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Collagenous Gastritis in Children: Incidence, Disease Course, and Associations With Autoimmunity and Inflammatory
Timo Käppi1,2, Alkwin Wanders3,4, Mats Wolving5
1Department of Pediatric Gastroenterology, Hepatology and Nutrition, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Göteborg, Sweden.
Insights
Childhood collagenous gastritis (CG) is rare and chronic. While autoimmune predisposition is common, autoimmune diseases are infrequent, with serum calprotectin and amyloid A as potential inflammatory biomarkers.
Area of Science:
- Pediatric Gastroenterology
- Immunology
- Rare Diseases
Background:
- Collagenous gastritis (CG) is a rare disorder with an unknown cause, potentially involving immune mechanisms.
- Understanding the characteristics and autoimmune associations of childhood-onset CG is crucial for diagnosis and management.
Purpose of the Study:
- To determine the incidence and prevalence of childhood-onset CG.
- To describe the clinical, endoscopic, and histologic features of pediatric CG.
- To investigate evidence of autoimmunity and inflammatory activity in children with CG.
Main Methods:
- A longitudinal review of 15 pediatric CG patients in Sweden (2008-2019).
- Analysis of autoantibodies, inflammatory biomarkers (calprotectin, amyloid A, C-reactive protein), and HLA-DQ2/DQ8 genotype.
- Cross-sectional analysis of autoimmune markers and disease course during follow-up.
Main Results:
- Childhood CG incidence was 0.25/100,000 person-years; prevalence was 2.1/100,000. Girls were more affected (4.2:1 ratio).
- Disease remained active throughout a median 4.4-year follow-up. Autoimmune predisposition was common (47% heredity, 40% autoantibodies, 53% HLA-DQ2/DQ8).
- Elevated serum calprotectin (67%) and amyloid A (33%) were observed; C-reactive protein was mostly normal. No autoimmune comorbidities were noted.
Conclusions:
- Childhood-onset CG is a rare condition with a chronic course.
- Autoimmune predisposition is frequent, but overt autoimmune diseases are uncommon in early childhood.
- Serum calprotectin and amyloid A may serve as novel biomarkers for inflammatory activity in CG.
Introduction:
Collagenous gastritis (CG), a rare disorder of unknown etiology, has been postulated to have immune-mediated mechanisms. We investigated (i) the incidence and prevalence of CG in a pediatric population; (ii) the clinical, endoscopic, and histologic characteristics of childhood-onset CG; and (iii) the evidence for autoimmunity and/or inflammatory activity in these patients.
Methods:
Clinical, endoscopic, and histologic data were reviewed longitudinally in a population-based Swedish cohort of 15 patients with childhood-onset CG diagnosed in the period 2008-2019. A set of 11 autoantibodies, 4 blood inflammatory biomarkers, and the human leukocyte antigen DQ2/DQ8 genotype was analyzed cross-sectionally.
Results:
The incidence rate of childhood-onset CG was 0.25/100,000 person-years, with an incidence rate ratio of girls to boys of 4.2 (95% confidence interval, 1.2-15). The prevalence of CG was 2.1/100,000 in children aged younger than 18 years. The endoscopic and histologic findings remained pathologic in all the examined patients during a median follow-up of 4.4 years. Many patients had heredity for autoimmune disorders (47%) and/or tested positive for autoantibodies (40%) or human leukocyte antigen DQ2/DQ8 (53%). No associated autoimmune comorbidities were observed. The serum levels of calprotectin and amyloid A were increased in 10/15 (67%) and 5/15 (33%) of the patients, respectively, whereas plasma C-reactive protein levels were normal in all, but 1 patient.
Discussion:
The results indicate that childhood-onset CG is rare and has a chronic disease course. Although signs of autoimmune predisposition are frequent, early development of autoimmune comorbidities seems seldom. Serum calprotectin and amyloid A represent novel candidate biomarkers of inflammatory activity in CG (see Visual Abstract, Supplementary Digital Content 4, http://links.lww.com/CTG/A349).
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