Abstract
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.
Highlights
IgG4-related disease (IgG4-RD) is a newly recognized entity characterized by tumour-like swelling in multiple sites caused by lymphoplasmacytic infiltration and sclerosis, and it has been associated with elevated serum IgG4 level and infiltration of IgG4-positive plasma cells in the involved organs and tissues [1, 2]
Various initial symptoms were predominantly related to retroperitoneal fibrosis (RPF), including back pain (10%), edema of the lower extremities (20%), oliguria (10%), weight loss (20%) and hypertension (10%), while other initial symptoms were due to non-RPF associated diseases, including swelling of salivary glands (30%), mediastinal tumour (10%), swelling of the lacrimal glands (10%), dry mouth (10%), pulsatile abdominal mass (10%) and pain of the lower extremities (10%)
There have been several reports on IgG4-RPF [12], but none of them were conducted from the aspect of renal function on the short and long-term outcomes, as well as prognosis risk factors
Summary
IgG4-related disease (IgG4-RD) is a newly recognized entity characterized by tumour-like swelling in multiple sites caused by lymphoplasmacytic infiltration and sclerosis, and it has been associated with elevated serum IgG4 level and infiltration of IgG4-positive plasma cells in the involved organs and tissues [1, 2]. IgG4-RD, which can occur as a multi-organ entity, unifies a large number of medical diagnoses previously considered as being confined to a single organ system. This disorder was firstly described from autoimmune pancreatitis (AIP) [3, 4], which is regarded as a pancreatic lesion of IgG4RD [5, 6]. The diagnoses were confirmed by pathology review according to consensus diagnostic criteria and clinicopathologic www.impactjournals.com/oncotarget correlations; but serum IgG4 concentrations were not necessary any more [13]
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