Usefulness of videoduodenoscopy and vital dye staining as indicators of mucosal atrophy of celiac disease: assessment of interobserver agreement.

BACKGROUND: The present study was designed to determine the diagnostic usefulness of videoduodenoscopic inspection alone and the addition of vital dye staining in the detection of celiac disease. We additionally sought to evaluate interobserver agreement for specific duodenoscopic markers of mucosal atrophy. METHODS: One hundred sixty-seven consecutive subjects who underwent duodenoscopy for intestinal biopsy were included in a prospective controlled study. Endoscopic examination was performed by experienced endoscopists according to a set protocol using methylene blue (1%) dye. All procedures were recorded on videotape, but only 20 (10 with atrophy and 10 normal) were used in a blinded, independent, randomized analysis by five reviewers to evaluate interobserver agreement. Endoscopic signs indicative of mucosal atrophy were as follows: reduction in the number or loss of Kerkring's folds, "scalloped" folds, "mosaic pattern," and visualization of the underlying blood vessels. RESULTS: Eighty-seven patients had celiac disease (57 newly diagnosed, 30 when treated). Seven treated patients had nonatrophic mucosa. In 80 patients the final diagnosis excluded celiac disease. Videoendoscopic inspection alone correctly identified 75 of 80 patients with complete mucosal atrophy and 86 of 87 with normal mucosa. False-negative diagnoses occurred in treated celiac patients with mild atrophy. Mosaic pattern (89%) and scalloped folds (86%) were the most useful endoscopic signs. Vital dye staining, as assessed by experienced endoscopists, provided identical results to those obtained by inspection alone. Sensitivity, specificity, and positive and negative predictive values for the presence of one or more than one feature were 94%, 100%, 100%, and 96%, respectively. The agreement (kappa statistics) among observers was excellent for the mosaic pattern (kappa: 0.76 for both the videoendoscopic inspection alone and dye staining) and the scalloped folds (kappa: 0.83 and 0.76, respectively) and was fair (kappa: 0.41 and 0.59, respectively) for the reduction in the number or loss of duodenal folds. CONCLUSION: This study confirms that videoduodenoscopy is useful in the detection of intestinal atrophy. Dye staining produces a better delineation of scalloped folds and mosaic pattern in the atrophic mucosa, but did not provide additional information to the expert endoscopist. Finally, interobserver agreement was excellent for the most prevalent signs.

Longitudinal study on the effect of treatment on body composition and anthropometry of celiac disease patients.

OBJECTIVES: This prospective study was designed to assess the nutritional changes associated with the long-term treatment of celiac disease. In addition, we analyzed whether these changes were related to the degree of compliance with a gluten-free diet. METHODS: We studied nutritional parameters and body composition in 25 newly diagnosed celiac patients after a mean period of 37 months (range 25-49 months) on a gluten-free diet. Body composition parameters (fat, lean tissue, and bone masses) were measured by dual energy x-ray absorptiometry. Anthropometry was measured according to conventional formulas. RESULTS: At diagnosis, fat (-49%), lean tissue (-12%), and bone (-24%) compartments were reduced, compared with that of sex- and age-matched controls. After treatment, we noted a significant increase in body weight (p < 0.0001), fat mass (p < 0.0005), bone mass (p < 0.002), and body mass index (p < 0.005). In contrast, we did not observe a significant increase in lean-tissue mass or muscle mass. Patients who adhered strictly to a gluten-free diet experienced a greater, though nonsignificant improvement in fat mass, body weight, and body mass index than patients whose compliance had been partial. Mean caloric intake at the end of the study was significantly lower among those patients who had adhered strictly to a gluten-free diet, compared with those who had complied only partially with the diet (p < 0.05). CONCLUSIONS: This study shows that the institution of a gluten-free diet in celiac disease patients results in a significant improvement in nutritional parameters, as measured by anthropometry and/or body composition. This effect was more pronounced in patients who followed strict gluten restriction and was related mainly to changes in fat and bone compartments.

Effect of treatment on bone mass, mineral metabolism, and body composition in untreated celiac disease patients.

