Differences in clinical characteristics of IgG4-related disease across age groups: a prospective study of 737 patients

Rheumatology ◽  
2020 ◽  
Author(s):  
Hui Lu ◽  
Fei Teng ◽  
Panpan Zhang ◽  
Yunyun Fei ◽  
Linyi Peng ◽  
...  
Keyword(s):  
Prospective Study ◽  
Clinical Characteristics ◽  
Clinical Symptoms ◽  
Age Groups ◽  
Organ Involvement ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Cox Analysis ◽  
A Prospective Study ◽  
The Impact

Abstract Objective The aim of this study was to compare the clinical characteristics of IgG4-related disease (IgG4-RD) among different age groups. Methods We conducted a prospective study of 737 patients who were newly diagnosed with IgG4-RD and compared detailed demographic features, organ involvements, laboratory tests, treatments and outcomes across age groups. The patients were divided into five groups according to their age at diagnosis: ≤39, 40–49, 50–59, 60–69 and ≥70 years. The clinical characteristics of paediatric patients were also described. Results Sex ratio, disease duration, allergy history and clinical symptoms were significantly different across age groups. Besides, the proportions of superficial organ involvement (lacrimal gland and sinus) decreased with age, while the proportions of internal organ involvement (pancreas, biliary tract, retroperitoneal tissue, lung and prostate) increased with age, which was more prominent in male patients. Mikulicz’s disease was the most common manifestation (70%) in paediatric IgG4-RD patients. Multiple Cox analysis identified that age ≤56 years at diagnosis was an independent risk factor of relapse. Conclusion We revealed the impact of age on clinical characteristics of IgG4-RD, which indicated that different management might be required among different age groups.

scholarly journals Study of correlation of ultrasonography with surgical evaluation of adnexal masses: a prospective study

2018 ◽  
Vol 7 (10) ◽  
pp. 4218
Author(s):  
Urmila Tripathi ◽  
Geetanjali Munda
Keyword(s):  
Prospective Study ◽  
Clinical Symptoms ◽  
Histopathological Examination ◽  
Abdominal Mass ◽  
Age Groups ◽  
Serous Cystadenoma ◽  
Ovarian Tumors ◽  
Adnexal Masses ◽  
Malignant Changes ◽  
A Prospective Study

Background: Adnexal masses are one of the most common pathologies among women of all age groups. Ovarian tumors, alone, represent two thirds of these cases. Malignant ovarian tumors are the fourth most common cause of death in women. Accurate diagnosis is required foremost for proper treatment and management of the patients.Methods: A prospective study done on 100 patients with adnexal masses presenting to Department of Obstetrics and Gynecology at Kamla Raja Hospital, G.R.M.C, Gwalior from February 2015 to August 2016. Firstly, the cases were studied by ultrasonography then intraoperatively and simultaneous sampling for HPE done. The study included women with clinical symptoms of pain abdomen/ discomfort, bleeding per vaginum, abdominal mass was subjected to ultrasonography, diagnosed with adnexal mass. 100 indicated patients were taken for surgery and intraoperative tissue and fluid samples were taken and sent for HPE.Results: No discordance found regarding laterality of adnexal masses between ultrasonographical findings and surgical findings. 69% cases were devoid of any septation/locules/nodules. Most common pathology found to be ectopic pregnancy. Most common benign ovarian mass encountered was serous cystadenoma (31.1%) and malignant mass was serous adenocarcinoma (12.7%). Apart from 13 malignant adnexal masses, 2 adnexal masses had malignant changes found on histopathological examination. 11 cases were found to be of advanced stage on surgical findings, which then confirmed by HPE.Conclusions: There is positive correlation between ultrasonographical and surgerical evaluation of adnexal masses. Correlation of the lesion’s location and appearance at imaging with the surgical findings will aid in the detection of potential pathology reporting errors.

