Purpura-free small intestinal IgA vasculitis complicated by cytomegalovirus reactivation

IgA vasculitis (Henoch-Schönlein purpura) affects various organs, including the skin, gastrointestinal (GI) tract, joints and kidneys. Its clinical course typically consists of two phases: initial appearance of purpura and delayed onset of arthralgia, GI symptoms and haematuria. We report the case of an adult patient with IgA vasculitis of the small bowel, without skin involvement, complicated by cytomegalovirus (CMV) enteritis following prednisolone administration. Single-balloon enteroscopy revealed mucosal oedema, redness, erosions and transverse ulcers of the duodenum and jejunum. Jejunal biopsy specimens showed IgA deposition in the capillary walls. CMV reactivation was confirmed by PCR and immunostaining using jejunal biopsy specimens. This case report strongly suggests that adult patients with IgA vasculitis can present with isolated GI involvement, without characteristic skin purpura. Furthermore, CMV reactivation needs to be considered in patients with IgA vasculitis showing poor response to glucocorticoids.

Behavioral and Cognitive Manifestations of Celiac Disease

Celiac disease (CD) is an immune-mediated chronic inflammatory disorder characterized by permanent gluten intolerance in genetically susceptible individuals. Exposure to gluten perpetuates an enteropathy leading to malabsorption with chronic diarrhea, weight loss, and abdominal distension. The small intestine mucosa is abnormal, and jejunal biopsy demonstrates various degrees of villous atrophy, absence of surface mucosa, and crypt hyperplasia. The diagnosis is based on the demonstration of a more or less pronounced villus atrophy in a jejunal biopsy. The villous atrophy improves after withdrawal of gluten from the diet. If undetected or neglected, CD may cause considerable late complications from malabsorption or secondary autoimmune diseases (Feigbery 1999; Maki and Collins 1997; Holmes 1996). The therapy consists of permanently excluding gluten from the diet and allows the healing of the mucosal lesion. Abnormalities of humeral and cell-mediated immunity suggest that celiac disease is an immunologic disorder (Walker-Smith 1996). It is caused by inappropriate immune response to the gliadin component in the dietary gluten (Dieterich 1997). Genetic susceptibility is present, and 90% of the patients have HLA DRG 3 DQ-2 haplotype, and some have the HLA DR4 DQ8 gene (Hadjivassiliou 1998). A close relationship exists between the biochemical properties of tissue transglutaminase and the basic molecular mechanisms responsible for CD, and possibly with the neuropsychiatric manifestations of CD (Gentile 2002). Anti–tissue transglutaminase antibody assay has been used as a serologic screening test for CD. In addition, antiendomysial, antigliadin, and antireticulin antibodies are associated with the disease. Nevertheless, the clinical symptomatology affecting the gastrointestinal (GI) system, histological abnormalities on gut biopsy, and presence of antiendomysial antibodies do not always coexist. Also, presentation with minor symptoms, such as irritable bowel syndrome, anaemia, slight weight loss, and fatigue, has become increasingly common, and in many cases the disease may be clinically silent, despite manifest small-bowel mucosal lesions. Therefore, CD is underdiagnosed (Catassi et al. 1996; Feigbery 1999; Holmes 1996; Kolho et al. 1998; Maki and Collins 1997).

Serological screening for celiac disease in symptomatic 12 to 36 month-old children

CONTEXT: The correct diagnosis of celiac disease in environmentally deprived children is frequently hindered by the common presence of other causes for the classical celiac disease symptoms: malnutrition, failure to thrive and frequent diarrheas. OBJECTIVES: To determine the prevalence of celiac disease in a group of 12 to 36 month-old children using immunoglobulin antibodies against gliadin (IgG and IgA-AGA), against endomysium (IgA-EMA), and against human tissue transglutaminase (IgA-tTG) as screening method. METHODS: A total of 214 children (114 boys), aged 12 to 36 months, on gluten-containing diet, were admitted to the study. IgG and IgA-AGA, IgA-tTG and IgA-EMA tests were performed in all sera. Biopsy was obtained from all children showing positive result in one or more of the serologic tests, excluding those in which IgG-AGA had been the only positive result. In those cases, polymerase chain reaction (PCR) HLA genotyping for the identification of celiac disease predisposing alleles was applied. HLA genotyping was also performed to confirm the diagnosis in children identified as celiac by means of positive serologic testing and compatible biopsy results. RESULTS: Normal results were obtained in 131 children. Ten children out of 68 identified as positive exclusively on the IgG-AGA test disclosed the presence of celiac disease predisposing alleles on PCR and underwent jejunal biopsy with normal results. All serologic tests were positive in four children. A fifth child showed positive IgG and IgA-AGA and IgA-tTG results but disclosed a negative IgA-EMA test. Jejunal biopsy of these five children revealed characteristic lesions of celiac disease. CONCLUSION: A prevalence of 2.3% was found among symptomatic 12- to 36-month-old children that had not been previously diagnosed as celiac.

Pediatric Celiac Disease: Clinicopathologic and Genetic Aspects

Evaluation for celiac disease (CD), an autoimmune enteropathy triggered by grain proteins in wheat, barley, rye, and possibly oats, is a common indication for pediatric endoscopy and biopsy. Duodenal or jejunal biopsy remains key for the initial diagnosis of CD. Small intestinal pathology may be diffuse or focal in CD, and histologic findings are nonspecific and must be interpreted in conjunction with clinical and serologic findings. A standardized grading system for CD biopsies is recommended. This review article summarizes the epidemiology, clinical manifestations, genetics, pathogenesis, and serologic and histologic findings of CD. Clinical management of CD and general handling of small intestinal biopsies are also addressed.