Coeliac Disease with Histological Features of Peptic Duodenitis: Value
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4204 Clin Pathol 1993;46:420-424 Coeliac disease with histological features of peptic duodenitis: Value of assessment of intraepithelial lymphocytes J Clin Pathol: first published as 10.1136/jcp.46.5.420 on 1 May 1993. Downloaded from M D Jeffers, D O'B Hourihane Abstract phonuclear leucocyte infiltrate and absence of Aims-To determine if a clinically gastric metaplasia in coeliac disease. important polymorphonuclear leucocyte We present four cases in which mucosal infiltrate and surface gastric epithelial biopsy specimens of the second part of the metaplasia occur in the second part of duodenum presented histological appear- the duodenum in coeliac disease; to eval- ances suggesting non-specific duodenitis but uate the utility of these morphological in which the diagnosis of coeliac disease was criteria in the differential diagnosis of subsequently confirmed on clinical, immuno- coeliac disease and peptic duodenitis. logical, and histological grounds. Methods-49 mucosal biopsy specimins of the second part of the duodenum reported as showing inflammation were Methods reviewed. Sections were prepared with All mucosal biopsy specimens of the second haematoxylin and eosin, periodic acid part of the duodenum reported as showing Schiff, and Warthin-Starry stains. Clini- inflammation were reviewed for the 6 months cal presentation, outcome, and immuno- from January to July 1991. This included logical investigations were assessed. cases reported as coeliac disease, peptic duo- Results-Four cases confirmed as coeliac denitis, non-specific inflammation and other disease on clinical and immunological specific diseases. Sections 4 pm thick were grounds showed acute inflammation and prepared with haematoxylin and eosin, peri- surface epithelial gastric metaplasia. odic acid Schiff (PAS), and Warthin-Starry Increased intraepithelial lymphocytes silver stains. Sections were reviewed with par- (IELs) were found in each ofthe four. ticular emphasis on villous atrophy, crypt Conclusions-Clinically important poly- hyperplasia, chronic inflammation, intraep- morphonuclear leucocyte infiltration and ithelial lymphocytes, acute inflammation, sur- surface epithelial gastric metaplasia may face epithelial abnormalities and surface http://jcp.bmj.com/ occur in the duodenal mucosa in coeliac epithelial gastric metaplasia. On the basis of disease and should not be used as diag- morphology, cases were designated as coeliac nostic features to exclude the diagnosis disease, peptic duodenitis, non-specific of coeliac disease in the absence of con- inflammation and other specific inflammatory firmatory clinical and immunological disorders. information. Case notes were reviewed with particular reference to clinical presentation, endoscopic on September 26, 2021 by guest. Protected copyright. (7 Clin Pathol 1993;46:420-424) appearance, response to gluten withdrawal and serum a gliadin and endomysial antibody titres. Intraepithelial lymphocytes were count- Inflammation of the duodenal mucosa occurs ed for 300 surface epithelial cells, and the in many different diseases. Specific morpho- result expressed as a mean, with the range per logical features are present which point to 100 surface epithelial cells. Alpha gliadin aetiology in some cases, while the remainder antibodies are present from 0-3 units/ml in are often grouped together under the collec- healthy subjects. tive title of non-specific (peptic) duodenitis. Coeliac disease is characterised on mucosal biopsy specimens by villous atrophy, surface Results epithelial cell disarray, mixed chronic inflam- The morphological diagnoses of the 49 biop- matory cell infiltrate of the lamina propria, sy specimens from 48 patients were as fol- and a sharp increase in intraepithelial lym- lows: coeliac disease n = 13; peptic phocytes, chiefly of the T8 + subtype. In duodenitis n = 12; peptic ulceration n = 3; peptic duodenitis the inflammation includes non-specific inflammation n = 16; Crohn's Department of prominent foci of polymorphonuclear leuco- disease n = 1; coeliac disease/peptic duodeni- Pathology, St James's cytes, and flattening of surface (villous atro- tis n = 4. Hospital and Trinity College, Dublin phy), and may be difficult to distinguish from Helicobacter-like organisms were identified in Ireland the changes seen in coeliac disease. Features four cases of peptic duodenitis, and were pre- M D Jeffers which have been proposed as useful diagnos- sent only on gastric type epithelium. D O'B Hourihane tic pointers in distinguishing severe peptic Serum a gliadin antibody titres were raised Correspondence to: Professor D O'B Hourihane duodenitis from coeliac disease include the in 10 cases (coeliac disease nine cases, range Accepted