Clinicopathological analysis of immunoglobulin G4-related diseases with interstitial nephritis
10.3760/cma.j.issn.1007-7480.2019.07.004
- VernacularTitle:以间质性肾炎为主要表现的免疫球蛋白G4相关性疾病临床病理分析
- Author:
Yaping ZHANG
1
;
Dan NIU
;
Lifang GAO
;
Siyu CHEN
;
Ying WANG
;
Rong WEI
;
Xiaoqin ZHANG
;
Hongyan WEN
;
Chen WANG
Author Information
1. 山西医科大学第二医院病理科
- Keywords:
IgG4-related diseases;
Pathology;
clinical;
Nephritis;
interstitial
- From:
Chinese Journal of Rheumatology
2019;23(7):449-453,封3
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinicopathological features and prognosis of immunoglobulin G (IgG)4-related diseases with interstitial nephritis. Methods Forty cases of IgG4-related diseases diagnosed by pathology in our hospital from 2014 to 2018 were collected and their clinicopathological features were analyzed. Four patients with IgG4-related disease with interstitial nephritis were analyzed, including clinical laboratory tests and histopathological features, and immunohistochemical analysis of the type and proportion of renal interstitial infiltrating cells. At the same time, the treatment and prognosis of the patients were analyzed. Results Among 40 cases of IgG4-related diseases, 11 cases had parotid submandibular gland involvement (accounted for 28％), labial gland involvement (7 cases, 18％) and lymph node enlargement (6 cases, 15％). Patients with kidney involvement (4 cases, 10％) all presented with IgG4-related tubulo-interstitial nephritis (IgG4-TIN). All of them were elderly males, and 2 had glomerulopathy. One of them had anti-neutrophil cytoplasmic antibodies (ANCA)-related vasculitis renal damage. The number of CD4-positive cells in renal interstitium was more than CD8-positive cells. Another case complicated with IgA nephropathy. Renal dysfunction occurred in all 4 cases, and serum IgG4 level ranged from 4.65 g/L to 23.8 g/L. All 4 patients received glucocorticosteroid and symptomatic treatment, and the prognosis was good. Conclusion IgG4-related diseases may involve mul-tiple organs, renal dysfunction may occur when the kidney is involved. Interstitial nephritis is the major clinical manifestation. Glomerular lesions may accelerate the progress of IgG4-TIN. Corticosteroid therapy is effective.
