Treatment of sclerosing mesenteritis with corticosteroids ...downloads.hindawi.com/journals/cjgh/2001/462823.pdf · A Bala, SP Coderre, DRE Johnson, V Nayak. Treatment of sclerosing

Sclerosing mesenteritis (also known as idiopathic mesen-teritis, retractile mesenteritis, liposclerotic mesenteri-

tis) is a very uncommon, benign process that usuallyinvolves the small bowel (1). Nonspecific inflammationand progressive fibrosis infiltrate, thicken and retract themesentery, which may lead to a number of gastrointestinalsymptoms, including abdominal pain and bowel obstruc-tion. The etiology and pathogenesis of this disorder areunknown. A patient with histologically proven sclerosingmesenteritis who presented with abdominal pain andincomplete small bowel obstruction is described. Thepatient was treated successfully with surgery, corticos-teroids and azathioprine.

CASE PRESENTATIONA 21-year-old white man was admitted with a 24 h history ofprogressive right lower quadrant abdominal pain. Nauseaand vomiting began after the onset of the pain. There wereno significant changes in bowel habit. He denied weightloss, night sweats or fever. There was no history of recenttravel or infectious contacts. His past medical, social andfamily histories were unremarkable. He was not on any med-ications. Physical examination revealed a toxic-appearingman with tachycardia but who was afebrile. The abdomenwas soft, with localized guarding and rebound in the rightlower quadrant. No masses were palpable, bowel sounds wereabsent and results of a rectal examination were normal. The

Can J Gastroenterol Vol 15 No 8 August 2001 533

BRIEF COMMUNICATION

Treatment of sclerosingmesenteritis with corticosteroids

and azathioprine

Anand Bala MD FRCPC, Sylvain P Coderre MD FRCPC, Douglas RE Johnson MD FRCS, V Nayak MD FRCS

Divisions of Gastroenterology and General Surgery, and Department of Pathology, Peter Lougheed Hospital, University of Calgary, Calgary, AlbertaCorrespondence: Dr Sylvain P Coderre, Division of Gastroenterology, Peter Lougheed Hospital, 3500–26th Street Northeast, Calgary,

Alberta T1Y 6J4. Telephone 403-219-1512, fax 403-291-8017, e-mail [email protected] for publication October 31, 2000. Accepted February 2, 2001

A Bala, SP Coderre, DRE Johnson, V Nayak. Treatment ofsclerosing mesenteritis with corticosteroids and azathioprine.Can J Gastroenterol 2001;15(8):533-535. Sclerosing (idio-pathic) mesenteritis is a rare disease that may present withabdominal pain or bowel obstruction. A 21-year-old man wasdiagnosed with sclerosing mesenteritis, and treated with a partialileal resection and defunctioning ileostomy. He was subse-quently started on corticosteroids and azathioprine. Five monthslater, at the time of ileostomy reversal, he was disease-free. Thediagnosis and management of this disease are discussed.

Key Words: Abdominal pain; Bowel obstruction; Sclerosing mes-enteritis

Traitement de la mésentérite sclérosante àl’aide de corticostéroïdes et d’azathioprineRÉSUMÉ : La mésentérite sclérosante, d’origine idiopathique, est unemaladie rare qui peut se manifester sous forme de douleurs abdominalesou d’obstruction intestinale. Voici le cas d’un homme de 21 ans chez quiune mésentérite sclérosante a été diagnostiquée; il a subi une résectionpartielle de l’iléon et une iléostomie temporaire. On a ensuite amorcé untraitement aux corticostéroïdes et à l’azathioprine. Cinq mois plus tard,au moment où l’on a procédé à la fermeture de l’iléostomie, il était com-plètement guéri. Suit dans le présent article une discussion sur le diag-nostic et le traitement de la maladie.

remainder of the examination was unremarkable. Results ofinitial testing of complete blood count, electrolytes andlipase, and urinalysis were normal. Chest and abdominalx-rays were unremarkable.

