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Antineutrophil cytoplasmic antibody and/or antiglomerular basement membrane antibody associated crescentic glomerulonephritis in combination with IgG4-related tubulointerstitial nephritis
G.Q. Wang1, Y.P. Chen2, H. Cheng3, X.Y. Xu4, L.J. Sun5, H.R. Dong6
- Division of Nephrology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.
- Division of Nephrology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.
- Division of Nephrology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China. drhongcheng@163.com
- Division of Nephrology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.
- Division of Nephrology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.
- Division of Nephrology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.
CER11295
2019 Vol.37, N°2
PI 0279, PF 0285
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PMID: 30183608 [PubMed]
Received: 01/04/2018
Accepted : 04/06/2018
In Press: 29/08/2018
Published: 19/03/2019
Abstract
OBJECTIVES:
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder and is characterised by elevated serum IgG4 concentrations and dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells. IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common manifestation of IgG4-related kidney disease (IgG4-RKD). We report four cases of kidney injury with concurrent IgG4-TIN and crescentic glomerulonephritis confirmed by renal pathology.
METHODS:
The medical charts of four patients were reviewed to collect clinical and laboratory data at the time of diagnosis, treatment and outcomes after 6-36 months. Two of them are cases of IgG4-TIN with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), and the other two cases are rare IgG4-TIN with antiglomerular basement membrane (anti-GBM) glomerulonephritis coexistent with ANCA-positive serum.
RESULTS:
Compared with IgG4-TIN, IgG4-TIN combined with AAV or anti-GBM glomerulonephritis is less associated with other organ injuries, and the clinical manifestations, treatment effects and prognosis were consistent with that of crescentic glomerulonephritis.
CONCLUSIONS:
IgG4-TIN concurrent with anti-GBM glomerulonephritis and positivity in serum has more severe clinical features and a worse renal prognosis than IgG4-TIN coexistent with AVV.