BACKGROUND/AIMS: Osteopenia is a common complication of celiac disease. The aims of this study were to evaluate whether treatment produces bone remineralization and whether calcium and vitamin D supplementation are necessary to reduce osteopenia. METHODS: Bone mineral density and biochemical parameters of bone and mineral metabolism were measured in 14 newly diagnosed adult celiac disease patients. All patients were treated with a gluten-free diet and were randomized to receive diet only (n = 7) or diet plus calcium (1.0 g/day) and vitamin D (32,000 IU/wk) supplementation (n = 7). Bone density was measured at baseline and at 6 and 12 months of follow-up. Tests for biochemical determinations were repeated every 3 months. RESULTS: At diagnosis, 11 patients had evidence of osteopenia (> 1 SD below normality) in the spine and total skeleton. After 12 months of gluten restriction, overall bone mass had increased 5.0% (p < 0.01) in the lumbar spine and 5.0% (p < 0.002) in the total skeleton. When one only considers those 11 patients who strictly followed gluten restriction, bone density increased 8.4% in the lumbar spine and 7.7% in the total skeleton. Remineralization occurred throughout the skeleton but was more pronounced in the axial than in the peripheral skeleton. The increase in bone mass was independent of age or menopause. Remineralization in patients treated with diet only was similar to that of patients treated with diet and supplements. Basal biochemical parameters showed a high bone turnover with secondary hyperparathyroidism. Treatment induced a decrease in bone turnover activity. However, a complete restoration of biochemical parameters was not achieved. CONCLUSIONS: Strict gluten avoidance promoted a significant increase in bone mineral density. However, values still remain markedly low after 1 yr in several patients. Although calcium and vitamin D supplementation did not provide additional benefit to that obtained by diet alone in the doses administered, our results do not preclude a possible effect of vitamin D at higher dose.

Long-term effect of gluten restriction on bone mineral density of patients with coeliac disease.

AIM: To assess the long-term effect of a gluten-free diet on bone mineral density of adults with untreated coeliac disease. METHODS: Bone mineral density was assessed at baseline and after a mean duration of 37 months of treatment in 25 unselected newly diagnosed coeliac patients. RESULTS: At baseline, osteopenia (> -1 s.d. below normal) was evident in the lumbar spine and total skeleton in 18 (72%) and 21 (84%) patients, respectively. At the end of the study, bone density had increased (mean bone mass Z-score increase: Z-score +1.0 for the lumbar spine and +1.1 for total skeleton) in 22 and 23 patients, respectively. Patients who adhered to strict gluten restriction (n = 15) demonstrated a similar bone remineralization in the spine than those patients with partial compliance (n = 10) (mean Z-score increase: +1.0, in both areas). A greater mean annual change in Z-score in the total skeleton was noted in patients who followed strict gluten restriction (0.4 +/- 0.1) respect to those with partial compliance (0.3 +/- 0.1); however, this difference was not statistically significant. Pre-menopausal women had significantly greater remineralization that post-menopausals (P > 0.05). Remineralization showed an inverse correlation with the degree of basal osteopenia (r = -0.525; P < 0.002). CONCLUSIONS: Long-term treatment with gluten-free diet produces a significant improvement in bone density in coeliac patients. Remineralization was more pronounced in patients who better comply with gluten-free diet, in pre-menopausal women and in patients with the lowest baseline bone mineral density.

Impaired monocyte function in patients succesfully treated for Wipple's disease

Peripheral blood mononuclear cells (monocytes) from patients with Whipple's disease in long-term remission were tested for their ability to handle intracellular microorganisms. Phagocytosis and lysis of Candida tropicalis by monocytes of patients (n=12) andcontrols (n=8) were quantified after 30 min of incubation. Phagocytosis was similar in both groups but intracellular Killing of Candida tropicalis was significativily lower in patients (p<0.001). We concluded that our study showed an in vitro defect in the intracellular Killing function of monocytes in subjects in remission many years after diagnosis of Whipple's disease. The defective function did not seem to be related to relapse or to the susceptibility to other infections.

Impaired monocyte function in patients succesfully treated for Wipples disease

Peripheral blood mononuclear cells (monocytes) from patients with Whipples disease in long-term remission were tested for their ability to handle intracellular microorganisms. Phagocytosis and lysis of Candida tropicalis by monocytes of patients (n=12) andcontrols (n=8) were quantified after 30 min of incubation. Phagocytosis was similar in both groups but intracellular Killing of Candida tropicalis was significativily lower in patients (p<0.001). We concluded that our study showed an in vitro defect in the intracellular Killing function of monocytes in subjects in remission many years after diagnosis of Whipples disease. The defective function did not seem to be related to relapse or to the susceptibility to other infections. (AU)

Serological markers identify histologically latent coeliac disease among first-degree relatives.

