Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis: a retrospective study of 132 patients

2019 ◽  
Author(s):  
Yanying Liu ◽  
Kunkun Wang ◽  
Zhenfan Wang ◽  
Qiaozhu Zeng ◽  
Lijuan Zhu ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Disease Onset ◽  
Allergic Diseases ◽  
Organ Involvement ◽  
Case Report Form ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Male Predominance ◽  
Serum Igg4 ◽  
Peking University ◽  
Group A

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.

scholarly journals Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis

PLoS ONE ◽  
2021 ◽  
Vol 16 (2) ◽  
pp. e0245601
Author(s):  
Kunkun Wang ◽  
Zhenfan Wang ◽  
Qiaozhu Zeng ◽  
Lijuan Zhu ◽  
Jingyuan Gao ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Disease Onset ◽  
Allergic Diseases ◽  
Organ Involvement ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Male Predominance ◽  
Serum Igg4 ◽  
Peking University ◽  
Chinese Cohort ◽  
Secondary Form

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.

Salivary gland involvement disparities in clinical characteristics of IgG4-related disease: a retrospective study of 428 patients

Rheumatology ◽  
2019 ◽  
Author(s):  
Yanying Liu ◽  
Miao Xue ◽  
Zhenfan Wang ◽  
Qiaozhu Zeng ◽  
Limin Ren ◽  
...  
Keyword(s):  
Salivary Gland ◽  
Disease Onset ◽  
Allergic Diseases ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Younger Age ◽  
Serum Igg4 ◽  
Peking University ◽  
Igg4 Level

Abstract Objectives IgG4-related disease (IgG4-RD) has recently been recognized as a fibro-inflammatory condition featuring tumefactive lesions in multiple organs, and the salivary gland is one of the most commonly involved sites. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-RD patients with salivary gland lesions (IgG4-RD SG+) and salivary-gland-free IgG4-RD (IgG4-RD SG−) in a large cohort. Methods We carried out a retrospective review of the medical records of 428 cases of IgG4-RD diagnosed at Peking University People’s Hospital between March 2006 and May 2018. Results Among 428 patients, 249 had salivary glands that were affected. IgG4-RD SG+ patients showed younger age at disease onset and diagnosis, and a longer interval between symptom onset and diagnosis. The IgG4-RD SG+ group involved more female patients, and allergic diseases were more common in this group. In terms of organ involvement, the IgG4-RD SG+ group were more frequently presented with lacrimal gland involvement, while lymph node, retroperitoneal fibrosis, pancreas, biliary system, kidney and aorta were more prominent in the IgG4-RD SG− group. In addition, the serum IgG4 level, IgG4/IgG ratio and IgE level were significantly higher in IgG4-RD SG+ patients. Patients with eosinophilia were more common in the IgG4-RD SG+ group, while elevated ESR, CRP and positive ANA were more common in the IgG4-RD SG− group. Conclusion We have revealed demographic, clinical and laboratory differences between IgG4-RD SG+ and SG− patients, which indicated potential differences in pathogenesis and important implications for the diagnosis and management of these two phenotypes.

scholarly journals Serum IgG4 Concentration is a Potential Predictive Biomarker in Glucocorticoid Treatment for Idiopathic Retroperitoneal Fibrosis

2022 ◽  
Author(s):  
Shoichiro Mukai ◽  
Naotaka Sakamoto ◽  
Hiroaki Kakinoki ◽  
Tadamasa Shibuya ◽  
Ryosuke Moriya ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Predictive Marker ◽  
Predictive Biomarker ◽  
Complete Response ◽  
Glucocorticoid Therapy ◽  
Smoking History ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Glucocorticoid Treatment ◽  
Female Ratio ◽  
Serum Igg4

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.

scholarly journals A Case of Immunoglobulin G4-Related Disease with Extensive Multiorgan Involvements

2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Kazuhiko Higashioka ◽  
Kenji Yoshida ◽  
Kensuke Oryoji ◽  
Kazuo Kamada ◽  
Shinichi Mizuki ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Elevated Serum ◽  
Clinical Findings ◽  
Organ Involvement ◽  
Immunoglobulin G4 ◽  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

We report a case of IgG4-related disease (IgG4-RD) with multiple ten-organ involvement. This case showed many clinical findings, such as bilateral swelling of salivary and lacrimal glands, autoimmune pancreatitis, interstitial nephritis, retroperitoneal fibrosis, periaortitis, systemic swelling of lymph nodes, pulmonary lesions, splenomegaly, and jejunal lesions. He was suspected as having SLE or malignant lymphoma but diagnosed as having IgG4-RD by the elevated serum IgG4 level and histological findings from kidney and lymph node. We report a case of IgG4-RD with multiple ten-organ involvement that was successfully treated with prednisolone therapy.

