• We are sorry, but NCBI web applications do not support your browser and may not function properly. More information
Logo of gutGutCurrent TOCInstructions to authors
Gut. Mar 2001; 48(3): 333–338.
PMCID: PMC1760126

Cryptogenetic multifocal ulcerous stenosing enteritis: an atypical type of vasculitis or a disease mimicking vasculitis

Abstract

BACKGROUND/AIMS—Cryptogenetic multifocal ulcerous stenosing enteritis (CMUSE) is a rare disease whose origin is unknown. The aim of this study was to describe the clinical spectrum of CMUSE, to determine the origin and pathophysiology of the disease, and to propose a treatment strategy.
METHODS—A total of 220 French gastroenterology departments were contacted to review patients with unexplained small bowel strictures. Of 17 responses, 12 corresponded to a diagnosis of CMUSE. These patients were hospitalised between 1965 and 1993 and their medical records were reviewed.
RESULTS—All patients (mean age 42.1 (4.4) years) had intestinal and five had extraintestinal symptoms (peripheral neuropathy, buccal aphthae, sicca syndrome, polyarthralgia, Raynaud's phenomenon, arterial hypertension). One patient had heterozygous type I C2 deficiency (28 base pair gene deletion). Two to 25 (mean 8.3 (1.9)) small intestine strictures were found. Stenoses of the large jejunoileal arteries were observed on two and aneurysms on three of five mesenteric angiograms. Despite surgery, symptoms recurred in seven of 10 patients and strictures in four. Steroid therapy was effective but caused dependence. One untreated patient died. Small bowel pathology showed superficial ulceration of the mucosae and submucosae, and an inflammatory infiltrate made of neutrophils and eosinophils.
CONCLUSIONS—CMUSE is an independent entity characterised by steroid sensitive inflammation of the small bowel which often recurs after surgery. CMUSE may be related to a particular form of polyarteritis nodosa with mainly intestinal expression or with an as yet unclassified vasculitis.


Keywords: cryptogenetic multifocal ulcerous stenosing enteritis; vasculitis; small intestine

