Clinical characteristics and primary outcomes of patients with ANCA-associated vasculitis and central diabetes insipidus

Front Endocrinol (Lausanne). 2023 May 12:14:1173903. doi: 10.3389/fendo.2023.1173903. eCollection 2023.

Abstract

Introduction: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by systemic small-vessel vasculitis and may rarely present as central diabetes insipidus (CDI). In this study, we aimed to determine the clinical characteristics and prognosis of patients with AAV-associated CDI.

Methods: 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 (1:5), 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 from AAV, 37.5% relapsed, and 12.5% died. Our literature review suggested that patients in Asian countries tend to be older men and have higher myeloperoxidase (MPO-ANCA) positivity than those in Western countries. Furthermore, proteinase 3 (PR3-ANCA) positivity may predict disease recurrence.

Discussion: AAV patients with CDI had more ENT involvement and a higher eGFR. MPO-ANCA positivity is more commonly observed in Asian countries than Western countries, and PR3-ANCA positivity may predict recurrence.

Keywords: MPO-ANCA; PR3-ANCA; antineutrophil cytoplasmic autoantibody-associated vasculitis; diabetes insipidus; recurrence rate.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / complications
  • Antibodies, Antineutrophil Cytoplasmic
  • Case-Control Studies
  • Diabetes Insipidus, Neurogenic*
  • Diabetes Mellitus*
  • Humans
  • Male
  • Middle Aged
  • Myeloblastin

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Myeloblastin

Grants and funding

This work was partially supported by grants from the National Key R&D Program of China (2022ZD0116003 to LC), National Natural Scientific Foundation of China (82170709, 81970607 to LC), CAMS Innovation Fund for Medical Sciences (CIFMS 2020-I2M-C&T-A-001, CIFMS 2021-I2M-1-003 to LC), Capital’s Funds for Health Improvement and Research (CFH 2020-2-4018 to LC); Beijing Natural Science Foundation (L202035 to LC); National High Level Hospital Clinical Research Funding (2022-PUMCH-B-019 to LC); the Capital Exemplary Research Wards Project (BCRW202001 to LC); and Fundamental Research Funds for the Central Universities (3332022109 to XC). The funders had no role in study design, data collection and analysis, decision to publish, or manuscript preparation.