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 Advances in IgG4-related Hepatobiliary Disease

2020 ◽  
Vol 25 (2) ◽  
pp. 100-106
Author(s):  
Sung-Hoon Moon
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Quantitative Polymerase Chain Reaction ◽  
High Serum ◽  
Hepatobiliary Disease ◽  
Glucocorticoid Therapy ◽  
Glucocorticoid Treatment ◽  
Immunoglobulin G4 ◽  
Serum Igg4 ◽  
Polymerase Chain

The emergence of the new disease entity of steroid responsive immunoglobulin G4 (IgG4)-related hepatobiliary disease has generated considerable interest among hepatobiliary society. IgG4-related hepatobiliary disease refers to mainly IgG4-related sclerosing cholangitis (IgG4-SC), and includes a small number of IgG4-related hepatopathy. As IgG4-SC responds well to glucocorticoid therapy, IgG4-SC should be differentiated from cholangiocarcinoma and primary sclerosing cholangitis. Timely diagnosis of IgG4-SC can lead clinicians to prescribe adequate glucocorticoid treatment that can reverse bile duct strictures and cholestatic liver function. Differentiation of IgG4-SC from primary sclerosing cholangitis is sometimes challenging because serum IgG4 and tissue IgG4 have demonstrated low positive predictive value in this setting. Recent research suggested that blood IgG4/IgG RNA ratio by quantitative polymerase chain reaction can be used for differentiation. Although most patients with IgG4-SC/autoimmune pancreatitis respond to glucocorticoid therapy, they frequently experience relapse of disease. The suggested relapse factors included very high serum IgG4, diffuse enlargement of the pancreas, proximal IgG4-SC, and multi-organ involvement. This review discusses the recent advances in the pathogenesis, diagnosis, management, disease relapse, and monitoring disease activity of IgG4-SC.

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.

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.

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

scholarly journals Clinical course of pituitary function and image in IgG4-related hypophysitis

2017 ◽  
Vol 2017 ◽  
Author(s):  
Takatoshi Anno ◽  
Fumiko Kawasaki ◽  
Maiko Takai ◽  
Ryo Shigemoto ◽  
Yuki Kan ◽  
...  
Keyword(s):  
Pituitary Gland ◽  
Adrenal Insufficiency ◽  
Clinical Course ◽  
Laboratory Data ◽  
Glucocorticoid Therapy ◽  
High Dose ◽  
Glucocorticoid Treatment ◽  
Serum Igg4 ◽  
Physiological Dose ◽  
Igg4 Level

Summary A 76-year-old man had a hypopituitarism including adrenal insufficiency, hypogonadism and hypothyroidism. Based on various findings including the swelling of the pituitary gland, increase of serum IgG4 level and abundant IgG4-positive plasma cell infiltration in immunostaining of the pituitary gland, we diagnosed this subject as IgG4-related hypophysitis. In general, a high-dose glucocorticoid treatment is effective for IgG4-related disease. His clinical symptom, laboratory data and adrenal insufficiency were almost improved without any therapy. The serum IgG4 level was decreased and pituitary size was normalized with hydrocortisone as physiological replacement. This case report provides the possibility that IgG4 level is decreased spontaneously or with physiological dose of glucocorticoid therapy. Learning points: We performed the pituitary gland biopsy and histochemical examination glucocorticoid therapy in a subject with IgG4-related hypophysitis. This case report provides the possibility that IgG4 level is decreased spontaneously or with a physiological dose of glucocorticoid therapy. We reported the clinical course of IgG4-related hypophysitis without a high-dose glucocorticoid treatment, although there were a few reports about the retrospective examination. Although the patient had still higher IgG4 level compared to normal range, his clinical symptom disappeared and his laboratory data were improved. We should keep in mind the possibility of IgG4-related hypophysitis when we examine one of the uncertain causes of a hypopituitarism including adrenal insufficiency, hypogonadism and hypothyroidism.

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 Cathepsin K: A Novel Diagnostic and Predictive Biomarker for Renal Tumors

Cancers ◽  
2021 ◽  
Vol 13 (10) ◽  
pp. 2441
Author(s):  
Anna Caliò ◽  
Matteo Brunelli ◽  
Stefano Gobbo ◽  
Pedram Argani ◽  
Enrico Munari ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Therapy Response ◽  
Cathepsin K ◽  
Mtor Inhibitors ◽  
Predictive Marker ◽  
Predictive Biomarker ◽  
Renal Tumors ◽  
Epithelioid Angiomyolipoma

Cathepsin K is a papain-like cysteine protease with high matrix-degrading activity. Among several cathepsins, cathepsin K is the most potent mammalian collagenase, mainly expressed by osteoclasts. This review summarizes most of the recent findings of cathepsin K expression, highlighting its role in renal tumors for diagnostic purposes and as a potential molecular target. Indeed, cathepsin K is a recognized diagnostic tool for the identification of TFE3/TFEB-rearranged renal cell carcinoma, TFEB-amplified renal cell carcinoma, and pure epithelioid PEComa/epithelioid angiomyolipoma. More recently, its expression has been observed in a subgroup of eosinophilic renal neoplasms molecularly characterized by TSC/mTOR gene mutations. Interestingly, both TSC mutations or TFE3 rearrangement have been reported in pure epithelioid PEComa/epithelioid angiomyolipoma. Therefore, cathepsin K seems to be a downstream marker of TFE3/TFEB rearrangement, TFEB amplification, and mTOR pathway activation. Given the established role of mTOR inhibitors as a pharmacological option in renal cancers, cathepsin K could be of use as a predictive marker of therapy response and as a potential target. In the future, uropathologists may implement the use of cathepsin K to establish a diagnosis among renal tumors with clear cells, papillary architecture, and oncocytic features.

scholarly journals Variable Symptomatology in Idiopathic Retroperitoneal Fibrosis

1983 ◽  
Vol 76 (12) ◽  
pp. 1023-1025 ◽  
Author(s):  
C D M Fletcher ◽  
P E M Jarrett
Keyword(s):  
Retroperitoneal Fibrosis ◽  
District Hospital ◽  
Differential Diagnoses ◽  
General Surgeon ◽  
Idiopathic Retroperitoneal Fibrosis

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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