title: 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.
authors.affiliation: Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Niigata, Japan.
authors.forename: Takako
authors.initials: T
authors.lastname: Saeki
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Kawano
authors.forename: Ichiro
authors.initials: I
authors.lastname: Mizushima
authors.forename: Motohisa
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Yoko
authors.initials: Y
authors.lastname: Wada
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Nakashima
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Homma
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Tsubata
authors.forename: Hiroki
authors.initials: H
authors.lastname: Takahashi
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Ito
authors.forename: Hajime
authors.initials: H
authors.lastname: Yamazaki
authors.forename: Takao
authors.initials: T
authors.lastname: Saito
authors.forename: Ichiei
authors.initials: I
authors.lastname: Narita
mesh_terms: D000305:Adrenal Cortex Hormones / Q000009:adverse effects / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001284:Atrophy / Q000453:epidemiology; D019468:Disease Management; D005260:Female; D005500:Follow-Up Studies; D005919:Glomerular Filtration Rate / Q000502:physiology; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism*; D015994:Incidence; D007668:Kidney / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D007674:Kidney Diseases / Q000188:drug therapy* / Q000276:immunology* / Q000503:physiopathology; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G