption syndrome, is caused by gluten respectively gliadin intolerance which leads to intestinal mucosal damage finally resulting in complete villous atrophy. CASE REPORT A propos of the case of a 60-year old woman with an uneventful course of celiac sprue of many years we report about a rare complication of this disease. Investigating recurrent attacks of abdominal pain a barium contrast examination revealed an intestinal stenosis which clinically resulted in an acute abdomen finally requiring surgery. Histologically a chronic ulcerative jejunoileitis was found. The main differential diagnosis is a malignant intestinal lymphoma, this however couldn't be found. Regarding this differential diagnosis surgical removal of the diseased parts of the bowel is the therapy of choice. Because of the possible transition from ulcerative jejunoileitis to intestinal lymphoma a postoperative close follow-up is recommended.
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Costello RW, Lyons DJ, Fielding JF. Ulcerative jejunitis: are we missing cases? Ir J Med Sci 1991; 160:342-3. [PMID: 1810901 DOI: 10.1007/bf02957890] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
We present two cases of ulcerative jejunitis unassociated with coeliac disease. The condition is probably being underdiagnosed especially since the reduction in the investigative laparotomy.
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Affiliation(s)
- R W Costello
- Department of Medicine and Gastroenterology, Beaumont Hospital, Dublin, Ireland
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12
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Roehrkasse RL, Roberts IM, Wald A, Talamo TS, Mendelow H. Celiac sprue complicated by lymphoma presenting with multiple gastric ulcers. Gastroenterology 1986; 91:740-5. [PMID: 3732772 DOI: 10.1016/0016-5085(86)90648-7] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/07/2023]
Abstract
A 40-yr-old woman with celiac sprue, which had responded clinically and histologically to gluten elimination, subsequently developed gastrointestinal lymphoma. Although this has been described in the small intestine of patients with celiac sprue, the unique feature in this patient was her initial presentation with multiple gastric ulcers refractory to conventional medical therapy. This case demonstrates that lymphoma complicating celiac sprue may present with multiple refractory gastric ulcers in addition to those occurring in the small intestine.
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Börsch G, Jahnke A, Bergbauer M, Nebel W. Solitary nonspecific ileal ulcer. Diagnosis by coloileoscopy in a patient with previously assumed irritable bowel syndrome. Dis Colon Rectum 1983; 26:732-5. [PMID: 6628147 DOI: 10.1007/bf02554985] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Abstract
We present a case of solitary nonspecific ileal ulcer found by coloileoscopy in a patient with previously assumed irritable bowel syndrome. Follow-up endoscopies two weeks after initiation of short-term prednisone therapy, and again four months later, demonstrated rapid and persistent healing. This observation raises the question of whether or not primary ileal ulcers are indeed as rare as previously assumed when only surgical and autopsy findings were taken into consideration. Also, the natural history of this clinical entity, in general, could be somewhat more benign than suggested by those ulcers in which complications make surgery necessary, since these cases may not adequately reflect the full clinical spectrum of nonspecific small-bowel ulcers.
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Abstract
Two patients with idiopathic chronic ulcerative enteritis (ICUE) having malabsorption, subtotal villous atrophy and non-specific small intestinal ulceration are presented with emphasis on the radiological features. No improvement was shown morphologically following gluten withdrawal. The literature on this condition is reviewed. Obstruction, perforation and melaena are serious complications and the prognosis is poor; only 11 out of 31 reported cases survived. Differentiation from coeliac disease, Crohn's disease and primary intestinal lymphoma is discussed.
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Kavin H. Celiac disease complicated by chronic nongranulomatous ulcerative enterocolitis, nodular lymphoid hyperplasia, and disseminated intravascular coagulation. Dig Dis Sci 1981; 26:73-80. [PMID: 7460709 DOI: 10.1007/bf01307978] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
A patient is presented in whom celiac disease was complicated by the development of chronic nongranulomatous ulcers in the small and large intestine. In addition, he had nodular lymphoid hyperplasia in the bowel wall and mesenteric lymphoadenopathy. The terminal illness was characterized by disseminated intravascular coagulation. It is postulated that the lymphoid hyperplasia was secondary to excessive stimulation of the reticuloendothelial system by nongluten antigens and may be a precursor of the development of lymphoma in celiac disease. Nongluten antigens such as bacteria or endotoxins were probably responsible for the consumptive coagulopathy.
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