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Abstract Number: 1235

Recovery of Renal Function after Corticosteroid Therapy for IgG4-Related Kidney Disease

Takako Saeki1, Mitsuhiro Kawano2, Ichiro Mizushima2, Motohisa Yamamoto3, Yoshifumi Ubara4, Hitoshi Nakashima5, Yoko Wada6, Tomoyuki Ito7, Hajime Yamazaki7, Ichiei Narita6 and Takao Saito5,8, 1Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan, 2Kanazawa University Hospital, Kanazawa, Japan, 3Sapporo Medical University School of Medicine, Sapporo, Japan, 4Toranomon Hospital, Tokyo, Japan, 5Div of Nephrol & Rheumatol, Dept of Int Med, Faculty of Medicine, Fukuoka University, Fukuoka, Japan, 6Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan, 7Nagaoka Red Cross Hospital, Nagaoka, Japan, 8General Medical Research Center, Faculty of Medicine, Fukuoka University, Fukuoka, Japan

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: corticosteroids, IgG4 Related Disease, Kidney, outcomes and treatment

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Session Information

Title: Miscellaneous Rheumatic and Inflammatory Diseases

Session Type: Abstract Submissions (ACR)

Background/Purpose: In our earlier study of IgG4-related kidney disease (IgG4-RKD), we found that renal dysfunction, which was mostly attributable to IgG4-related tubulointerstitial nephritis, was significantly improved at 1 month after the start of corticosteroid therapy, but reached a plateau thereafter, and renal atrophy developed in many patients (Saeki, et al. Kidney Int 2013). Little is known about the appropriate initial corticosteroid dose for induction therapy or the long-term renal outcome in IgG4-RKD with renal dysfunction.

Methods: This retrospective cohort analysis evaluated the recovery of renal function during the initial 1 month of corticosteroid therapy, and the long-term course of renal function after treatment, in 41 patients with confirmed IgG4-RKD in whom the eGFR before corticosteroid treatment had been less than 60 ml/min. The patients were collected from 16 collaborating institutions in Japan between 2004 and 2013, and divided into two groups (group L, initial prednisolone dose <0.6 mg/kg/day; group H, >0.6 mg/kg/day).

Results: Among the patients, 88% were male, and the mean age at the time of diagnosis of renal disease was 66.6±9.3 years. Renal pathology data were available for 38 of the 41 patients, and all of them had the tubulointerstitial features characteristic of IgG4-RKD. One patient with renal failure in group L showed no recovery of renal function and maintenance hemodialysis became necessary within 1 month after the start of treatment. Except for this patient, eGFR data at 1 month after treatment were available for 31 patients (group L 17; group H 14). The initial prednisolone dose was 0.47±0.12 mg/kg daily in group L and 0.84±0.16 mg/kg daily in group H, being significantly lower (P<0.001) in the former. There was no significant inter-group difference in patient age, sex or pretreatment eGFR. In both groups, the pretreatment eGFR was significantly improved at 1 month after the start of corticosteroid therapy [30.5±15.7 to 41.8±14.9 ml/min in group L (p<0.05) and 32.7±13.8 to 46.6±17.0 mi/min in group H (p<0.05)], and the degree of improvement showed no significant inter-group difference. Fifteen of the 41 patients were followed up for over 36 months (39 – 210 months, median 56 months), and all of them had been maintained on low-dose prednisolone (5.1 ± 2.1 mg daily) at the last review. No patient showed progression to end-stage renal disease, and there was no significant difference in eGFR at the last review (45.1 ±11.3 ml/min) in comparison to that at 1 month after the start of treatment (42.5 ±12.8 ml/min).

Conclusion: In IgG4-RKD, prednisolone 0.5 mg/kg daily is sufficient for induction therapy, and the recovery of renal function during the first month of this treatment can be maintained for a long period on low-dose corticosteroid maintenance therapy.


Disclosure:

T. Saeki,
None;

M. Kawano,
None;

I. Mizushima,
None;

M. Yamamoto,
None;

Y. Ubara,
None;

H. Nakashima,
None;

Y. Wada,
None;

T. Ito,
None;

H. Yamazaki,
None;

I. Narita,
None;

T. Saito,
None.

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