scholarly journals The Baseline Serum IgG4/IgG3 Ratio Might be a Suitable Marker for Predicting Favorable Treatment Response in Patients With Idiopathic Retroperitoneal Fibrosis-associated Hydronephrosis

2021 ◽  
Vol 28 (2) ◽  
pp. 53-54
Author(s):  
Seung-Ki Kwok
2017 ◽  
Vol 36 (4) ◽  
pp. 903-912 ◽  
Author(s):  
L.G. Pelkmans ◽  
T.R. Hendriksz ◽  
P.J. Westenend ◽  
H.J. Vermeer ◽  
E.F.H. van Bommel

2022 ◽  
Author(s):  
Shoichiro Mukai ◽  
Naotaka Sakamoto ◽  
Hiroaki Kakinoki ◽  
Tadamasa Shibuya ◽  
Ryosuke Moriya ◽  
...  

Abstract Objectives: To evaluate the management and outcome of idiopathic retroperitoneal fibrosis (iRPF) in Japan, and identify its clinical biomarker. Methods: We retrospectively analyzed 129 patients with iRPF treated between January 2008 and May 2018 at 12 university and related hospitals. Patients treated with glucocorticoid were analyzed to identify a predictive biomarker. These patients were classified into three groups according to overall effectiveness (no change: NC, complete response: CR and partial response groups: PR), and each parameter was compared statistically.Results: Male-female ratio was 5: 1, and median age at diagnosis was 69 (33-86) years. Smoking history was reported in 59.6% of the patients. As treatment, 95 patients received glucocorticoid therapy with an overall response rate of 84%. As a result, serum concentration of IgG4 was significantly decreased in NC group compared with the other two groups (56.6mg/dL vs 255mg/dL, 206mg/dL, P=0.0059 and 0.0078). ROC analysis was performed between the non-responder (NC) and responder groups (CR+PR) to identify the cut-off value of serum IgG4 as a predictive marker. As a result, AUC was 0.793 and the values of sensitivity and specificity were 0.85 and 0.64, respectively, under the cut-off values of 67.6mg/dL. Conclusions: In the majority of iRPF patients, glucocorticoid therapy resulted in a favorable response. Pre-treatment serum IgG4 concentration may have potential as a predictive biomarker of steroid treatment.


PLoS ONE ◽  
2021 ◽  
Vol 16 (2) ◽  
pp. e0245601
Author(s):  
Kunkun Wang ◽  
Zhenfan Wang ◽  
Qiaozhu Zeng ◽  
Lijuan Zhu ◽  
Jingyuan Gao ◽  
...  

Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large Chinese cohort. We retrospectively reviewed the medical records of 132 RPF patients diagnosed at Peking University People’s Hospital between March 2010 and March 2018. Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients. We described the demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, indicating their potential differences in pathogenesis, which was of great importance to diagnose and manage the two phenotypes.


2019 ◽  
Author(s):  
Yanying Liu ◽  
Kunkun Wang ◽  
Zhenfan Wang ◽  
Qiaozhu Zeng ◽  
Lijuan Zhu ◽  
...  

Abstract Backgroud Retroperitoneal fibrosis (RPF) is a condition characterized by the presence of inflammation and fibrosis in the retroperitoneal space. More than two-thirds of all cases of RPF are idiopathic retroperitoneal fibrosis (IRPF), while the remaining one-third stem from different secondary causes. Many studies suggest that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large China cohort.Methods We carried out a retrospective review of the medical records of 132 cases of RPF diagnosed at Peking University People’s Hospital between March 2010 and March 2018. We divided the patients into two groups, IgG4-related RPF group and IRPF group. A standardized case report form was used for data collection. All statistical analyses were performed by SPSS 24.0.Results Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients.Conclusions We have revealed demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, which indicated potential differences in pathogenesis and important implications for the diagnosis and management of these two phenotypes.


2020 ◽  
Vol 8 (1) ◽  
pp. 101-107
Author(s):  
S.V. Shchekaturov ◽  
◽  
M.M. Kaabak ◽  
A.K. Zokoev ◽  
E.R. Charchyan ◽  
...  

1983 ◽  
Vol 76 (12) ◽  
pp. 1023-1025 ◽  
Author(s):  
C D M Fletcher ◽  
P E M Jarrett

Idiopathic retroperitoneal fibrosis is uncommon enough to be placed low down on a list of differential diagnoses of any problem facing a general surgeon in a district hospital. In this paper, 4 cases of retroperitoneal fibrosis are described, all of whom presented within a 5-year period to the same surgeon; each described a different symptomatology. This paper serves to reiterate the diversity of presenting features in retroperitoneal fibrosis and to demonstrate that this condition may not be as rare as is widely believed.


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