Elevated serum IgG4 levels in diagnosis and treatment response in patients with idiopathic retroperitoneal fibrosis

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
Keyword(s):  
Treatment Response ◽  
Retroperitoneal Fibrosis ◽  
Elevated Serum ◽  
Diagnosis And Treatment ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Serum Igg4

Clinical features of IgG4-related retroperitoneal fibrosis among 407 patients with IgG4-related disease: a retrospective study

Rheumatology ◽  
2020 ◽  
Author(s):  
Yanying Liu ◽  
Lijuan Zhu ◽  
Zhenfan Wang ◽  
Qiaozhu Zeng ◽  
Fei Yang ◽  
...  
Keyword(s):  
Medical Records ◽  
Retroperitoneal Fibrosis ◽  
Disease Onset ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Kidney Involvement ◽  
Peking University ◽  
Male Patients ◽  
The Common

Abstract Objectives IgG4-related disease (IgG4-RD) is recently recognized as a fibro-inflammatory condition featured by tumefactive lesions in multiple organs, and the retroperitoneum is one of the common involved sites. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-RD patients with retroperitoneum lesion (IgG4-RD RPF+) and retroperitoneum free IgG4-RD (IgG4-RD RPF−) in a large cohort. Methods We carried out a retrospective review of the medical records of 407 cases of IgG4-RD diagnosed at Peking University People’s Hospital between March 2009 and May 2019. Results Among 407 patients, 58 had retroperitoneum affected. As compared with IgG4-RD RPF− patients, IgG4-RD RPF+ patients showed older age at disease onset and diagnosis. IgG4-RD RPF+ group involved more male patients. In terms of organ involvement, IgG4-RD RPF+ group was more frequently presented with kidney involvement, while salivary gland, lacrimal gland and pancreas were more prominent in the IgG4-RD RPF− group. In addition, the CRP, ESR level and creatinine level were significantly higher in IgG4-RD RPF+ patients, and hypocomplementemia were more common in this group. Conclusion We have revealed demographic, clinical and laboratory differences between IgG4-RD RPF+ and RPF− patients, which indicated potential differences in pathogenesis and important implications for the diagnosis and management of these two phenotypes.

A case of idiopathic retroperitoneal fibrosis

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

scholarly journals Low-dose sublingual immunotherapy compared to subcutaneous immunotherapy and conventional therapy in childhood asthma

2016 ◽  
Vol 44 (6) ◽  
pp. 243
Author(s):  
Ariyanto Harsono
Keyword(s):  
Childhood Asthma ◽  
Low Dose ◽  
Sublingual Immunotherapy ◽  
Disease Onset ◽  
Ethics Committee ◽  
Subcutaneous Immunotherapy ◽  
Control Group ◽  
High Dose ◽  
Treat Ment ◽  
Group A

Background Evidence begin to accumulate that high-dose sub-lingual immunotherapy (SLIT) is as effective as subcutaneousimmunotherapy (SIT) in the treatment of childhood asthma.Since the capacity of sublingual area is similar whether the doseis high or low, the efficacy of low dose may be important to bestudied.Objective To investigate the efficacy of low-dose sublingual im-munotherapy in the treatment of childhood asthma.Methods Parents signed informed consent prior to enrollment,after having received information about the study. Patients weremoderate asthma aged 6-14 years with disease onset of lessthan 2 years before the commencement of the study and peakexpiratory flow rate (PEFR) variability of more than 15%. Pa-tients were randomly allocated into group A, B, and C whoreceived subcutaneous immunotherapy, low-dose sublingualimmunotherapy, and conventional asthma therapy, respectively.Randomization was stratified into two strata according to agei.e., 6-11 years or 11-14 years. Patients of each stratum wererandomized in block of three for each group. At the end of threemonths, lung function tests were repeated. The primary outcomewas PEFR variability at the end of the study. The study wasapproved by the Ethics Committee of Soetomo HospitalSurabaya.Results Distribution of variants as represented by sex, age,eosinophil count, and total IgE concentration were normal inthe three groups. PEFR variability decreased significantly from16.97+0.81 to 8.50+5.08 and 17.0+0.87 to 8.40+4.72 in groupreceiving SIT and SLIT, respectively (p<0.05), but decreasednot significantly from 17.00+0.83 to 10.82+0.5.41 in control group(p>0.05).Conclusion Low-dose SLIT is as efficacious as SIT in the treat-ment of moderate asthma in children

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