Budesonide in Patients With Immune Mediated Enteropathies

This study aims to evaluate patients with small intestinal diseases caused by the immune
system (refractory celiac disease type 1, CVID enteropathy, autoimmune enteropathy, and
collagenous enteropathy) who have had improvement in symptoms and small intestine healing
with oral budesonide.

Patients who meet inclusion criteria and agree to enter the study will be included for 12
weeks or until they choose to discontinue or there are concerns for safety.

At the beginning of the study, patients will undergo questionnaires, blood draw, urine and
stool collections, physical exam, and an upper scope (esophagogastroduodenoscopy) with
biopsies from the small intestine. They will then be randomized to either continued therapy
on their current dose and formulation of budesonide (medication provided by the study) or to
withdrawal of the medication over 2 weeks and then no medication during the study. Daily
questionnaire on symptoms will be recorded.

Patients will be assessed in the clinical research unit monthly during the study with a
questionnaire, physical exam, blood draw, and urine/stool collections.

Finally, at the end of the trial (12 weeks) or at the time of withdrawal from the trial, the
patients will complete questionnaires, physical exam, blood collection, urine/stool
collection, and upper scope (esophagogastroduodenoscopy) with small bowel biopsies.

Inclusion Criteria

- Patients will be recruited from the Division of Gastroenterology and Hepatology and
the Mayo Clinic in Rochester, MN.

- Adult patients with immune mediated enteropathies who have had improvement in symptoms
and histology on oral budesonide therapy.

Exclusion Criteria

- Age <18 years

- Positive stool gluten testing in patients with refractory celiac disease

- Small bowel malignancy or history of small bowel malignancy

- Refractory celiac disease type 2

- Post-transplant lymphoproliferative disorder associated enteropathy

- No prior improvement in symptoms and histology with budesonide therapy

- Discontinuation of budesonide therapy prior to the trial

- Other concurrent systemic corticosteroids

- Other immune mediating medications, for example but not limited to azathioprine,
6-mercaptopurine, cyclosporine, methotrexate, anti-TNF monoclonal antibodies, alpha-4
beta-7 integrin inhibiting monoclonal antibody, interleukin 12/23 inhibiting
monoclonal antibody, JAK inhibitors.