Clinical outcome of Crohn's disease: analysis according to the vienna classification and clinical activity.

The aim of this study was to describe the clinical course of Crohn's disease (CD) in a well-defined, homogeneous groups of patients. A total of 480 patients with CD were followed up from diagnosis up to 20 years. Definitions of patient subgroups were made according to the Vienna Classification. Markov chain analysis was used to estimate the probabilities of remissions and relapses during the disease course. Both age at diagnosis and behavior were associated with different disease locations. Patients with ileal disease had a greater need for surgical and a lesser need for immunosuppressive treatment; patients with ileocolonic disease were diagnosed at an earlier age and showed a lower probability of remaining in remission during the disease course; patients with colonic disease needed less surgical or steroid treatments; patients with intestinal penetrating disease were frequently submitted to abdominal surgery, whereas those with anal-penetrating disease often needed immunosuppressive treatment. Approximately 40% of the patients were in clinical remission at any time, but only about 10% maintained a long-term remission free of steroids after their initial presentation. A more benign clinical course could be predicted in patients who stay in remission in the year after diagnosis. The grouping of patients with CD according to the Vienna Classification and/or the clinical activity in the year after diagnosis is useful in predicting the subsequent course of disease.

Small-bowel changes in recurrent ulceration of the mouth.

Jejunal biopsies of twenty-four patients with aphthous ulcers in whom malabsorption was not found, were studied. Four patients (16%) had subtotal villous atrophy and this structural change reversed after gluten withdrawal. There was a significant increase in intra-epithelial lymphocytes in the aphthous patients compared with healthy controls (p less than 0.001). The lymphocytic infiltration of the epithelium was higher, even in specimens with normal morphology (p less than 0.001). The findings suggest that a significant number of patients with recurrent aphthous stomatitis may have a mild form of gluten enteropathy.

Hepatic abscess in Crohn's disease.

Hepatic abscess is a rare complication of Crohn's disease. The present report describes an additional case diagnosed by CT scanning and successfully treated by surgery. Recognition of this complication is important because early therapy could improve the prognosis.

Macroglobulinemia and small intestinal disease. A case report with review of the literature.

Involvement of the gastrointestinal tract in macroglobulinemia is exceedingly rare. We describe a patient with IgM biclonal immunoglobulin disorder associated with diffuse lymphoplasmacytic infiltration of the small intestine. This chronic illness was characterized by diarrhea, steatorrhea, and intestinal pseudo-obstruction. Full-thickness biopsy specimens of the jejunum showed stunted and fused villi, giardiasis, and a widespread cellular infiltrate in the lamina propria extending through the muscularis mucosae into the submucosa and muscular layers. The infiltrate had a cytologically benign appearance that was shown to be polyclonal by immunochemical stains. Intestinal vacuolated plasma cells were occasionally observed in electron microscopic study. The patient has not developed the features of Waldenstrom's disease on a clinical follow-up of 14 years.

Immune-related systemic manifestations of inflammatory bowel disease. A prospective study of 792 patients.

The prevalence of immune-related systemic manifestations associated with inflammatory bowel disease varies widely in different studies. This prospective hospital study examines the prevalence and clinical aspects of these manifestations. A total of 792 patients, 449 with Crohn's disease and 343 with ulcerative colitis, were followed up from diagnosis to 20 years. Data related to systemic manifestations and to the extent and severity of intestinal lesions were obtained at diagnosis and during follow-up. Extraintestinal manifestations related to the activity of intestinal inflammation appeared at least once in 25.8% of the patients. The cumulative probability of extraintestinal manifestations increased from 12%-30% during the 20-year follow-up; it was significantly higher in patients with Crohn's disease (p < 0.01). Arthritis (p < 0.001) and erythema nodosum (p < 0.01) were more frequent in Crohn's disease. A significant positive association of arthritis with skin, eye, and mouth complications was observed (p < 0.001). Bowel-related systemic manifestations appeared frequently together in the same patient (p < 0.001). Patients with any of these manifestations ran a higher risk of developing another one. Autoimmune manifestations were associated with both Crohn's disease and ulcerative colitis.

Spontaneous umbilical fistula in Crohn's disease.

Spontaneous umbilical fistula developed in two young women with Crohn's disease. This peculiar complication of Crohn's disease has been rarely reported. In view of the complete closure of the fistulas with medical treatment, we recommended conservative medical management, at least initially, in such patients.

Autoimmune hemolytic anemia in ulcerative colitis. A case report with review of the literature.

Autoimmune hemolytic anemia is a rare complication of ulcerative colitis. This report chronicles a patient successfully treated with steroids, and reviews treatment in previously reported cases. The fact that 21% of patients recovered with steroid therapy encourages a conservative approach before surgery is considered.

Atypical plasma cells in alpha-chain disease.

Pathologic features of the jejunal mucosa of a patient with alpha-chain disease in its early transition to malignant stage, are described. Ultrastructural alterations were seen in some plasma cell, close similar to those described in myeloma plasma cells. Ultrastructural studies of biopsy specimens in alpha-chain disease, may permit recognition of its early transition to malignant stage and thereby encourage earlier use of cytotoxic drugs.

