The clinical course of patients with IgG4-related kidney disease

Abstract

Long-term follow-up for IgG4-related kidney disease, including relapse information, is sparse. To gather data on this we retrospectively examined the clinical course of 43 patients with IgG4-related kidney disease, in which most patients were treated with, and maintained on, corticosteroids. One month after the start of treatment, most of the abnormal serology and radiology parameters had improved. In 34 of the steroid-treated patients whose follow-up period was more than 12 months (median 34 months), excluding one hemodialysis patient, the estimated glomerular filtration rate (eGFR) before treatment was over 60 ml/min in 14 patients (group A) and under 60 ml/min in 20 patients (group B). In group A, there was no difference between the eGFR before therapy and at the last review. In group B, the mean eGFR before treatment (34.1 ml/min) was significantly improved after 1 month (45.0 ml/min), and renal function was maintained at a similar level through last follow-up. Among 24 evaluated patients at the last review, however, renal atrophy had developed in 2 of 9 in group A and in 9 of 15 in group B. Relapse of IgG4-related lesions occurred in 8 of 40 treated patients. Thus, the response of IgG4-related kidney disease to corticosteroids is rapid, not total, and the recovery of renal function persists for a relatively long time under low-dose maintenance. A large-scale prospective study to formulate more useful treatment strategies is necessary.

Hemoptisis recurrente asociada a falla renal y hematuria: otra de las mil caras de la enfermedad relacionada a IgG4

“…Los primeros parecen ser más sensibles y pueden ser más útiles para la detección en fases tempranas de la enfermedad o casos atípi-cos. Los segundos son más estrictos y específicos 10 . La NTI-IgG4 requiere de tratamiento inmediato para suprimir la inflamación, evitar la fibrosis y preservar la función renal 3,11 . Los glucocorticoides son el tratamiento de primera línea.…”

“…Reducen las "megalias", optimizan la función renal y disminuyen los niveles de IgG4. Un rasgo característico es la rápida respuesta a la terapia esteroidal, sin embargo, en los pacientes con NTI-IgG4 asociada a disfunción renal avanzada, la respuesta parece ser parcial 4,11 . El esquema más utilizado es el de la Sociedad Japonesa de Nefrología con prednisona a dosis de inducción de 0,6 mg/kg/día por 2-4 semanas y posterior disminución progresiva hasta llegar a dosis de mantención de 5-10 mg día [3][4][5] .…”

A case of IgG4-related kidney disease complicated by eosinophilic lung disease

“…However, many patients do not undergo contrast-enhanced CT, and contrast-enhanced CT is considered necessary as long as the renal function tolerates the modality. FDG PET-CT and Ga [6,9] scintigraphy have been reported to be effective for evaluation of the sites and activities of lesions [11], but positive accumulation in the kidney on Ga [6,9] scintigraphy was in only 11 % of patients according to Saeki et al [12]. and further evaluation is considered necessary as an imaging modality for kidney lesions.…”

IgG4-related inflammatory pseudotumor of the kidney mimicking renal cell carcinoma: A case report

“…In the few previous reports of renal involvement with IgG4-RD, the renal lesions have been observed to decrease in size following corticosteroid therapy, with some disappearing and others recurring subsequent to cessation of treatment (10)(11)(12). Furthermore, corticosteroids have been reported to improve renal function and decease serum IgG4 levels after 1 month of therapy; however, renal function may not be totally recovered (10).…”