Abstract
IgG4-related disease (IgG4-RD) is a rare but increasingly recognised condition that can involve multiple organs, including the kidneys which often presents as tubulointerstitial nephritis. Treatment with glucocorticoids is the first line of therapy, but other options may be needed in refractory cases. This case report explores a 68-year-old female, diagnosed with the patient initially responded to glucocorticoids but had a relapse, leading to progressive renal insufficiency and ultimately death. Our case is a rare case observing the progression to end-stage kidney disease from IgG4-RD, and the first case of which the patient had died in a short period.