Clinical patterns in CVID-associated enteropathy

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

DRKS

Registry ID

DRKS00037932

Enrollment

Registered

2025-12-23

Start date

2026-01-20

Completion date

Unknown

Last updated

2026-01-12

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

D83.9

Interventions

Group 1: This study is retrospective in design and therefore does not affect the ongoing clinical course of the patients. All analyzed data were collected retrospectively and are part of the patients’

Eligibility

Sex/Gender

All

Age

18 Years to No maximum

Inclusion criteria

Inclusion criteria: - Older than 18 year old - Confirmed diagnosis of CVID or selective IGA deficiency

Exclusion criteria

Exclusion criteria: Younger than 18 year old

Design outcomes

Primary

Measure	Time frame
Primary Objective: To establish a subclassification of CVID-associated enteropathy in which objective measurements of the duodenal mucosa, small intestinal absorption, and the mucosa-associated immune system (including the number of intraepithelial lymphocytes [IELs per 100 enterocytes], the absorption score according to Branchi et al. (2022), and immunophenotyping of intestinal lymphocytes) are correlated with the clinical severity of enteropathy. As the clinical endpoint, we will assess the extent of gut-specific clinical symptoms that reflect the patient's disease burden and are intended to be incorporated into a future Clinical Composite Outcome (CCO) score. These symptoms represent the endpoint of the analysis (see below) and include: - Stool frequency - BMI progression - Abdominal pain - Infection rate The cellular and clinical-chemical measurements of small intestinal structure and function that will be used for patient classification include: - Villus-to-crypt ratio of the duodenal mucosa - Number of duodenal IELs per 100 enterocytes - Immunophenotyping of intestinal lymphocytes - Malabsorption score - Genetic mutations affecting B-cell maturation - FACS analysis of B-cell maturation from peripheral blood Primary Endpoint: Correlation analysis of the above-mentioned clinical symptoms, potentially as a composite score (CCO), with the results of the cellular and clinical-chemical measurements listed above.	—

Measure

Time frame

Primary Objective: To establish a subclassification of CVID-associated enteropathy in which objective measurements of the duodenal mucosa, small intestinal absorption, and the mucosa-associated immune system (including the number of intraepithelial lymphocytes [IELs per 100 enterocytes], the absorption score according to Branchi et al. (2022), and immunophenotyping of intestinal lymphocytes) are correlated with the clinical severity of enteropathy. As the clinical endpoint, we will assess the extent of gut-specific clinical symptoms that reflect the patient's disease burden and are intended to be incorporated into a future Clinical Composite Outcome (CCO) score. These symptoms represent the endpoint of the analysis (see below) and include: - Stool frequency - BMI progression - Abdominal pain - Infection rate The cellular and clinical-chemical measurements of small intestinal structure and function that will be used for patient classification include: - Villus-to-crypt ratio of the duodenal mucosa - Number of duodenal IELs per 100 enterocytes - Immunophenotyping of intestinal lymphocytes - Malabsorption score - Genetic mutations affecting B-cell maturation - FACS analysis of B-cell maturation from peripheral blood Primary Endpoint: Correlation analysis of the above-mentioned clinical symptoms, potentially as a composite score (CCO), with the results of the cellular and clinical-chemical measurements listed above.

—

Secondary

Measure	Time frame
Secondary Hypothesis: Retrospective analysis of the prognostic value of the newly proposed subclassification with regard to treatment response (particularly to budesonide). This involves two clinical assessments—one prior to treatment initiation and a second after completion of therapy. Secondary Endpoint: Evaluation of the aforementioned gut-specific clinical symptoms in response to therapy, i.e., comparison of symptom severity before and after initiation of treatment.	—

Measure

Time frame

Secondary Hypothesis: Retrospective analysis of the prognostic value of the newly proposed subclassification with regard to treatment response (particularly to budesonide). This involves two clinical assessments—one prior to treatment initiation and a second after completion of therapy. Secondary Endpoint: Evaluation of the aforementioned gut-specific clinical symptoms in response to therapy, i.e., comparison of symptom severity before and after initiation of treatment.

—

Countries

Germany, Italy, Netherlands

Contacts

Public ContactMichael Schumann

Medizinische Klinik für Gastroenterologie, Infektiologie und Rheumatologie | Charité Campus Benjamin Franklin

michael.schumann@charite.de+49030 84450

Outcome results

None listed

Source: DRKS (via WHO ICTRP) · Data processed: Feb 4, 2026