Here we
report the
diagnosis and
treatment of a rare
IgG4-related kidney disease with
nephrotic syndrome as the first manifestation. A 62-year-old
male patient, presented with
edema in both
lower limbs and foam
urine, had a
history of "
lung malignant
tumor with
brain and
lymph node metastasis". The increase of
IgG4 and decrease of
glomerular filtration rate were detected at admission, and the pathological consideration of renal
biopsy was
membranous nephropathy with
IgG4-related
tubulointerstitial nephritis. After the combination of low-
dose glucocorticoids therapy and
rituximab treatment, the
patient showed good
prognosis in a 9 month follow-up.