Sex disparities in clinical characteristics and prognosis of immunoglobulin G4–related disease: a prospective study of 403 patients

Rheumatology ◽  
2018 ◽  
Vol 58 (5) ◽  
pp. 820-830 ◽  
Author(s):  
Liwen Wang ◽  
Panpan Zhang ◽  
Xia Zhang ◽  
Wei Lin ◽  
Hanqi Tang ◽  
...  
Keyword(s):  
Treatment Outcomes ◽  
Symptom Onset ◽  
Clinical Characteristics ◽  
Organ Involvement ◽  
Female Patients ◽  
Related Disease ◽  
Sex Disparities ◽  
Male Patients ◽  
Male Sex ◽  
The Impact

Abstract Objectives To study the impact of sex on the clinical presentation of IgG4-related disease (IgG4-RD). Methods We prospectively enrolled 403 newly diagnosed IgG4-RD patients. We compared the demographic features, clinical manifestations, organ involvement, laboratory tests and treatment outcomes between female and male patients. The organs involved were divided into superficial organs (salivary glands, lacrimal glands, orbit, sinus and skin) and internal organs (all the other organs). The patients treated with glucocorticoids with or without additional immunosuppressants were included in the assessment of treatment outcomes, and potential confounding factors were corrected by propensity score matching or multivariate Cox regression analysis. Results Female patients showed younger age at both symptom onset and diagnosis, and a longer interval between symptom onset and diagnosis. Allergy history, Mikulicz’s disease and thyroiditis were more common in female patients, while autoimmune pancreatitis, sclerosing cholangitis and retroperitoneal fibrosis were more common in male patients. In accordance, female patients more frequently presented with superficial organ involvement, while male patients more frequently had internal organ involvement, and the discrepancy was more prominent in the patients with older age. Male sex was associated with higher peripheral eosinophils, CRP and IgG4 levels at baseline. In response to glucocorticoid-based therapies, male sex was associated with a higher IgG4-RD responder index during follow-up as well as a greater risk of relapse (hazard ratio 3.14, P = 0.003). Conclusion Our study revealed the sex disparities in clinical characteristics of IgG4-RD, and indicated that male sex was independently associated with worse prognosis in response to glucocorticoid-based therapies.

scholarly journals Clinical characteristics of immunoglobulin G4–related disease: a prospective study of 118 Chinese patients

Rheumatology ◽  
2015 ◽  
Vol 54 (11) ◽  
pp. 1982-1990 ◽  
Author(s):  
Wei Lin ◽  
Sha Lu ◽  
Hua Chen ◽  
Qingjun Wu ◽  
Yunyun Fei ◽  
...  
Keyword(s):  
Prospective Study ◽  
Clinical Characteristics ◽  
Chinese Patients ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
A Prospective Study

IgG4-related disease in a Chinese cohort: a prospective study

2013 ◽  
Vol 43 (1) ◽  
pp. 70-74 ◽  
Author(s):  
H Chen ◽  
W Lin ◽  
Q Wang ◽  
Q Wu ◽  
L Wang ◽  
...  
Keyword(s):  
Prospective Study ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Chinese Cohort ◽  
A Prospective Study

Lymphadenopathy in IgG4-related disease: a phenotype of severe activity and poor prognosis, with eotaxin-3 as a new biomarker

Rheumatology ◽  
2020 ◽  
Author(s):  
Satoshi Takanashi ◽  
Jun Kikuchi ◽  
Takanori Sasaki ◽  
Mitsuhiro Akiyama ◽  
Hidekata Yasuoka ◽  
...  
Keyword(s):  
Proteomic Analysis ◽  
Poor Prognosis ◽  
Clinical Characteristics ◽  
Organ Involvement ◽  
Healthy Controls ◽  
Newly Diagnosed ◽  
Serum Concentrations ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