Whipple's disease. Report of a case with clinical immunological studies.

A case of Whipple's disease is described in which humoral and cellular immunity were tested. Histologic ultrastructural and immunocytochemical study were performed, before, during and after treatment. Cutaneous anergy was the main immunological dysfunction observed. During active Whipple's disease the patient had consistently negative skin tests for all antigens tested. This abnormality of delayed hypersensitivity regressed during treatment together with clinical remission of the disease. These findings are considered as secondary to Whipple's disease.

Ulcerative colitis, primary biliary cirrhosis, and chronic pancreatitis: coincident or coexistent?

We report a 45-year-old woman with chronic ulcerative colitis complicated by primary biliary cirrhosis and concomitant chronic pancreatitis. The combination of primary biliary cirrhosis and ulcerative colitis has only rarely been reported, and the association of these diseases with chronic pancreatitis is even rarer. These systemic manifestations of ulcerative colitis should be looked for more often.

Hepatic amebiasis-analysis of 56 cases. II. Laboratory and chest x-ray findings.

Laboratory and chest x-ray findings in 56 cases of hepatic amebiasis previously reported are described. Anemia and an elevated E.S.R., frequently without an elevated leucocyte count, were the most frequent hematologic findings. Abnormalities of liver function tests were mild and nonspecific. The most frequent abnormalities seen in chest x-ray were an elevated right hemidiaphragm whose motility on fluoroscopy was reduced or abolished. The abnormalities may be of great help in diagnosis in endemic areas were more sophisticated facilities may not be available.

Hepatic amebiasis, analysis of 56 cases. I. Clinical findings.

The clinical findings in 56 patients with hepatic amebiasis are reviewed. This illness was most frequent in male black patients, 20-39 years old. The clinical picture is dominated by upper right quadrant abdominal pain, general malaise, pain on percussion of the right hypochondrium and tender hepatomegaly. Jaundice is not rare and appears to have no prognostic significance. The importance of early diagnosis is emphasized.

Quality of health care in inflammatory bowel disease: development of a reliable questionnaire (QUOTE-IBD) and first results.

OBJECTIVES: As inflammatory bowel disease is a chronic disorder, usually with an early onset in life, quality of care plays an important role for patients. The aim of this study was to develop a questionnaire to measure quality of care through the eyes of patients with inflammatory bowel disease. METHODS: Ten generic questions were already available because the questionnaire is based on an existing instrument. Patients with inflammatory bowel disease in seven countries were involved in the development of additional disease-specific items. Validation and first field testing of the total questionnaire (QUOTE-IBD) was performed in The Netherlands. RESULTS: A total of 380 patients cooperated in the development of 13 disease-specific items, with high internal reliability (Cronbach's alpha = 0.83). Another 162 patients were involved in validating and testing of the QUOTE-IBD, which consists of 23 items in total. Pearson's correlation coefficient between QUOTE-IBD and visual analog scale scores of health care items was 0.55. Intraclass correlation coefficient of two assessments was 0.64. First testing showed that patients gave relatively poor marks to some part of health care services, such as providing information about extraintestinal complaints and the psychological as well as physical approach to complaints. CONCLUSIONS: A short, valid, reliable questionnaire was developed to measure the opinions of patients with inflammatory bowel disease on quality of health care. The QUOTE-IBD can be used for identification of areas for improvement, with the aim of optimizing health care in inflammatory bowel disease.

A comparison of budesonide and mesalamine for active Crohn's disease. International Budesonide-Mesalamine Study Group.

BACKGROUND: Crohn's disease is often treated with glucocorticoids or mesalamine. We compared the efficacy and safety of controlled-ileal-release budesonide capsules and slow-release mesalamine tablets in patients with active Crohn's disease affecting the ileum, the ascending colon, or both. METHODS: In a double-blind, multicenter trial, we enrolled 182 patients with scores of 200 to 400 on the Crohn's Disease Activity Index (with higher scores indicating greater disease activity) and randomly assigned 93 to receive 9 mg of budesonide once daily and 89 to receive 2 g of mesalamine twice daily for 16 weeks. The primary efficacy variable was clinical remission, defined as a score of 150 or less on the Crohn's Disease Activity Index. RESULTS: In the analysis of all patients who received at least one dose of study drug, the rates of remission after 8 weeks of treatment were 69 percent in the budesonide group and 45 percent in the mesalamine group (P=0.001); the respective rates after 16 weeks of treatment were 62 percent and 36 percent (P<0.001). Seventy-seven patients in the budesonide group completed the 16 weeks of treatment, as compared with 50 patients in the mesalamine group (P<0.001). The numbers of patients with adverse events were similar in the two groups, but those assigned to budesonide had fewer severe adverse events. Among patients who completed 16 weeks of treatment, the morning plasma cortisol value was normal in 67 percent of budesonide-treated patients and 83 percent of mesalamine-treated patients (P=0.06); 90 percent and 100 percent, respectively, had normal increases in cortisol in response to cosyntropin (P=0.02). CONCLUSIONS: In patients with active Crohn's disease affecting the ileum, the ascending colon, or both, a controlled-ileal-release formulation of budesonide was more effective in inducing remission than a slow-release formulation of mesalamine.