Abstract: PUB305
Clinicopathological Features of IgG4-Related Kidney Disease: Case Series Study
Session Information
Category: Pathology and Lab Medicine
- 1602 Pathology and Lab Medicine: Clinical
Authors
- Zhang, Ping, Sichuan Academy of Sciences & Sichuan Provincial People''s Hospital, School of medicine, University of Electronic Science and Technology of China, Chengdu, China
- Yin, Qing, Sichuan Academy of Sciences & Sichuan Provincial People''s Hospital, School of medicine, University of Electronic Science and Technology of China, Chengdu, China
- Zou, Yurong, Sichuan Academy of Sciences & Sichuan Provincial People''s Hospital, School of medicine, University of Electronic Science and Technology of China, Chengdu, China
- Wang, Wei, Sichuan Academy of Sciences & Sichuan Provincial People''s Hospital, School of medicine, University of Electronic Science and Technology of China, Chengdu, China
- Li, Guisen, Sichuan Provincial People's Hospital, Chengdu, SiChuan, China
- Wang, Junru, Sichuan Academy of Sciences & Sichuan Provincial People''s Hospital, School of medicine, University of Electronic Science and Technology of China, Chengdu, China
Background
To investigate the clinicopathological features of IgG4-related kidney disease (IgG4-RKD) in Chinese patients.
Methods
We retrospectively analyzed the clinicopathological features of patients with IgG4-RKD and reviewed the relevant literature.
Results
There were 7 patients with IgG4-RKD, the male-to-female ratio was 4:3, and the mean age was 57±12 years. The most common clinical manifestation of these patients was acute or chronic renal insufficiency, while only one presented as nephrotic syndrome.Six patients had evidence of extrarenal involvement.Laboratory examination indicated that the serum IgG and IgG4 levels were elevated in all patients and IgE levels were elevated in four patients.Two patients were clinically diagnoses as ANCA associated vasculitis(AAV). All of the renal biopsies showed diffuse or focal dense lymphoplasmacytic cell infiltrates with scattered eosinophils within the renal interstitium, accompanied by interstitial fibrosis with a“storiform” pattern and tubular atrophy and dropout in fibrotic regions. On immunostaining, IgG4/IgG+ plasma cells ratio was ＞40% in all of the patients and the number of IgG4+ plasma cells was ≥10/HPF in five patients. Apart from tubulointerstitial nephritis(TIN), three patients had glomerular lesions, one of them had membranous nephropathy, the other two revealed necrotizing vasculitis with formation of crescents in the glomerular capsule, that was consistent with AAV. All the patients were treated with prednisolone, and combination of cyclophosphamide was administered in the two patients with AAV. The median follow-up time was 2 years, 5 of 7 patients revealed complete remission or partial remission, and all patients were alive at the most recent follow-up.One patient with AAV was on dialysis.
Conclusion
The combined application of morphology, immunohistochemistry, and clinical data may facilitate the diagnosis of IgG4-RKD. In the progressive stage of IgG4-RKD, the ratio of IgG4+ to IgG+ cell should be considered a useful metric for diagnosis. In rare cases,we should consider possible coexistence or overlap of AAV and IgG4-RKD.
Funding
- Government Support - Non-U.S.