Clinicopathological features of IgG4-related kidney diseaseâ©.
Clin Nephrol
; 94(3): 135-141, 2020 Sep.
Article
em En
| MEDLINE
| ID: mdl-32691729
ABSTRACT
AIMS:
To explore the clinicopathological features of IgG4-related kidney disease (IgG4-RKD) in Chinese patients. MATERIALS ANDMETHODS:
We retrospectively analyzed the clinicopathological features of 7 patients with IgG4-RKD and reviewed relevant literature.RESULTS:
There were 7 patients (4 males and 3 females) aged 41 - 78 years (median age 57.1 years), with a mean affliction of 2.6 organs per patient. All 7 patients had elevated serum IgG4 levels, and most of them had acute or chronic renal insufficiency. Two patients had positive myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA), and another 2 patients had positive serum antinuclear antibodies (ANA). All 7 patients had tubulointerstitial nephritis (TIN) with IgG4+ plasma cell infiltration, 3 patients also had glomerular lesions, 2 patients had ANCA-associated glomerulonephritis (ANCA-GN), and the remaining patient had membranous nephropathy (MN). All these patients were treated with glucocorticoids, and 3 of them were given cyclophosphamide simultaneously. Six patients achieved remission and had improved renal function, while 1 patient was on maintenance dialysis.CONCLUSION:
IgG4-RKD should be diagnosed by combining the clinical data and renal pathological changes, and it should be differentiated from a variety of diseases. Although ANCA-associated vasculitis (AAV) and IgG4-related disease are distinguishable in most cases, coexistence of ANCA-GN and IgG4-RKD should be considered in some special cases.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Doença Relacionada a Imunoglobulina G4
Tipo de estudo:
Diagnostic_studies
/
Observational_studies
Limite:
Adult
/
Aged
/
Female
/
Humans
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Male
/
Middle aged
Idioma:
En
Revista:
Clin Nephrol
Ano de publicação:
2020
Tipo de documento:
Article