BACKGROUND AND AIM: Serological markers detect asymptomatic coeliac disease among first-degree relatives of patients with sprue. However, some relatives with coeliac disease-related antibodies have 'normal' jejunal mucosa by conventional histology. Whether these serological abnormalities represent false-positives or are consequences of gluten sensitivity is not known. Our aim was to evaluate, through quantitative histology, intestinal biopsies of asymptomatic relatives of probands seeking abnormalities consistent with latent coeliac disease. MATERIALS: Fifty-nine intestinal biopsies obtained from asymptomatic relatives were evaluated; 40 samples were suitable for histological quantification. Seven samples showed severe mucosal atrophy (coeliac disease) and 33 were considered as 'normals'. In the 'normal' group, nine samples were obtained from patients with one or more positive serological tests and 24 from those with negative tests. Morphometry was compared for samples obtained from healthy control individuals (n = 10) and for those from coeliac patients (n = 7). METHODS: Serological tests used were: antigliadin antibodies type immunoglobulin (Ig)A and IgG (enzyme-linked immunosorbent assay), antirrecticulin antibody (immuno-fluorescence) and endomysial antibody (immunofluorescence). Biopsy samples were obtained with endoscopic forceps from the distal duodenum (second portion). Quantitative histology of duodenal biopsies was performed with a computerized image analysis system. RESULTS: Relatives with positive serology showed shorter villi (P < 0.05) and higher number (P < 0.01) and numerical density (P < 0.01) of intraepithelial lymphocytes in crypts than healthy controls. Numerical density of intraepithelial lymphocytes in crypts in antibody-positive patients was significantly higher than that observed in relatives with negative serology (P < 0.03). Four of nine (44%) relatives with positive serology had a number of intraepithelial lymphocytes in crypts within the range of coeliac disease patients. However, only one patient with negative serology (4%) was in this range. CONCLUSION: Our study shows quantitative histological evidence that relatives of probands with positive coeliac disease-related serology are not false-positives, and that they should be considered as individuals with latent coeliac sprue.

Increased mucosal levels of leukotriene B4 in pouchitis: evidence for a persistent inflammatory state.

DESIGN AND METHODS: In order to evaluate its possible role in the pathogenesis of pouchitis we measured the release, into the incubation medium of leukotriene B4 from mucosal samples from patients with ileal pouch-anal anastomosis and correlated release with clinical, endoscopic and histological features. RESULTS: Leukotriene B4 release was significantly elevated in patients with active pouchitis in comparison to those with a normal pouch mucosa (P < 0.007). No overlap was observed between leukotriene B4 levels from patients with active pouchitis samples and those obtained from individuals without pouchitis. Effective treatment of pouchitis was associated with a significant reduction in leukotriene B4 mucosal release to the incubation medium (P < 0.03). However, even in remission, levels of leukotriene B4 release remained significantly increased in these patients in comparison to people who never experienced pouchitis (P < 0.003). A modest correlation was observed between pouchitis disease activity index and leukotriene B4 release (r = 0.596; P < 0.01). CONCLUSION: These results suggest that the increased production of leukotriene B4 may be implicated in the pathogenesis of pouchitis. The persistence of an increased mucosal release of leukotriene B4 in pouchitis patients during clinical remission suggests the presence of a chronic, ongoing, underlying inflammatory process.

Impaired monocyte function in patients successfully treated for Whipple's disease.

Peripheral blood mononuclear cells (monocytes) from patients with Whipple's disease in long-term remission were tested for their ability to handle intracellular microorganisms. Phagocytosis and lysis of Candida tropicalis by monocytes of patients (n = 12) and controls (n = 8) were quantified after 30 min of incubation. Phagocytosis was similar in both groups but intracellular killing of Candida tropicalis was significatively lower in patients (p < 0.001). We concluded that our study showed an in vitro defect in the intracellular killing function of monocytes in subjects in remission many years after diagnosis of Whipple's disease. The defective function did not seem to be related to relapse or to the susceptibility to other infections.

Faecal alpha 1-antitrypsin concentration in the diagnosis and management of patients with pouchitis.

