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BACKGROUND: Granulomatosis with polyangiitis (GPA) is a rare form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis that can involve multiple organ systems, including the gastrointestinal (GI) tract. Although GI manifestations are relatively uncommon, they may be associated with serious complications and adverse outcomes. This study aimed to assess the frequency and types of GI involvement in patients with GPA and to examine their relationship with disease severity, prognosis, and treatment response. METHODS: In this retrospective cohort study, clinical records of 220 patients with a confirmed diagnosis of GPA who were referred to Amir Alam Hospital between 2013 and 2021 were reviewed. Data on demographic characteristics, GI symptoms, Birmingham Vasculitis Activity Score (BVAS), therapeutic response, relapse rates, and mortality were collected and analyzed. RESULTS: GI involvement was observed in 18 patients (8.2%). The most common manifestations included hepatitis, mesenteric ischemia, diarrhea, pancreatitis, and elevated liver enzymes. Patients with GI involvement had significantly higher BVAS scores (mean 21 vs. 15.8, p = 0.004), a markedly increased mortality risk (hazard ratio = 3.24, p < 0.001), and a shorter time to first relapse (mean 5.2 vs. 10.3 months, p = 0.041) compared to those without GI symptoms. CONCLUSION: Gastrointestinal involvement in GPA is associated with more severe disease activity, diminished treatment response, and increased mortality. Early detection and appropriate management of GI manifestations may improve clinical outcomes. Further prospective studies are warranted to elucidate the underlying mechanisms and optimize treatment strategies for this high-risk subgroup.
Siri et al. (Fri,) studied this question.