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Abstract: SA-PO421

The Antineutrophil Cytoplasmic Antibody–Associated Vasculitides Concomitant with IgG4-Related Disease: A Case Series

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials


  • Han, Fei, First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, ZHEJIANG, China

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) and IgG4-related disease (IgG4-RD) have similarities in clinical characteristics and histopathological features. Concomitance of these two diseases have been reported previously.


We compared the characteristics of AAV patients, IgG4-RD patients and concomitant AAV and IgG4-RD patients in our center. One hundred and sixty-nine AAV patients, 58 IgG4-related disease patients and 8 concomitant patients were included. The serum IgG subclasses of MPO-ANCA were measured.


The patients in concomitant group had both elevated serum IgG4 level and positive ANCA. They had lower levels of hemoglobin, serum albumin, glomerular filtration rate and higher levels of platelet, serum creatinine, erythrocyte sedimentation rate and C-reactive protein compared with IgG4-RD group. Their involved organs were mainly kidney (100%), lung (62.5%) and lacrimal glands (62.5%). They had higher serum globulin level and even lower serum albumin level than patients with AAV. Six renal biopsies were performed in concomitant group. They achieved remission with improved renal function, one patient was on maintained dialysis and one patient died of acute gastric perforation. The IgG4 subclass of MPO-ANCA was higher in concomitant group than in AAV group (OD450 value 12.42±6.63 vs 3.03±3.67, P=0.017), whereas the other three subclasses (IgG1, IgG2 and IgG3) of MPO-ANCA were parallel between these groups.


We showed a new overlap syndrome of AAV and IgG4-RD, and the IgG4 subclass of ANCA may be a pathogenic factor in this concomitant disease. Besides, we suggested the concomitant patients be treated as AAV, as they were more likely to show the clinical features of AAV.