OBJECTIVE: To investigate the role of faecal alpha 1-antitrypsin concentration in the diagnosis and management of patients with ileal pouch-anal anastomosis. DESIGN: Prospective study. METHODS: Fifty-two measurements of faecal alpha 1-antitrypsin concentration were taken from 33 patients operated on for ulcerative colitis. RESULTS: Patients with active pouchitis (44.4 +/- 7.1 mg%) had a three-fold higher mean faecal alpha 1-antitrypsin concentration than patients in remission (13.7 +/- 1.3 mg%; P < 0.0001), than patients who had never had pouchitis (14.4 +/- 2.3 mg%; P < 0.003) and than patients with incontinent ileostomies (12.7 +/- 1.3 mg%; P < 0.004). Faecal alpha 1-antitrypsin measurements were 80% sensitive and 97% specific for active pouchitis. A significant positive correlation between the pouchitis disease activity index and faecal protein loss was observed (r = 0.702; P < 0.0001). The correlations between protein loss and other parameters were weaker (protein loss versus clinical score, r = 0.309; versus endoscopic score, r = 0.583; and versus histologic score, r = 0.558). CONCLUSION: Faecal alpha 1-antitrypsin concentration is a good indicator of the degree of intestinal inflammation in pouchitis and may be useful as a quantitative index of disease activity in prospective studies.

Abnormal colonic transit time in untreated celiac sprue.

Motility disorders of the digestive tract have long been implicated in the pathophysiology of diarrhea in patients with celiac sprue. However, the contribution of the colon to the intestinal transit of celiac sprue has not been reported. Our aim was to determine whether sprue alters gut transit and whether differences in the clinical status of the disease influences colonic transit. We prospectively studied 25 patients with untreated celiac sprue, 15 treated patients and 15 healthy controls. Oro-cecal transit time, measured by the lactulose breath H2 test, was significantly delayed in untreated patients compared with treated patients and controls (p < 0.001 and p < 0.01 respectively). The delayed transit through the stomach and small bowel was not related to the presence of the steatorrhea. Transit of radiopaque makers, a measure of total colonic transit, was significantly faster in untreated patients (p < 0.05). The major finding was that this abnormal colonic behavior was principally due to a subpopulation of untreated patients with very fast transit times (< 18 hours). A weakly significant inverse correlation between transit and fecal weight (r: -0.55, p < 0.01), and between transit and steatorrhea (r: -0.38, p < 0.05), was observed. We confirm previous descriptions of delayed oro-cecal transit time in untreated patients, and also provide the first evidence that disordered colonic transit contributes to the pathophysiology of the diarrhea in sprue.

Abnormal colonic transit time in untreated celiac sprue

Motility disorders of the digestive tract have long been implicated in the pathophysiology of diarrhea in patients with celiac sprue. However, the contribution of the colon to the intestinal transit of celiac sprue has not been reported. Our aim was to determine whether sprue alters gut transit and whether differences in the clinical status of the disease influences colonic transit. We prospectively studied 25 patients with untreated celiac sprue, 15 treated patients and 15 healthy controls. Oro-cecal transit time, measured by the lactulose breath H2 test, was significantly delayed in untreated patients compared with treated patients and controls (p<0.001 and p<0.01 respectively). The delayed transit through the stomach and small bowel was not related to the presence of the steatorrhea. Transit of radiopaque makers, a measure of total colonic tansit, was significantly faster in untreated patients (p<0.05). The major finding was that this abnormal colonic behavior was principally due to a subpopulation of untreated patients with very fast transit times (<18 hours). A weakly significant inverse correlation between transit and fecal weight (r:-0.55, p<0.01), and between transit and steatorrhea (r:-0.38, p<0.05), was observed. We confirm previous descriptions of delayed oro-cecal transit time in untreated patients, and also provide the first evidence that disordered colonic transit contributes to the pathophysiology of the diarrhea in sprue.

Abnormal colonic transit time in untreated celiac sprue.

Motility disorders of the digestive tract have long been implicated in the pathophysiology of diarrhea in patients with celiac sprue. However, the contribution of the colon to the intestinal transit of celiac sprue has not been reported. Our aim was to determine whether sprue alters gut transit and whether differences in the clinical status of the disease influences colonic transit. We prospectively studied 25 patients with untreated celiac sprue, 15 treated patients and 15 healthy controls. Oro-cecal transit time, measured by the lactulose breath H2 test, was significantly delayed in untreated patients compared with treated patients and controls (p < 0.001 and p < 0.01 respectively). The delayed transit through the stomach and small bowel was not related to the presence of the steatorrhea. Transit of radiopaque makers, a measure of total colonic transit, was significantly faster in untreated patients (p < 0.05). The major finding was that this abnormal colonic behavior was principally due to a subpopulation of untreated patients with very fast transit times (< 18 hours). A weakly significant inverse correlation between transit and fecal weight (r: -0.55, p < 0.01), and between transit and steatorrhea (r: -0.38, p < 0.05), was observed. We confirm previous descriptions of delayed oro-cecal transit time in untreated patients, and also provide the first evidence that disordered colonic transit contributes to the pathophysiology of the diarrhea in sprue.

