Setting up the First Registry for Inflammatory Bowel Disease, Suspected to Primary Immunodeficiency Diseases in Iran

Background: inflammatory bowel disease (IBD) has been considered a hyperinflammatory state due to disturbed interactions between the immune system and the commensal bacterial flora of the gut and suggesting IBD asa primary immunodeficiencies (PID). Therefore a registry launched about IBD, suspected to PID (IBDSPID) for the first time in Iran to raise awareness about this disease, to identify its risk factors, to early diagnosis and treatment. Materials and Methods: Among of 365 patients, 39 have inclusion criteria that were as below:(1) IBD diagnosis before age 5 years (2) Resistance to conventional therapy of IBD (3) Severe IBD (4) Signs of SPID (Recurrent sinus or ear infections or pneumonias within a 1 year period; failure to thrive; poor response to prolonged use of antibiotics; persistent thrush or skin abscesses; or a family history of PID).The achieved data of patients was computerized using the MySQL Database. Results: The mean age was 32.92± 15.90 years old. 51.3% of patients were males. The Ulcerative colitis was the most common type of diagnosed IBDSPID. Among patients, 23.1, 29.4, 50.0% were mild, moderate, severe cases of IBDSPID respectively. Age at onset of IBD in the majority of patients was after 17 years old. Among patients, 12.9% had resistance to drugs. The percent of patients with history of autoimmune, allergy, PID and SPID was 33.3, 33.3, 89.7, 12.9; respectively. consanguinity, family history of IBD, autoimmune disease and allergy, was 47.4%, 15.4%, 25.7% and 28.2%; respectively, also the percent of family history of PID and malignancy was 2.6 and 20.5; respectively. Conclusions: This successful registry provides useful data on IBD especially with SPID and therefor can help audit and research purposes Mainconclusions: This registry increases the awareness about this disease for determination the prevalence, early diagnosis and effective treatment. Keyword: Crohn’s disease, inflammatory bowel diseases, registry, primary immunodeficiencydisease