collagenous colitis: histologic progression...

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BRIEF COMMUN I CATION Collagenous colitis: Histologic progression, extraintestinal features and lack of response to 5--ASA -A case report FRANZJOSEF SCI lWEIGER, BSc, MO, FRCPC, FACG ABSTRACT: Co llagenous colitis is a clinicopathologic syndrome which pre- sents as chron ic, watery, nonb loody diarrhea. Co lonic biopsies characteristi ca lly show a thick subepithelial collagen band. Barium enema and colonoscopy ge nera ll y fail co estab lish the diagnosis. This case report provides evidence for histol og ic progression over time, pancolonic involvement and the presence of extraintestinal features of co llagenous co litis. The reported patient showed no response to 5-aminosa licylic acid. Co lonoscopic biopsies in cases of chro nic diarrhea can save the patient repeated, uncomfortable and costly investigations. CanJ Gastroenterol 1990;4(1):19-22 Key Words: 5-aminosalicylic acid, Collagenous colitis, Ch ronic diarrhea , Extraintes- nnal features La colite du collagene: Evolution histologique progressive, manifestations extraintestinales et manque de reponse a 5-ASA RESUME: La colite du co llagene es t un syndrome anatomoclinique caracterise par une diarrhee chronique aqueuse non sanglante. De maniere caracteristique, l es biopsies co liques montrent une epaisse bride de collagene sous-epitheliale. l e l aveme nt baryte et la co lonoscopie ne parviennent generalement pas a etab lir le diagnostic. le cas present montre une evoluti on histologique progressive, une atteinre pancolique et des manifestations exrraintestinales propres a la co li re du collagene. Le patient conceme n'a pas repondu a l'ac ide 5-aminosalicylique. Dans l es cas de diarrhee ch r on ique, le recours aux biopsies colonoscopiques peut epargner au patient des investigations repetees, desagreables et couteuses. The Moncion Hospital , Moncion, New Brunswick Correspondence and reprints: Dr F Schweiger, I 00 Arden Street, Suite 405, Monct0n, New Bnmswick EiC 4B7. Telephone (506)858-8441 Received f OT publication J rme 23, 1988. Accepted November 13, 1989 CAN J GASTROENTEROL VOL 4 NO 1 JANUARY/FEBRUARY 1990 T HE CLINICOPATI I OLlX,IC ENTITY of collagenous coliLi s has hecome increasingly recognized since its first descripti on l 3 yea r~ ago (1 ), with at least 50 cases rerortcd in the English literature. A case of co llagenous colitis is re - ported which nol on ly demonstrates th e difficulty in diagnosis but also adds information wi th respect to mucosa ] progression, rossible excraincestinal manifestations and treatment of this condition. CASE PRESENTATION A 71-year-o ld female presented with a 16 year history of intermittent diar- rhea. The diarrhea was characterized by up to 15 loose to watery bowel move- ments per day, which often awakened her at night. It was preceded by bi- lateral lower abdominal cramps and as- sociated with tenesmu s. She denied the passage of blood but did not i ce mucus. These e pisodes occurred several times per year lasting up tO th ree to four weeks and were fo llowed by more regu- lar soft to loose bowel moveme nts num- 19

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BRIEF COMMUNICATION

Collagenous colitis: Histologic progression, extraintestinal

features and lack of response to 5--ASA - A case report

FRANZJOSEF SCI lWEIGER, BSc, MO, FRCPC, FACG

ABSTRACT: Collagenous colitis is a clinicopathologic syndrome which pre­sents as chronic, watery, nonbloody diarrhea. Colonic biopsies characteristically show a thick subepithelial collagen band. Barium enema and colonoscopy generally fail co establish the diagnosis. This case report provides evidence for histologic progression over time, pancolonic involvement and the presence of extraintestinal features of collagenous colitis. The reported patient showed no response to 5-aminosalicylic acid. Colonoscopic biopsies in cases of chronic diarrhea can save the patient repeated, uncomfortable and costly investigations. CanJ Gastroenterol 1990;4(1):19-22