Abnormal colonic transit time in untreated celiac sprue

Motility disorders of the digestive tract have long been implicated in the pathophysiology of diarrhea in patients with celiac sprue. However, the contribution of the colon to the intestinal transit of celiac sprue has not been reported. Our aim was to determine whether sprue alters gut transit and whether differences in the clinical status of the disease influences colonic transit. We prospectively studied 25 patients with untreated celiac sprue, 15 treated patients and 15 healthy controls. Oro-cecal transit time, measured by the lactulose breath H2 test, was significantly delayed in untreated patients compared with treated patients and controls (p<0.001 and p<0.01 respectively). The delayed transit through the stomach and small bowel was not related to the presence of the steatorrhea. Transit of radiopaque makers, a measure of total colonic tansit, was significantly faster in untreated patients (p<0.05). The major finding was that this abnormal colonic behavior was principally due to a subpopulation of untreated patients with very fast transit times (<18 hours). A weakly significant inverse correlation between transit and fecal weight (r:-0.55, p<0.01), and between transit and steatorrhea (r:-0.38, p<0.05), was observed. We confirm previous descriptions of delayed oro-cecal transit time in untreated patients, and also provide the first evidence that disordered colonic transit contributes to the pathophysiology of the diarrhea in sprue. (AU)

Steatocrit: a reliable semiquantitative method for detection of steatorrhea.

Steatocrit is a semiquantitative method for determination of fat content in fecal samples. Previous studies, mostly performed in children, reported controversial results. The aim of our study was to compare the determination of fat content in 148 fecal samples by two methods: the conventional van de Kamer and the steatocrit. Seventy-seven fecal samples had steatorrhea (> 7 g/day). The upper normal limit for the steatocrit (determined by the mean +/- 2 SD of samples without steatorrhea) was 2.1%. The steatocrit showed a sensitivity of 87%, specificity of 97%, and positive and negative predictive values of 97 and 87%, respectively. When fecal fat excretion > 20 g/day was evaluated, sensitivity increased to 98%. A significant linear correlation was found between steatocrit and the quantitative chemical method (r = 0.80; p < 0.0001). In conclusion, the steatocrit is satisfactory in the discrimination of patients with and without fat malabsorption. It is a simple, rapid, inexpensive, and reliable semiquantitative test that can be used when other methods are impractical.

Increased rDNA transcriptional activity in celiac disease.

The activity of nucleolar organizer regions (NORs) in chromosomes of peripheral blood lymphocyte cultures from 20 healthy subjects and 32 patients with celiac disease (CD) (nine untreated patients, nine treated but with dietary lapses, and 14 treated with gluten-free diet (GFD) and normal small bowel histology) was studied. Furthermore, three female patients diagnosed with small bowel non-Hodgkin's lymphoma (NHL) complicating CD were studied. Silver (Ag)-staining technique was used to visualize positive NORs. In each individual, 20 metaphases were analyzed to determine the number of NORs per cell. The average of Ag-NOR+ per cell, expressed as mean +/- SD, was found to be higher in the CD group (6.62 +/- 0.65) compared with controls (5.70 +/- 0.81) (p less than 0.001). This increase was evident in both groups of chromosomes analyzed (D and G). No differences were found among the three groups, but all of them were found to be statistically different compared to controls (p less than 0.001). The NHL complicating CD patients showed a statistically increased frequency of Ag-NORs (7.20 +/- 0.39) with respect to CD patients (p less than 0.02) and controls (p less than 0.001). These findings show an increase of the transcriptional activity of rDNA in CD that could be related to the high incidence of malignancy in this pathology. Longitudinal studies of CD patients should be performed to confirm this evidence.

Class I and class II HLA antigens in a homogeneous Argentinian population with Whipple's disease: lack of association with HLA-B 27.

