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Antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) consists of a group of systemic autoimmune diseases. The roles of serum anti-nuclear antibodies (ANA) and anti-double-stranded DNA (anti-dsDNA) antibodies in AAV patients remain unknown. This study investigated the prevalence of serum ANAs and anti-dsDNA antibodies in AAV patients and characterized the clinical and pathological features of these patients. A total of 218 AAV patients were enrolled. Clinical and pathological data of patients were analyzed retrospectively. Of the 218 AAV patients, 109 (50.0%) were positive for ANA, 45 (20.6%) were positive for anti-dsDNA, and 43 (19.7%) were positive for both. The AAV patients with ANA had severer kidney damage and more chronic renal histopathological changes compared to those who were negative for ANA. Specifically, patients positive for ANA had more hypertension, higher levels of urea nitrogen and serum creatinine, lower estimated glomerular filtration rate (eGFR), more end-stage renal disease (ESRD), severer proteinuria, glomerular sclerosis, tubular interstitial fibrosis and tubular atrophy, and were more likely to receive renal biopsies compared to ANA negative patients. The study found ANA and anti-dsDNA in AVV patients were not rare, ANA-positive AAV patients had severer kidney damage and more chronic renal histopathological changes compared to ANA-negative AAV patients. Renal biopsy is strongly recommended for differential diagnosis in such cases.

Clinical and pathological characteristics of ANA/anti-dsDNA positive patients with antineutrophil cytoplasmic autoantibody-associated vasculitis