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Background: It has been reported that antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (ANCA-GN) and immunoglobulin G4 (IgG4)-related kidney disease (IgG4-RKD) could overlap. The current study aimed to investigate clinical and histopathological characteristics of patients with ANCA-GN and IgG4-RKD overlap syndrome.

Methods: Eleven patients with combined ANCA-GN and IgG4-RKD were included in this study. Their clinical and pathological data were analyzed.

Results: Of the 11 patients, 8 were male and 3 were female. Seven, three and one patient were classified as microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiits (EGPA), respectively. Hematuria was absent or slight in 8 (72.7%) cases. Renal histology of these patients revealed concurrent ANCA-GN and IgG4-RKD. Regarding the histological classification of ANCA-GN, five (45.5%) and 6 (54.5%) patients were classified as focal and crescentic ANCA-GN, respectively. MPO-ANCA could be detected in 10/11 (91.0%) patients. IgG subclasses of MPO-ANCA were tested, and all were positive for IgG4-MPO-ANCA. In patients with combined ANCA-GN and IgG4- RKD, the percentage of positive IgG1-MPO-ANCA was significantly lower than the control group of 20 AAV patients without IgG4-RKD (P = 0.002), and the percentage of positive IgG4-MPO-ANCA was marginally higher than the control group (P = 0.14).

Conclusion: ANCA-GN and IgG4-RKD overlap syndrome concerned mainly MPO-ANCA positive patients, with a striking male predominance. The IgG subclass analysis of MPO-ANCA showed lower percentage of IgG1 subclass. The association between ANCA-GN and IgG4-RKD is possible and represents a special entity.

Disclosures: This study was supported by the grant from National Key Research and Development Program (No. 2016YFC0906102), three grants from the National Natural Science Fund (No. 81870477, No. 81425008 and 81621092), the grant by the University of Michigan Health System and Peking University Health Sciences Center Joint Institute for Translational and Clinical Research, and a grant from the Scientific Research Seed Fund of Peking University First Hospital (No. 2018SF004).

Abstract 101 Table 1:
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IgG subclasses distribution of MPO-ANCA

No. (%)
IgG subclass of MPO- ANCAPatients with combined ANCA-GN and IgG4-RKD (n = 10)ANCA-GN patients (n = 20)P for χ2 test
IgG14 (40%)19 (95%)0.002
IgG21 (10%)2 (10%)1
IgG33 (30%)11 (55%)0.26
IgG410 (100%)15 (75%)0.14
No. (%)
IgG subclass of MPO- ANCAPatients with combined ANCA-GN and IgG4-RKD (n = 10)ANCA-GN patients (n = 20)P for χ2 test
IgG14 (40%)19 (95%)0.002
IgG21 (10%)2 (10%)1
IgG33 (30%)11 (55%)0.26
IgG410 (100%)15 (75%)0.14
Abstract 101 Table 1:
Open in new tab

IgG subclasses distribution of MPO-ANCA

No. (%)
IgG subclass of MPO- ANCAPatients with combined ANCA-GN and IgG4-RKD (n = 10)ANCA-GN patients (n = 20)P for χ2 test
IgG14 (40%)19 (95%)0.002
IgG21 (10%)2 (10%)1
IgG33 (30%)11 (55%)0.26
IgG410 (100%)15 (75%)0.14
No. (%)
IgG subclass of MPO- ANCAPatients with combined ANCA-GN and IgG4-RKD (n = 10)ANCA-GN patients (n = 20)P for χ2 test
IgG14 (40%)19 (95%)0.002
IgG21 (10%)2 (10%)1
IgG33 (30%)11 (55%)0.26
IgG410 (100%)15 (75%)0.14

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