Key Words: 5-aminosalicylic acid, Collagenous colitis, Chronic diarrhea, Extraintes­nnal features

La colite du collagene: Evolution histologique progressive, manifestations extraintestinales et manque de reponse a 5-ASA

RESUME: La colite du collagene est un syndrome anatomocl inique caracterise par une diarrhee chronique aqueuse non sanglante. De maniere caracteristique, les biopsies coliques montren t une epaisse bride de collagene sous-epitheliale. l e lavement baryte et la colonoscopie ne parviennent generalement pas a etablir le diagnostic. le cas present montre une evolution histologique progressive, une atteinre pancolique et des manifestations exrraintestinales propres a la coli re du collagene. Le patient conceme n'a pas repondu a l'acide 5-aminosalicylique. Dans les cas de diarrhee chronique, le recours aux biopsies colonoscopiques peut epargner au patient des investigations repetees, desagreables et couteuses.

The Moncion Hospital , Moncion, New Brunswick Correspondence and reprints: Dr F Schweiger, I 00 Arden Street, Suite 405, Monct0n, New

Bnmswick EiC 4B7. Telephone (506)858-8441 Received f OT publication J rme 23, 1988. Accepted November 13, 1989

CAN J GASTROENTEROL VOL 4 NO 1 JANUARY/FEBRUARY 1990

THE CLINICOPATI IOLlX,IC ENTITY

of collagenous coliLis has hecome increasingly recognized since its first description l 3 year~ ago (1 ), with at least 50 cases rerortcd in the English literature.

A case of collagenous colitis is re­ported which nol only demonstrates the difficulty in diagnosis but also adds information with respect to mucosa] progression, rossible excraincest inal manifestations and treatment of th is condition.

CASE PRESENTATION A 71-year-old female presented with

a 16 year history of interm ittent diar­rhea. The diarrhea was characterized by up to 15 loose to watery bowel move­ments per day, which often awakened her at night. It was preceded by bi­lateral lower abdominal cramps and as­sociated with tenesmus. She denied the passage of blood but did notice mucus. These episodes occurred several times per year lasting up tO th ree to four weeks and were followed by more regu­lar soft to loose bowel movements num-

19

SCHWEIGER

Figure 1) Rectal biopsy showing marked mononuclear inflammatory cell infiltration in che lamina propria with preservation of crypt architecture and normal goblet cells. ( Hematoxylin and eosin X 300)

bering two to three per day. Over the past year prior to admiss ion she began having more frequent bouts of diarrhea often leading to fecal incontinence. In addition , she complained of mild ano­rexia, nausea and severe heartburn that was relieved by H2 blocker therapy.

At age 56 she was found to have a slightly raised erythrocyte sed imenta­tion rate, but stool cultures, sigmo ido­scopy and barium x-rays of her upper and lower gastrointestina l tract were normal. An exploratory laparotomy re­vealed cho lelithiasis and her gallblad-

der was removed. A jejuna! biopsy was reported as normal.

At age 64 she was readmitted be­cause of worsening diarrhea. She was found to have seronegative deforming arthritis involving the small joints of her hands and knees. Stool cultures, sigmoidoscopy, barium enema and liver­spleen scan were negative and she was treated with a fibre-enriched die t and psyllium hydrophilic mucillo id .

One year later she required readmis­sion to hospital because of persistence of her symptoms despite the admini-

Figure 2) Biopsy of transverse colon displayinginarked subepithelial collagen de/JOsition with marked increase of mononuclear inflammatory cells in the lamina />ropria. ( Hematoxylin and eosin X 900)

stration of codeine and loperamide. Re­peat ba rium studies, a D-xylose ex­c retion test, Schi ll ing test, serum gastrin levels and gastric analysis were a ll normal as was the scrum cortisol anJ a 24 h urine sample for 5-hydroxy­indoleacetic ac id (5-HIAA). A 72 h stool collection yielded an average of 165 g of feces with l .8 g of fat per 24 h. A colonoscopy and repeat small bowel biopsy were within normal limits.

