Clinical Research Papers:

Clinical features and prognostic factors of ten patients with renal failure caused by IgG4-related retroperitoneal fibrosis

Wei Zhang, Feng Xue, Cui Wang and Leping Shao _

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Oncotarget. 2018; 9:2858-2865. https://doi.org/10.18632/oncotarget.23103

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Wei Zhang1,*, Feng Xue2,*, Cui Wang1 and Leping Shao1

1Department of Nephrology, The Affiliated Hospital of Qingdao University, Qingdao 266003, China

2Department of Anesthesiology, The Affiliated Hospital of Qingdao University, Qingdao 266003, China

*Co-first authors

Correspondence to:

Leping Shao, email: [email protected]

Keywords: IgG4-related disease; retroperitoneal fibrosis; renal failure; prognostic factors; clinical features

Received: June 23, 2017     Accepted: November 15, 2017     Published: December 07, 2017


IgG4-related retroperitoneal fibrosis (IgG4-RPF) is a newly recognized entity which often mimics cancer. We aimed to investigate the clinical features and the causes of renal failure, as well as to explore the factors affecting the prognosis of renal function by analysis of the clinical data of patients with IgG4-RPF. We reviewed clinical features of 10 patients with renal failure caused by IgG4-RPF, which was confirmed by pathology review and clinic-pathologic correlations. All patients were male, and the mean age at onset was 64.2 ± 10.0 years. Five patients were revealed with acute renal failure (ARF), while the other five ones with ARF on chronic kidney disease (CKD) (A on C) at diagnosis. Initial favorable responses obtained in 90% of the patients who underwent steroid therapy. The serum creatinine (SCr) level returned to normal in six patients including five with ARF and one with A on C, while those of the rest four patients with A on C restored to baseline levels (GFR remained below 60 ml/min/1.73 m2 however) after therapy. These four unrecovered patients had a history of CKD, a longer period of persistently elevated SCr, a thinner total renal parenchyma thickness, and continuous elevated serum IgG4 levels after steroid therapy, compared with those recovered patients (P < 0.05). We concluded that recovery and long-term prognosis of the disease were primarily associated with timely diagnosis and proper treatment.

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