Immunoglobulin deficiency, especially deficiency of IgA, has been described in patients with celiac sprue (CS). Our study was performed in an area of high prevalence of CS to determine the prevalence of immunodeficiency states in patients with CS, to examine their clinical characteristics, response to treatment, and HLA phenotypes compared with a group of age- and sex-matched persons with CS but without immunoglobulin deficiency. Fourteen of 604 patients with CS were identified as being selectively deficient in IgA, whereas one had common variable immunodeficiency. At diagnosis, anemia was present in 8 of 14 IgA-deficient patients compared with 10 of 42 controls (p = 0.047), whereas abdominal pain was more common in controls with CS. Autoimmunity and recurrent infection were more prevalent in the IgA-deficient group. Response to gluten-free diet was similar in both groups in terms of histologic structure and recovery of intestinal brush-border enzyme activity. IgA-deficient participants with CS had no increased risk of associated malignancy or lymphoma. HLA phenotypes were similar in both groups. The prevalences of selective IgA deficiency and common variable immunodeficiency in this series of patients with CS are 2.31 in 100 and 0.16 in 100, respectively. Although this group is unique in character, close follow-up coupled with conscientious compliance with a gluten-free diet, remains the mainstay of treatment for these patients.

SUMMARY Between 1960 and 1974 the incidence of coeliac disease in children under 12 years in County Galway remained fairly constant, but since 1975 it has fallen by 62% and the lowered incidence seems well established. The number of those who were breast fed and the age at which first gluten feeding took place during the 22 years both increased significantly and from the mid-70s total protein content and osmolarity of proprietary cow's milk formulas were reduced. All three factors may be relevant. A negative correlation with the incidence of gastroenteritis was found.

SUMMARY Lactase, sucrase, and alkaline phosphatase activities were measured in 833 peroral small intestinal biopsies from 373 patients with coeliac disease. Enzyme activities decreased with increasing degrees of mucosal damage. Enzyme activities in mucosae of patients with coeliac disease in remission were lower than in control groups matched for age, sex, and site of biopsy. Enzyme activities were measured in 81 patients when the mucosa was severely damaged and later when considerable improvement had occurred. Lactase activity remained low in 13% of patients under the age of 18 and in 33% of those over 18 years. Sucrase activity usually improved with histological recovery, but alkaline phosphatase activity tended to remain depressed in patients in whom lactase activity failed to improve.Activities of small intestinal brush border enzymes such as lactase, sucrase, and alkaline phosphatase are reduced in patients with untreated coeliac disease but recover again during remission.'-6 It has been suggested that the measurement of such enzymes may be used as a quantitative index of improvement of the small intestinal mucosa. The rates of recovery of the aforementioned enzymes vary: sucrase activity recovers more rapidly than lactase, which may remain severely depressed for many years especially in older patients.4 We have previously described the influence of age, sex, and site of intestinal biopsy on enzyme activity in control subjects.' This paper investigates the importance of these factors in a large group of patients with coeliac disease with due regard to the histological state of the intestinal mucosa. We also present an analysis of data from 81 patients where mucosal enzyme activity was examined in severely damaged mucosae and subsequently when the mucosae had improved considerably with the withdrawal of gluten from the diet. Material and methodsA total of 833 small intestinal biopsies were studied in 373 coeliac patients (218 female, 155 male) over 10 years. In all cases coeliac disease was diagnosed Accepted for publication 28 September 1983 on the basis of severe small bowel mucosal damage with histological or clinical improvement, or both, on a gluten free diet. Patients' ages ranged from 1 to 78 years, and 230 of the patients were under the age of 18 when first studied. Enzyme data are presented on biopsies from 157 patients originally on normal diets and subsequently on gluten free diets, 126 patients on gluten free diets only, and 90 patients on normal diets only. The patients were divided into three age groups: 0 to 18 years, 19 to 45 years, and 45+ years.Small intestinal biopsies were obtained and processed as previously described.7 Biopsies were obtained from the proximal duodenum (site A) in 40 cases, distal duodenum (site B) in 533 cases, and jejunum (site C) in 240 cases and were graded histologically on a scale of 0-3 as described by McNicholl and Egan.' Briefly these gradings are: grades 0/1 = normal or minor abnormalities, grade 2 = moderate mucosal damage, and grade 3 = severe mucosal dama...

Serum immunoglobulins were measured in one hundred and eighty‐four healthy controls. One hundred and fifty‐nine of these were also HLA typed. IgG levels did not differ with sex or HLA‐B8 status. IgM levels did not differ with HLA‐B8 status but were significantly higher in females than males. IgA levels were lower in females than males; they were also lower in HLA‐B8 positive compared with HLA‐B8 negative individuals; the lowest IgA levels were found in HLA‐B8 positive females. The IgA variation may be relevant to the higher incidence of certain disorders among HLA‐B8 positive individuals and among females.