Further investigations during the following year again revealed negative stool cultures as well as a negative mt for Clostridium difficile cytotoxin. A small bowel aspirate and string test failed to recover Giardia lamblia cysts or t rophozoites. A sigmoidoscopy was nor­mal but a random rectal biopsy de­monstrated 'nonspec ific colit is' (Figure I). She was treated with total paren­teral nutrition for l l Jays which led to

improvement of her diarrhea and weight ga in. She was d ischarged on co­deine but experienced intermittent re· lapses of cramps and dia rrhea over the next five years.

She presented again recently anJ was found to have a slightly clcvateJ blood glucose level which was treated by diet. She compla ined of periodic de­pression particularly since her hus­band's death two years earl ier. She denied the use of laxfltives. Other past medical history included recurrent pneumonias over the past l 5 years, a hysterectomy, bi lateral ligation of vari­cose veins and a hemorrhoiJecromy.

There was no family h istory of in­flammatory bowel disease or other in­testinal disorders.

On examination the patient ap­peared depressed. I !er head and neck were normal and there was no thyromegaly. T he chest and cardiovas­cular system examination were un­remarkable apart from a grade 11/Vl sy~­tolic ejection murmur radiating to the base. There were two old scars on her abdomen, which was slightly distended and tympanitic. There was mi ld diffuse tenderness throughout her abdomen but no ev idence of organomegaly or mass. Rectal examina tio n was nor­mal. S he had jo in t deformities affect· ing he r proxima l and dista l in ter­phalangeal joints, wrists and knee~,

20 CAN J 0ASTROENTEROL VOL 4 No I JANUARY/FEBRUARY l 990

but no acuvcly inflameJ joints were founJ. She haJ several patches of v1tiligo over her forearms.

Further investigations in hospital JcmonstrateJ a normal complete hlood count and erythrocyte scuimentation rate. Serum electrolytes, calcium anJ proteins were normal hut the fasting blood glucose and glycosylated hemo­globin were mildly clevateJ. A 72 h ,tool collection resulted in a stool wcightof225 g per 24 h and 14 g of fat per Jay while on a 100 g fa t containing diet. Stool electrolytes were potassium 99 mmol/L, sodium 61 mmol/L, chlo­ride 68 mmol/L, with a stool osmolality of 468 mmol/L. The pH was 8 and sttX)l was negative for occult blood and re­ducing substances.

Upper endoscopy revealed grade ll ulcerative esophagitis and esophageal biopsy was consistent with this. Small bowel biopsy was normal. A colono­scopic examinauon was unremarkable but multiple biopsies obtained ar 10 cm intervals from cecum to rectum showed the presence of collagenous col itis in all specimens (Figure 2). The panenr was ,tarred on 5-aminosalicylic acid (5-ASA) , tlOO mg tid), a high fibre diet and psyllium mucilloid. Although she improved initially her diarrhea recurred after three weeks while on rbe medication and repeat biopsies showed no histologic improvement.

Subsequent therapy with sulfasa la­zme and oral prednisone was poorly tol­erated and <lid nor lead to symptomatic improvement. Currently, she is being treated with steroid enemas and she passes three m five scmiforrned bowel movements per day.

DISCUSSION Collagcnous colitis was fi rst de­

scribed by Lindstr6m in 1976 (1) and has been the subject of recent reviews (2,3). This clinicoparhologic syndrome characteristically presents as a chronic watery diarrhea anJ colonic mucosa! biopsies reveal the presence of a linear subepithelial fibrous thickening and a chronic inflammaLOry infilLrate in the lamina propria. As in the patient de­scribed, radiologic and even colonosco­pic findings may be entirely normal or nonspecifically abnormal and the diag-

no~,s 1s eventually m,1de only by mu­cosa! biopsy (2,4) .