Given the severity of pain and localized right lower quad-rant peritonitis, a decision was made to proceed to laparo-tomy, with a tentative diagnosis of acute appendicitis. Thepossibility of terminal ileal Crohn’s disease was also enter-tained. At laparotomy, it was noted that the base of theappendix was thickened; however, there was no evidence ofperforation or abscess. The distal terminal ileum was said tobe normal. The appendix was removed, and the patientreturned to the ward to recover. Postoperatively, he contin-ued to experience significant right lower quadrant pain forwhich he required frequent intravenous narcotics.Pathological examination of the excised appendix did notreveal any abnormality. On day 4 of hospitalization, heunderwent a small bowel follow-through, which showed

dilation of the proximal ileal loops with focal areas of nar-rowing and associated edema (Figure 1). Computed tomog-raphy scan with intravenous and oral contrast demonstrateda thickened loop of bowel in the proximal ileum. In light ofthe patient’s ongoing severe abdominal pain and significantradiological abnormalities, an exploratory laparotomy wasperformed. The operative findings were suggestive of markedinflammation, and thickening at the junction of the mesen-tery and the distal 150 cm of the ileum. The ileum wasdilated. The mesenteric root was grossly normal. Many loopsof small bowel were firmly adherent to each other. Giventhese findings, a partial ileal resection was performed, and adefunctioning ileostomy was created. Pathological examina-tion of the resected portion of ileum revealed thickened,palpable nodularities in the mesentery and on the serosalsurface of the bowel. Microscopic examination showedextensive thickening and fibrosis of the serosal surface of theileum (Figure 2). Proliferating fibroblasts and collagen,arranged in thickened bands, were noted in the small intes-tinal mesentery (Figure 3). Postoperatively, the patient wasstarted on a course of prednisone (1 mg/kg/day) and azathio-prine (1 mg/kg/day). He began to improve clinically and wasdischarged.

Five months later, he presented for closure of theileostomy. Surprisingly, no gross disease was apparent in themesentery or small intestine, even at the distal 150 cm ofileum, which had been previously affected. A functionalend to end anastamosis was successfully performed. Hisimmunosuppressants were tapered and were discontinuedafter a five-month course. After nine months of follow-up,he remains well without therapy.

DISCUSSIONIn this report, a young patient with sclerosing mesenteritisresponded well to a treatment regimen of corticosteroids,azathioprine and surgery. Sclerosing mesenteritis is anextremely uncommon condition with an often nonspecificpresentation, making it a difficult and challenging diagno-

Bala et al

Can J Gastroenterol Vol 15 No 8 August 2001534

Figure 1) Standard small bowel follow-through demonstrating dilationof the proximal ileal loops with focal areas of narrowing with associatededema (open arrows)

Figure 2) Sclerosing peritonitis involving the small bowel with fibrosisand thickening of serosa and sparing the muscularis propria (arrow)(hematoxylin and eosin stain, original magnification ×10)

Figure 3) Retractile mesenteritis involving the small intestinal mesen-tery; note the thickened band of proliferating fibroblasts and collagen inmesenteric fat (arrows) (hematoxylin and eosin stain, original magni-fication ×40)

sis. Either sex may be affected, although there is a slightmale preponderance (1.9:1). Caucasians are more com-monly affected. Cases have been described in almost all agegroups (range five years to 87 years). The etiology of thedisease is unknown. However, some have postulated anassociation with mesenteric panniculitis, in which chronicinflammation may lead to progressive mesenteric fibrosis.Initial mesenteric damage may be a result of trauma, infec-tion, autoimmune disease, or thermal, chemical or otherinjury. The small bowel mesentery is most commonlyinvolved; however, there are reports of colonic mesenteryinvolvement (2). Isolated or multiple rubbery nodules areoften present in the mesentery. Diffuse mesenteric thicken-ing may also be noted. The microscopic appearance mayvary from a predominantly acute inflammatory reaction,including fat necrosis and inflammatory infiltrate, to a morechronic fibrotic phase (3). The natural history is usuallyone of progressive fibrosis and retraction, leading to bowelobstruction or, less commonly, mesenteric ischemia (4).

The clinical presentation is varied; patients may beasymptomatic in up to one-third of cases. Presentationshave included abdominal pain, bowel obstruction, palpablemasses, fever and even malabsorption in one report (5,6).Laboratory investigations generally are not helpful. Bariumstudies provide nonspecific information, as obtained in ourcase. Computed tomography or magnetic resonance imag-ing may assist in identifying masses within the mesentery.The definitive diagnosis is made histologically.