for publication relative lack of architectural changes in non- 6 to 30 units/ml, mean 13-1 units/ml, 4 November 1992 specific duodenitis, and the scanty polymor- Crohn's disease one case, 32 units/ml). Coeliac disease with histologicalfeatures ofpeptic duodenitis 421 Serum endomysial antibody titre was avail- other symptoms. able in four cases of coeliac disease and was There was no family history of coeliac dis- positive in two cases. eae, although one sibling also had iron defi- Gastric metaplasia was present in 15 cases, ciency anaemia. At endoscopy the 12 cases of peptic duodenitis, and three cases oesophagus, stomach, and duodenum were J Clin Pathol: first published as 10.1136/jcp.46.5.420 on 1 May 1993. Downloaded from of coeliac disease originally reported as most all macroscopically normal. A biopsy speci- suggestive of peptic duodenitis. Biopsy speci- men of the second part of the duodenum mens from these three patients showed archi- showed villous atrophy (fig 1) and prominent tectural changes and inflammatory infiltration intraepithelial lymphocytes, with focal gastric suggestive of coeliac disease but also con- metaplasia (less than 5% of surface entero- tained acute inflammation and surface epithe- cytes) (fig 2) and pronounced polymorphonu- lial gastric metaplasia. These three cases are clear leucocyte infiltrate in the lamina propria presented in detail below. A fourth example which extended into the surface epithelium. of gastric metaplasia and focal poly- This was interpreted as peptic duodenitis morphonuclear leucocyte exudate was rather than coeliac disease. Serum a gliadin encountered during 1992, and has been antibodies were positive at a titre of >32 added to the series (case 4). units/ml and serum endomysial antibodies (EMA) were positive. A gluten challenge was performed (50 g Case reports gluten a day for 10 weeks) and a biopsy speci- CASE 1 men of the second part of the duodenum was A 42 year old man presented with a history taken at the end of this. This again showed of persistent unexplained iron deficiency architectural features suggestive of coeliac anaemia (haemoglobin 114 g/l, mean cell vol- disease, but it also showed a noticeable poly- ume 74X5 fl). There was no history of diar- morphonuclear leucocyte infiltrate, raising rhoea, nausea, weight loss, mouth ulcers or the possibility of non-specific duodenitis; a gliadin antibodies was again positive at a titre of >32 units/ml, and EMA were positive. The patient was started on a gluten free diet. After 6 months a duodenal biopsy was performed which showed that the mucosa had reverted to normal (fig 3). CASE 2 A 13 year old girl who had not yet begun to menstruate presented with lassitude and fatigue and was found to have iron deficiency anaemia (haemoglobin 37 g/l, mean cell vol- http://jcp.bmj.com/ ume 67-6 fl). She was also folate deficient (1.2 mg/l normal 2-7 > 20 mg/l). She had no gastrointestinal symptoms. There was no family history of coeliac disease. At endoscopy the oesophagus and stomach appeared normal and the duodenum appeared atrophic. A mucosal biopsy speci- on September 26, 2021 by guest. Protected copyright. PIT;, ;i;'.£P ^. men from the second part of the duodenum Figure I Case 1: duodenal mucosa with vilous atrophy and severe inflammmation showed villous atrophy, mixed chronic (haematoxylin and eosin.) inflammatory infiltrate of the lamina propria, increased intraepithelial lymphocytes, and a polymorphonuclear leucocyte infiltrate of lamina propria, crypt, and surface epithelium (fig 4), and focal gastric metaplasia of surface epithelium. Serum a gliadin antibodies were positive, at a titre of 30 units/ml; EMA were positive. CASE 3 (. A 74 year old woman was admitted with weakness and dyspnoea. Other symptoms included heartburn, anorexia, and a weight loss of 5 lbs. A full blood count showed iron deficiency anaemia (haemoglobin 83 g/l, mean cell volume 82 fl). At endoscopy there was oesophagitis at 28 cm and the stomach and duodenum were macroscopically normal. A mucosal biopsy specimen of the oesopha- gus showed changes suggestive of reflux oesophagitis. A biopsy specimen of the sec- ond part of the duodenum showed subtotal Figure 2 Case 1: focal surface epithelial gastnic metaplasia (arrow) (periodic acid-Schiff. villous atrophy with crypt hyperplasia, epithe- 4224Jeffers, Hourihane lial cell abnormalities, increased numbers of intraepithelial lymphocytes and a mixed chronic inflammatory cell infiltrate of the lamina propria, with, in addition, a polymor- phonuclear leucocyte infiltrate with extension J Clin Pathol: first published as 10.1136/jcp.46.5.420 on 1 May 1993. Downloaded from upwards into the surface epithelium which also showed focal gastric metaplasia. The patient was discharged with oral iron supple- ments. Three months later she still had per- sistent abdominal