Full Text

The Full Text of this article is available as a PDF (145K).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Jeffries GH, Steinberg H, Sleisenger MH. Chronic ulcerative (nongranulomatous) jejunitis. Am J Med. 1968 Jan;44(1):47–59. [PubMed]
  • DEBRAY C, BESANCON F, HARDOUIN JP, MARTIN E, MARCHE C, KHOURY K. ENT'ERITE ST'ENOSANTE ULC'EREUSE PLURIFOCALE CRYPTOG'EN'ETIQUE. Arch Mal Appar Dig Mal Nutr. 1964 Mar;53:193–206. [PubMed]
  • ROCHA A, ARTIGAS V. [Stenosing ulcerous disease of the jejuno-ileum]. Arch Mal Appar Dig Mal Nutr. 1959 Oct;48:1230–1236. [PubMed]
  • Gauthier AP, Rampal M, Lieutaud R, Sarles H. Sténoses multiples "idiopathiques" de l'intestin grêle. A propos de deux observations. Arch Fr Mal App Dig. 1966 Jan-Feb;55(1):31–43. [PubMed]
  • Macinot C, Lorenzini CL, Ravey M, Mauvary G, Rauber G. Entérite segmentaire sténosante multifocale. Sem Hop. 1974 Oct 14;50(41):2518–2520. [PubMed]
  • Lescut J, Grégoire L, Weisemburger JP. Stenoses multiples cryptogenetiques de l'intestin grele. Lille Med. 1969;14(5):602–607. [PubMed]
  • Chagnon JP, Devars du Mayne JF, Marche C, Vissuzaine C, Cerf M. L'entérite sténosante multifocale cryptogénétique: une entité autonome? Gastroenterol Clin Biol. 1984 Nov;8(11):808–813. [PubMed]
  • Doutre LP, Paccalin J, Périssat J, Traissac FJ. Entérite ténosante ulcéreuse plurifocale. Une nouvelle observation. Arch Fr Mal App Dig. 1966 Jun;55(6):537–540. [PubMed]
  • SHOESMITH JH, TATE GT, WRIGHT CJ. MULTIPLE STRICTURES OF THE JEJUNUM. Gut. 1964 Apr;5:132–135. [PMC free article] [PubMed]
  • Perlemuter G, Chaussade S, Soubrane O, Degoy A, Louvel A, Barbet P, Legman P, Kahan A, Weiss L, Couturier D. Multifocal stenosing ulcerations of the small intestine revealing vasculitis associated with C2 deficiency. Gastroenterology. 1996 May;110(5):1628–1632. [PubMed]
  • Johnson CA, Densen P, Hurford RK, Jr, Colten HR, Wetsel RA. Type I human complement C2 deficiency. A 28-base pair gene deletion causes skipping of exon 6 during RNA splicing. J Biol Chem. 1992 May 5;267(13):9347–9353. [PubMed]
  • Brahme F, Hildell J. Angiography in Crohn's disease revisited. AJR Am J Roentgenol. 1976 May;126(5):941–951. [PubMed]
  • Wakefield AJ, Sawyerr AM, Dhillon AP, Pittilo RM, Rowles PM, Lewis AA, Pounder RE. Pathogenesis of Crohn's disease: multifocal gastrointestinal infarction. Lancet. 1989 Nov 4;2(8671):1057–1062. [PubMed]
  • Ruan EA, Komorowski RA, Hogan WJ, Soergel KH. Nongranulomatous chronic idiopathic enterocolitis: clinicopathologic profile and response to corticosteroids. Gastroenterology. 1996 Sep;111(3):629–637. [PubMed]
  • Tandon HD, Prakash A. Pathology of intestinal tuberculosis and its distinction from Crohn's disease. Gut. 1972 Apr;13(4):260–269. [PMC free article] [PubMed]
  • Lang J, Price AB, Levi AJ, Burke M, Gumpel JM, Bjarnason I. Diaphragm disease: pathology of disease of the small intestine induced by non-steroidal anti-inflammatory drugs. J Clin Pathol. 1988 May;41(5):516–526. [PMC free article] [PubMed]
  • Baer AN, Bayless TM, Yardley JH. Intestinal ulceration and malabsorption syndromes. Gastroenterology. 1980 Oct;79(4):754–765. [PubMed]
  • Mills PR, Brown IL, Watkinson G. Idiopathic chronic ulcerative enteritis. Report of five cases and review of the literature. Q J Med. 1980 Spring;49(194):133–149. [PubMed]
  • Camilleri M, Pusey CD, Chadwick VS, Rees AJ. Gastrointestinal manifestations of systemic vasculitis. Q J Med. 1983 Spring;52(206):141–149. [PubMed]
  • Fauci AS, Haynes B, Katz P. The spectrum of vasculitis: clinical, pathologic, immunologic and therapeutic considerations. Ann Intern Med. 1978 Nov;89(5 Pt 1):660–676. [PubMed]
  • Schölmerich J, Schmidt E, Schümichen C, Billmann P, Schmidt H, Gerok W. Scintigraphic assessment of bowel involvement and disease activity in Crohn's disease using technetium 99m-hexamethyl propylene amine oxine as leukocyte label. Gastroenterology. 1988 Nov;95(5):1287–1293. [PubMed]
  • Lie JT. The Canadian Rheumatism Association, 1991 Dunlop-Dottridge Lecture. Vasculitis, 1815 to 1991: classification and diagnostic specificity. J Rheumatol. 1992 Jan;19(1):83–89. [PubMed]
  • Agnello V. Complement deficiency states. Medicine (Baltimore) 1978 Jan;57(1):1–23. [PubMed]
  • Williams DH, Kratka CD, Bonafede JP, Katon RM. Polyarteritis nodosa of the gastrointestinal tract with endoscopically documented duodenal and jejunal ulceration. Gastrointest Endosc. 1992 Jul-Aug;38(4):501–503. [PubMed]
  • McCauley RL, Johnston MR, Fauci AS. Surgical aspects of systemic necrotizing vasculitis. Surgery. 1985 Jan;97(1):104–110. [PubMed]
  • Kuehne SE, Gauvin GP, Shortsleeve MJ. Small bowel stricture caused by rheumatoid vasculitis. Radiology. 1992 Jul;184(1):215–216. [PubMed]
  • Hekali P, Kajander H, Pajari R, Stenman S, Somer T. Diagnostic significance of angiographically observed visceral aneurysms with regard to polyarteritis nodosa. Acta Radiol. 1991 Mar;32(2):143–148. [PubMed]
  • Lightfoot RW, Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, McShane DJ, Arend WP, Calabrese LH, Leavitt RY, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990 Aug;33(8):1088–1093. [PubMed]
  • Williams DH, Kratka CD, Bonafede JP, Katon RM. Polyarteritis nodosa of the gastrointestinal tract with endoscopically documented duodenal and jejunal ulceration. Gastrointest Endosc. 1992 Jul-Aug;38(4):501–503. [PubMed]
  • Lopez LR, Schocket AL, Stanford RE, Claman HN, Kohler PF. Gastrointestinal involvement in leukocytoclastic vasculitis and polyarteritis nodosa. J Rheumatol. 1980 Sep-Oct;7(5):677–684. [PubMed]
  • Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb;37(2):187–192. [PubMed]

Figures and Tables

Figure 1
Superior mesenteric artery. Selective conventional angiography; arterial phase showing aneurysms of the distal jejunal arteries (arrowheads).
Figure 2
Longitudinal section of the resected small bowel through the strictures.

Articles from Gut are provided here courtesy of BMJ Group

Formats:

Related citations in PubMed

See reviews...See all...

Cited by other articles in PMC

See all...

Links

  • MedGen
    MedGen
    Related information in MedGen
  • PubMed
    PubMed
    PubMed citations for these articles
  • Substance
    Substance
    PubChem Substance links

Recent Activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...