The treatment of this condition is controversial. Giventhe rarity of disease, only anecdotal reports can be used toguide potential treatment. Some authors have used corticos-teroids, particularly where the inflammatory component issignificant. Corticosteroids have been shown to be useful inretroperitoneal fibrosis and, therefore, may also affect thefibrotic component of the disease process (7). One authorreported the successful use of a 1.5-year course of taperingazathioprine (1 mg/kg/day) and a three-year tapering courseof prednisone (0.5 mg/kg/day) after initial surgery (4). Ourcase report clearly is in agreement with these findings. Wehave demonstrated clinical improvement and gross patholog-ical improvement with a shorter course of this regimen.

Other treatment approaches have included the use oftamoxifen, reported in an human immunodeficiency virus-positive patient, and the use of cyclophosphamide (8,9).The combination of colchicine with corticosteroids has alsobeen reported to be of benefit (10). Finally, oral proges-terone has been suggested as an alternative to more tradi-tional immunosuppressant therapy (11). Surgery may benecessary to correct bowel, ureteric or vascular obstruction.

Currently, the clinician is faced with a difficult choicein selecting the appropriate treatment for sclerosingmesenteritis. Given the excellent outcome of our patientand others, it is reasonable to consider azathioprine andcorticosteroids early in the course of the disease, once thediagnosis is established.

Sclerosing mesenteritis treatment

Can J Gastroenterol Vol 15 No 8 August 2001 535

REFERENCES1. Kelly JK, Hwang WS. Idiopathic (reactile) mesenteritis and its

differential diagnosis. Am J Surg Pathol 1989;13:513-21.2. Ikoma A, Tanaka K, Komokata T, Ohi Y, Taira A. Reactile

mesenteritis of the large bowel: report of a case and review of theliterature. Surg Today 1996;26:435-6.

3. Emory TS, Carr NJ, Monihan JM, et al. Sclerosing mesenteritis: A clinicopathologic study of 85 cases. Mod Pathol 1996;9:56A. (Abst)

4. Tytgat GN, Roozendaal K, Winter W, Esseveld MR. Successfultreatment of a patient with reactile mesenteritis with prednisone and azathioprine. Gastroenterology 1980;79:352-6.

5. Horing E, Hingerl T, Hens K, von Gaisberg U, Kieninger G. Protein-losing enteropathy: first manifestation of sclerosingmesenteritis. Eur J Gastroenterol Hepatol 1995;7:481-3.

6. Sans M, Varas M, Anglada A, Esperenza Bachs M, Navarro S, Brugues J. Mesenteric panniculitis presenting as fever of unknownorigin. Am J Gastroenterol 1995;90:1159-61.

7. Field C, Arnold W, Gloster ES, Sharp G, Redman J. Steroid therapyas treatment for idiopathic fibrosis of the retroperitoneum andmediastinum. Pediatrics 1986;78:936-8.

8. Bush RW, Hammar SP, Rudolph RH. Sclerosing mesenteritis.Response to cyclophosphamide. Arch Intern Med 1986;146:503-5.

9. Venkatramani A, Behling CA, Lychie KD. Sclerosing mesenteritis: an unusual presentation of abdominal pain in an HIV positivepatient. Am J Gastroenterol 1997;92:1059-60.

10. Genereu T, Bellin MF, Wechsler B, et al. Demonstration of efficacy combining corticosteroids and colchicine in two patients with idiopathic sclerosing mesenteritis. Dig Dis Sci1996;41:686-8.

11. Mazure R, Fernandez MP, Niveloni S, et al. Successful treatment ofreactile mesenteritis with oral progesterone. Gastroenterology1998:114:1313-7.

Submit your manuscripts athttp://www.hindawi.com

Stem CellsInternational

Hindawi Publishing Corporationhttp://www.hindawi.com Volume 2014


MEDIATORSINFLAMMATION

of


Behavioural Neurology

EndocrinologyInternational Journal of



Disease Markers


BioMed Research International

OncologyJournal of



Oxidative Medicine and Cellular Longevity


PPAR Research

The Scientific World JournalHindawi Publishing Corporation http://www.hindawi.com Volume 2014

Immunology ResearchHindawi Publishing Corporationhttp://www.hindawi.com Volume 2014

Journal of

ObesityJournal of



Computational and Mathematical Methods in Medicine

OphthalmologyJournal of


Diabetes ResearchJournal of



Research and TreatmentAIDS


Gastroenterology Research and Practice


Parkinson’s Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttp://www.hindawi.com

Documents

Treatment of sclerosing mesenteritis with corticosteroids ...downloads.hindawi.com/journals/cjgh/2001/462823.pdf · A Bala, SP Coderre, DRE Johnson, V Nayak. Treatment of sclerosing