#3420 clinical characteristics and primary outcomes of patients with anca-associated vasculitis and central diabetes insipidus

Abstract Background and Aims Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by systemic small-vessel vasculitis and may rarely present as central diabetes insipidus (CDI). AAV-associated CDI should be recognized to avoid unnecessary biopsies of the pituitary gland and minimize the risk of irreversible loss of pituitary function. In this study, we aimed to determine the clinical characteristics and prognosis of patients with AAV-associated CDI. Method This was a nested case-control study where AAV patients with CDI at the Peking Union Medical College Hospital were followed from January 2012 to April 2022. Case-control matching with AAV patients without CDI was performed (1:2), and participants were matched by age, sex, and AAV classification. We collected clinical data every 3–6 months and conducted a literature review using PubMed to identify relevant articles published from 1983–2022. Results Among 1203 hospitalized AAV patients, 16 patients with CDI were included (1.3%). The average age was 49 years, and men accounted for 56.3%. Granulomatosis with polyangiitis (GPA) accounted for 87.5% of patients. AAV patients with CDI had more ear, nose, and throat (ENT) (81.3%) involvement and less renal impairment than those in the control group (P<0.05). After a mean follow-up of four years, 50% of patients were in remission, 37.5% relapsed, and 12.5% died. Our literature review suggested that patients in Asian countries tend to be older men and have higher MPO-ANCA positivity than those in European countries. Furthermore, PR3-ANCA positivity may predict disease recurrence. Conclusion AAV patients with CDI had more ENT involvement and a higher eGFR. MPO-ANCA positivity is more commonly observed in Asian countries than in European countries, and PR3-ANCA positivity may predict recurrence.