The dbeasc appears w be most com­mon 111 middle-aged co elderly women. The diarrhea is often intermittent, nonbkxxJy, and associated with ahdo­minal cramps. It is frequencly misd iag­nosed as a manifestation of irritable bowel syndrome (5).1 lowever, the pre­sence of nocturnal diarrhea, feca l in­continence and weight loss, should suggest an organic cause for diarrhea.

The patient in this case report suf­fered from a seronegative peripheral polyarthritis; its association with col­lagenous colitb has repeatedly been re­ported (6-8). In addition, the occur­rence of glucose intolerance, Lhyroid disease and the finding of antinuclear ant1bodie::. m some patients with col­lagenous coli tis has been ci teJ as evidence for an autoimmune patho­genenc melharnsm (9). The presence of vitiligo has not been described pre­viously.

The reported pat1enl in1t1ally had a rectal biopsy five years prior to the find­ing of a th ickened suhcpithelial collagen table 111 thesameanawm1c location. This initial biopsy revealed an active colitis consistent with the recently recognized emit)' of microscopic colitis (10). Hence, this case adds further support to the hypothesis that collagenous colitis and microscopic colitis are but Jifferent aspects of the same condition (9, 11 ,12). The pathogenesis of the fonnation of the collagen banJ 1s not completely under­stood, but likely requires rhe presence of chronic subepithelial inflammation ( 13 ). ln addition, the existence of a local ab­nonnaliry of collagen synthesis has been postulated (2).

T his case demomtrates the panco­lonic distribution of collagenous col it is (3, 14) . Charactcmtica lly, jeiunal biopsie~ are normal except 111 rare cases in which celiac disease was found to be associated with col­lagenous or mic roscopic coli t is (12,14-17). However, there was no ev idence of small bowel villous atrophy in this pat ient and her mild steatorrhea remains unexplain ed.

The treatment response co various drugs is unpredictable and often disap­po111ting ( 14). Both sulfasalazme and

CAN J GASIBOENTl:.ROL VOL 4 NO I jANUARY/FEBRL,ARY 1990

Collagenous colitis

corucnstermds have 111dl11 .. cd rembs1lm 111 ~evcral cases (2,9) but have heen ineffective in others ( 14 ). Anecdotal therapeutic success has been achieved with a number of mher compounds in­cluding mepau-inc ( 11), metro111dazole ( 18), and hbmuth subsa l1cylate ( 19). Sulfasalazine has been shown in several cases to lead not only to cl inical hut also h1smlog1c 1mprovemem seen 111 subsequent hiopsies (9,12). Wht'thcr this effect was brought ahout by the antimicrobial (su lfonamide) or ant1-1n­flammatory (5-ASA) mrnety, however, is not clear. T his patient was treated with 5-ASA and Jespite a good therapeutic Jose and du rat 1011 of thera­py there was no cl inical or hiswlngic response. Funhennore, su lfasalaz111e did not lead to 11nprovcment of di.ir­rhea. Rams ct al (2) proposed that rnl­lagcnous col1t b is an inflammatory disorJer, possibly of infectious origm, which 1s initia lly characterized by an acute mflammatory process which pro­gresses over time and results 111 a gra­dual mcrcase llf collagen which may act as a diffusinn b.1mer, further contribut­ing w diarrhea. Concc1vahly, neither anti-inflammatory nor ancimicmhial agents arc effective at this later stage of the disease.

Finally, th is t.ase report underlines the importance of colnnic hiopsies even in the absence of colonic find ings in patients w1tl1 unexplained chronic diarrhea. If pos1t 1ve, the diagnosis is established, and repeated, uncomfort­able and costly mvcstigarions can he avoided.

