Mayo Clinic Proceedings

Individuals with PiZZ have an increased risk for development of gastrointestinal diseases (Crohn disease, ulcerative colitis, noninfectious gastroenteritis, and diverticular disease encompassing symptomatic diverticulosis and its complications), as compared with the general population.

Findings indicate that broader PCS definitions may mask clinically relevant heterogeneity and support the need for differentiated, phenotype-oriented definitions that reflect clinical presentations of PCS, including differential responses to vaccination. Such refined clinical definitions may facilitate exploration of whether these phenotypes reflect distinct underlying pathophysiological mechanis…