Abstract Objective To clarify relevant proteins and clinical characteristics of a phenotype of IgG4-related disease (IgG4-RD) with lymphadenopathy. Methods We enrolled patients newly diagnosed with IgG4-RD in our department between January 2000 and June 2018 and performed proteomic analysis to measure serum concentrations of 1305 proteins. We extracted proteins overexpressed in patients with IgG4-RD with lymphadenopathy by comparing between those with lymphadenopathy, those without lymphadenopathy and healthy controls. We further reviewed all the patients with IgG4-RD in our institution and investigated the characteristics and prognosis of the patients with IgG4-RD with lymphadenopathy. Results Eighty-five patients with IgG4-RD were enrolled, of which, 55% had lymphadenopathy. Proteomic analysis in 31 patients with IgG4-RD and 6 healthy controls revealed that eotaxin-3 was a potential serum biomarker in the patients with lymphadenopathy versus those without lymphadenopathy and healthy controls. A cohort of 85 patients with IgG4-RD demonstrated that patients with lymphadenopathy showed a significantly higher serum IgG4, IgG4:IgG ratio, IgG4-RD responder index and eosinophilia (P < 0.001 for all), irrelevant of the extent to which organ involvement developed. Patients with lymphadenopathy treated with glucocorticoid alone relapsed with significantly higher rates than those without lymphadenopathy (P = 0.03). Conclusion Lymphadenopathy in IgG4-RD represents a phenotype associated with high disease activities, eosinophilia and relapsing disease. Eotaxin-3 is a novel biomarker related to IgG4-RD with lymphadenopathy.

scholarly journals AB1061 2019 ACR/EULAR CLASSIFICATION CRITERIA FOR IGG4-RELATED DISEASE IN RUSSIAN COHORT OF PATIENTS.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1820.1-1821
Author(s):  
E. Sokol ◽  
S. Palshina ◽  
A. Torgashina ◽  
J. Khvan
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Clinical Symptoms ◽  
Classification Criteria ◽  
The Body ◽  
Cell Counting ◽  
Organ Involvement ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
The Mean

Background:IgG4-related disease (IgG4-RD) is a systemic immunomediated fibroinflammatory condition that can affect almost any organ in the body. This is the reason for dramatic variety of clinical symptoms and complexity of diagnostics. 2011 Comprehensive diagnostic criteria (CDC) for IgG4-RD are used to establish the diagnosis for all lesions (except autoimmune pancreatitis type 1). In 2019 the new ACR/EULAR classification criteria for IgG4-RD were proposed to facilitate the formation of more homogenous groups of patients primarily for clinical trials inclusion purpose. They also provide a framework for clinicians considering diagnosis of IgG4-RD.Objectives:To evaluate 2019 ACR/EULAR classification criteria for IgG4-RD in Russian cohort of patients with IgG4-RD.Methods:59 patient with IgG4-RD according to CDC with biopsy proven diagnosis were included.Results:The mean number of affected organs was 2.1; 31 patients (52,5%) were women. Majority of patients had sialoadenitis (25 patients) and/or orbital disease (31 patients), 9 had retroperitoneal fibrosis (RPF). Other affected organs were lungs, pancreas, lymph nodes, paranasal sinuses, thyroid and low urinary tract. Twenty five (25) patients (42,4%) had definite, 14 (23,3%) probable and 20 (34,3%) possible diagnosis of IgG4-RD. Twenty three (23) patients (39%) didn’t fulfill the 2019 ACR/EULAR classification criteria for IgG4-RD. Among them were the majority of patients with RPF (7 patients) who were lacking other organ involvement and IgG4 hypersecretion either in the tissue or serum. The majority of excluded cases were due to inadequate pathomorphological evaluation (lacking of the exact number and percentage (if >40%) of IgG4+ cells), lacking of multi-organ involvement or different patterns of involvement, e.g. in case of lungs involvement.Conclusion:The new 2019ACR/EULAR classification criteria for IgG4-RD are very useful in evaluation of typical organ involvement and systemic course of IgG4-RD. It is essential to adjust Russian pathomorphologists’s approach to cell counting and percentage determination for IgG4-RD cases to get suitable protocols.Disclosure of Interests:None declared

Abstract No. 294: Optimizing IVC filter utilization: a prospective study of the impact of IR consultation

2012 ◽  
Vol 23 (3) ◽  
pp. S119
Author(s):  
R.K. Ryu ◽  
R.J. Lewandowski ◽  
A.C. Eifler ◽  
R. Salem ◽  
R.A. Omary ◽  
...  
Keyword(s):  
Prospective Study ◽  
Ivc Filter ◽  
A Prospective Study ◽  
The Impact
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