ACKNOWLEDGEMENTS: The author ,1cknnwlcJgcs rhc help of Dr DA MalatJa­lian anJ Dr W Ying with rhc prcpamt1on of the phlltomicmgraphs.

REFERENCES I. LinJstriim CG. 'Collagenow, col1us'

with watery Jiarrhea - A new enrny? Pathol Eur 1976; 1 l:87-9.

2. Rams 11, Roger., Al, GhanJur-Mnaym­neh L. Collagenous colms. Ann Intern Mc<l 1987; 106: I 08-13.

3. Covcrl1zza S, Ferr.ui A, Sccvola F, ct al. C lm1co-pacholog11.:a l features o(

collagenous colitis: C.isc report anJ literature revu:w. A m J G,1smientcml I 986;81: 1098-103.

21

SCIIWEIGER

4. Salt WB 11, Llaneza PP. Collagenous 1986;8:677-80. potentially reversible J1sorJer. J Clin col iris: A cause nf chronic diarrhea 9. Jcssurun J, Yardley Jl I, Giardicllo FM, Pacho! 1982; 35: 338-40. diagnosed only by biopsy nf n1lrmal ap- Hamilton SR, Bayless TM. Chronic 14. Kingh;:un JGC, Levison DA, Morson pearing colonic mucosa. Gastrointcst colitis with thickening of the suh- BC, Dawson AM. Collagcnou:, colitis. Endosc 1986;32:421-3. epithelial collagen layer (col lagenous Gut 1986;27:570-7.

5. G iardicllo FM, Bayless TM, Jessurun J, colitis): Hiscopathologic findings in 15 15. Hwang WS, Ke lly JK, Shaffer EA, Hamilton SR, Yardley JH. Collagenous patients. Hum Pathol 1987;18:839-48. Hershfield NB. Collagenous colitis: A colitis: Physiologic and histopathologic LO. Bo-Linn GW, Vendrell DD, Lee E, disease of pericryprnl fibmhlast sheath / studies in seven patients. Ann Intern Fordtrnn JS. An evaluation of the sig- J Pacho! L 986; 149:33-40. Med 1987;106:46-9. nificance of microscopic colitis in 16. Hamilton I, Sanders S, Hopwood D,

6. Erlendsson J, Fenger C, Meinicke J. patients with chronic diarrhea. J Clin Bouchier !AD. Collagenous col itis as-Arthritis and collagcnous colitis: Report Invest 1985;75:1559-69. soc iated with small intestinal villous of a case wirh concomitant chronic 11. Teglbjaerg PS, Thaysen EH, Jensen atrophy. Gut 1986;27: 1394-8. polyanhritis and collagenous colitis. I {H. Development of collagenous 17. Breen EG, Coughlan G, Connolly CE, Scand J Rheumatol 1983; 12:93-5. colitis in sequential biopsy specimens. Stevens FM, McCarthy CF. Coeliac

7. Farah DA, Mills PR, Lee FD, Mclay Gastroencerology l 984;87:703-9. proctitis. Scam! J Gastroenterol A, Russell RI. Collagennus colitis: Po~- 12. Sylwestrowicz T, Kelly JK, Hwang WS, 1987;22:47 l • 7. sible response to sulfasalazme and local Shaffer EA. Collagenous colitis and 18. Mogensen AM, Olsen JI l, GuJmand-steroid therapy. Gastrucnterology microscopic colitis: The watery diar- Hoyer E. Collagenous colitis. Acrn 1985;88:792-7. rhea-colitis syndrome. Am J Med Scand I 984;216:535-40.

8. Wiener MO. Collagenous colitis and Gastroenterol 1989;84: 763-8. 19. Girard DE, Keeffc EB. Therapy for col-pulmonary fibrosi,. Manifestations of a 13. Pieterse AS, Hecker R, Rowland R. lagenous colirb. Ann Intern Med single disea~c?J Clin Gastroenrerol Collagenous colitis: A distinctive and 1987;106:909.

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