Clinical Rheumatology

, Volume 38, Issue 1, pp 97–106 | Cite as

Prevalence of overlap of antineutrophil cytoplasmic antibody associated vasculitis with systemic autoimmune diseases: an unrecognized example of poliautoimmunity

  • Eduardo Martín-Nares
  • Diego Zuñiga-Tamayo
  • Andrea Hinojosa-AzaolaEmail author
Original Article


We aimed to estimate the frequency of overlap of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with systemic autoimmune diseases. Retrospective single-center study to identify patients with AAV diagnosis and concomitant autoimmune systemic diseases, simultaneously, before or after the diagnosis of AAV. Sociodemographic characteristics, such as comorbidities; follow-up time; type of AAV; disease duration; relapses; treatment and response; clinical, serological, and histological characteristics; disease activity and damage; prognosis; dialysis requirements, and death were assessed. Twenty-eight of two hundred and forty-seven patients (11.3%) with AAV had a concomitant diagnosis of autoimmune disease. The predominant AAV type was renal-limited vasculitis (39%), followed by granulomatosis with polyangiitis (29%), mycroscopic polyangiitis (25%), and eosinophilic granulomatosis with polyangiitis (7%). Mean age at AAV diagnosis was 50 ± 17 years and 24/28 were ANCA positive. The main clinical manifestations were renal (79%), otorhinolaryngologic (43%), and pulmonary and peripheral neuropathy (32%). Sixteen patients (57%) experienced partial or total remission at a median follow-up of 34 months, and four patients (14%) died. The most frequent autoimmune disease overlapped was rheumatoid arthritis (39%), followed by Sjögren’s syndrome and systemic sclerosis (14%), mixed connective tissue disease (11%), systemic lupus erythematosus and juvenile idiopathic arthritis (7%), and ankylosing spondylitis and IgG4-related disease (4%). In nine patients (32%), both diagnoses were simultaneous; in the rest, median time elapsed between the autoimmune disease and AAV diagnosis was 173 months. The prevalence of overlap AAV with other autoimmune diseases was low. The most common AAV phenotype was renal-limited vasculitis, and the most frequent overlap disease was rheumatoid arthritis.


ANCA Autoimmune diseases Overlap syndromes Vasculitis 



No acknowledgements to report.

Authors’ contributions

EMN and AHA designed the study; EMN and DZT participated in acquisition of data; AHA analyzed and interpreted data; EMN and AHA drafted the manuscript; EMN, DZT, and AHA revised the manuscript.

Compliance with ethical standards

Research was conducted in compliance with the Helsinki Declaration. Informed consent was not obtained due to the retrospective nature of the study and no need for approval by the local ethical committee was needed.



Ethical standards statement

The manuscript does not contain clinical studies or patient data.

Supplementary material

10067_2018_4212_MOESM1_ESM.pdf (152 kb)
ESM 1 (PDF 151 kb)


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Copyright information

© International League of Associations for Rheumatology (ILAR) 2018

Authors and Affiliations

  • Eduardo Martín-Nares
    • 1
  • Diego Zuñiga-Tamayo
    • 2
  • Andrea Hinojosa-Azaola
    • 1
    Email author
  1. 1.Department of Immunology and RheumatologyInstituto Nacional de Ciencias Médicas y Nutrición Salvador ZubiránMexico CityMexico
  2. 2.Department of PathologyInstituto Nacional de Ciencias Médicas y Nutrición Salvador ZubiránMexico CityMexico

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