Background/Purpose: This study aimed to characterize the presenting features and outcomes of patients with small- or medium-vessel vasculitis and gastrointestinal perforation.

Methods: Using a retrospective cohort design, we identified cases with verified granulomatosis with polyangiitis (GPA), eosinophilic GPA (EGPA), microscopic polyangiitis (MPA), or polyarteritis nodosa (PAN) by ACR criteria along with confirmed gastrointestinal perforation at our institution between 1998 and 2017. The standardized mortality ratio (SMR) was estimated using persons of the same age, sex, and calendar year derived from United States white population life tables.

Results: Over the twenty year time period, 20 total patients with small or medium-vessel vasculitis experienced bowel perforation. Three had EGPA, 11 GPA, three MPA, and three PAN. Four of the twenty cases experienced vasculitis-induced perforation. Cases with perforations due to vasculitic involvement had more small bowel involvement, longer duration of abdominal pain prior to perforation (41 days vs 0 days, p = 0.005), and a higher proportion of active tobacco use (75% vs 7%, p = 0.01) compared to the cases with non-vasculitis perforation.  A majority (88%) of the non-vasculitic-induced perforations were associated with glucocorticoid use. The median cumulative glucocorticoid dose at perforation in patients with additional, non-vasculitic risk factors for perforation was 4,320 mg prednisone and was 22,170 mg for those without such risk factors. Mortality rates for the whole cohort were higher than the general population (SMR 2.19, 95% confidence interval 1.05 to 4.02, Figure 1). The cases with vasculitis-induced perforation tended to have increased number of surgeries and length of stay; however, those differences failed to reach statistical significance.

Conclusion: In patients with known history of vasculitis, small bowel location and longer abdominal pain duration may help distinguish vasculitis-induced bowel perforation from other etiologies. Overall mortality in patients with vasculitis and bowel perforation is increased, highlighting the importance of a high index of suspicion and prompt management.