The prevalence of class I and class II HLA antigen was analyzed in 14 patients (12 males, two females) with Whipple's disease, diagnosed an average of 9.7 yr (range 6 months to 25 yr) before the typing. They were compared with 174 healthy control subjects of the same geographic area in Argentina. Class I antigens (locus A, B, C) were studied by lymphocytotoxic test, and class II antigens (locus DR, DQ) were detected by the double immunofluorescence technique. HLA-B27 was positive in one patient (7.7%) and in 4% of the control population. No significant association was found with the antigens tested. We observed no difference in the clinical picture or in the frequency of arthralgias, compared with those reported in the literature. Our data suggest that there is no conclusive proof of an association between HLA-B27 and Whipple's disease.

Short-term antibiotic treatment in Whipple's disease.

We report the results of short-term antibiotic treatment in 19 patients with Whipple's disease (WD). The diagnosis was based on clinical features and on a characteristic small bowel biopsy. Patients received treatment for a mean of 7.9 weeks (range 4-20). Fourteen were treated with de-methyl-chlortetracycline (600 mg/day), and 1 also received chloramphenicol (1 g/day); 1 was treated with ampicillin (2 g/day), and 4 were treated with amoxicillin (1.5 g/day). In all patients, the clinical response was rapid and excellent, body weight increased significantly, diarrhea subsided, and fecal fat values returned to normal. Intestinal biopsies obtained after treatment was completed showed significant improvement based on a decrease in the number of macrophages staining positive with periodic acid-Schiff (PAS), normalization of villous structure, and decreased dilatation of lymphatic channels; free bacilli were absent, as shown both by light and electron microscopy. Seventeen patients have been followed for a mean of 99.4 months (range 6-300). Two died 30 and 72 months after diagnosis of Whipple's disease, 1 of laryngeal carcinoma and the other of colonic carcinoma. Fifteen patients are in excellent health. Three patients treated with tetracycline have had clinical and/or histologic relapses. In our experience, short-course antibiotic treatment with tetracycline or ampicillin and derivatives can be effective in WD, with few relapses and excellent outcome. No neurologic symptoms, either initially or during follow-up were observed.

Gluten challenge in patients with celiac disease: evaluation of alpha 1-antitrypsin clearance.

Our aim in this study was to monitor changes of the intestinal structure by alpha 1-antitrypsin clearance (alpha 1-ATCL) in order to offer an alternative to the gluten challenge biopsy. In addition, we evaluated the possibility of reducing the time of gluten challenge. Twelve patients had a presumptive diagnosis of celiac disease based on clinical and histological grounds. They were studied when the jejunal histology was normal after gluten-free diet and an alpha 1-ATCL was normal. The gluten was introduced by returning to a normal diet. The challenge lasted 4 wk. We measured alpha 1-ATCL at the end of the 1st and 4th wk, and a new jejunal biopsy was obtained at the end of the 4th wk. By wk 1, alpha 1-ATCL was abnormal in 11 patients but normal in one. By wk 4, alpha 1-ATCL was abnormal in 10 patients and still normal in one. The post-challenge biopsies showed atrophy in 11 and was normal only in the patient with normal alpha 1-ATCL at wk 1 and 4. One patient with abnormal alpha 1-ATCL had to stop the challenge at the first week. The patient with normal clearance at wk 1 and 4 and normal biopsy at wk 4 had abnormal results at 6 months. These data support our hypothesis that alpha 1-ATCL can be used as evidence of gluten toxicity after gluten challenge, and that this test can be abnormal as early as 1 wk after gluten is reintroduced.

[Primary lymphoma of the small bowel and malabsorption]. / Linfoma primario de intestino delgado y malabsorción.

Between 1974 and 1984 we saw 69 patients with lymphoma that involved the gastrointestinal tract. In ten patients the lymphoma compromised the small bowel and were associated to malabsorption. Seven patients fulfilled the criteria to be considered as primary small bowel lymphoma. We presumed the intestinal origin in the other 3 patients, but it was impossible to confirm it. The peroral small bowel biopsy showed histological findings compatible with celiac disease in 7 patients. Other particular histological signs were patchy alterations, inconstant epithelial pseudo-stratification and ulcerations. In 2 cases we found findings that suggested the diagnosis of lymphoma. In 50% of patients we found unspecific malabsorption signs in the small bowel radiology. We found giant ulcers and stenosis too. The gluten-free diet or the steroid therapies resulted in temporary or inconstant improvement. The laparotomy was the most effective diagnostic approach. It was performed electively in 6 patients and in 1 because of a small bowel perforation. The primary small bowel lymphoma is an entity of difficult diagnosis. The most important trouble is to differentiate it with celiac disease.