PMID: 40414421 https://pubmed.ncbi.nlm.nih.gov/40414421/

Abstract

OBJECTIVE: Co-occurrence of Sickle Cell Disease (SCD) and Inflammatory Bowel Diseases (IBD) has been scarcely reported. Our aim was to explore the intersection between SCD and IBD, focusing on the impact of SCD on the natural course of IBD and drug safety.

METHODS: We conducted a multicenter retrospective case-control study including consecutive patients diagnosed with IBD and SCD. Each IBD patient with SCD was matched with up to 4 IBD patients without SCD. Matching criteria were IBD type, sex, date of birth, length of follow-up and year of diagnosis. The primary outcome was a complicated IBD course.

RESULTS: 125 IBD patients were studied, including 24 SCD. 23/24 SCD patients had colonic involvement. 33.3 % had concomitant primary sclerosing cholangitis (PSC) compared to 1 % of controls (p < 0.001). Survival without a complicated IBD course was estimated at 58.7 % (CI95[49.6-69.5]) at 5 years for non-SCD patients, as compared to 63.3 % (CI95[45.7-87.6]) for SCD patients SCD (p = 0.36). The survival without the need of advanced therapy was estimated at 66.1 % (CI95[57.3-76.2]) at 5 years for non-SCD patient, and at 78.2 % (CI95[63-97.2]) in SCD patients (p = 0.45) Regarding treatment safety, 26.3 % of patients with SCD and 13.5 % of controls experienced adverse events with biologics (p = 0.17). There was one reported vaso-occlusive crisis associated with thiopurines.

CONCLUSION: Patients with SCD and IBD displayed a distinctive phenotype with constant colonic involvement and high prevalence of PSC.