scholarly journals 109 Cross-sectional study of seven cases of IgG4 related disease

Rheumatology ◽  
2018 ◽  
Vol 57 (suppl_3) ◽  
Author(s):  
Remesh Bhasi
2020 ◽  
Author(s):  
Eduardo Martín-Nares ◽  
Vanessa Saavedra-González ◽  
Reynerio Fagundo-Sierra ◽  
Blanca Estela Santinelli-Núñez ◽  
Teresa Romero-Maceda ◽  
...  

Abstract Introduction: The clinical utility of serum immunoglobulin free light chains (sFLC) in IgG4-related disease (IgG4-RD) is unknown. Herein we evaluated their association with clinical phenotypes, serology and activity in patients with IgG4-RD.Methods: Cross-sectional study that included 45 patients with IgG4-RD, and as controls 25 with Sjögren’s syndrome (SS) and 15 with sarcoidosis. IgG4-RD patients were classified in clinical phenotypes: pancreato-hepato-biliary, retroperitoneum/aorta, head/neck-limited and Mikulicz/systemic; as well as proliferative vs. fibrotic phenotypes. We assessed the IgG4-RD Responder Index (IgG4-RD RI) at recruitment and measured IgG1, IgG4, κ and λ sFLC serum levels by turbidometry.Results: sFLC levels were similar among IgG4-RD, SS and sarcoidosis groups. Regarding the IgG4-RD patients, the mean age was 49 years, 24 (53.3%) were men and 55.5% had activity. Eight (17.7%) belonged to pancreato-hepato-biliary, 6 (13.3%) to retroperitoneum/aorta, 14 (31.1%) to head/neck-limited, 16 (35.5%) to Mikulicz/systemic phenotypes, whereas 36 (80%) to proliferative and 9 (20%) to fibrotic phenotypes. High κ sFLC, λ sFLC and κ/λ ratio were present in 29 (64.4%), 13 (28.9%) and 13 (28.9%) patients, respectively. There were no differences in sFLC among phenotypes. κ sFLC and κ/λ ratio correlated positively with the number of involved organs and IgG4-RD RI. Patients with renal involvement had higher k sFLC and λ sFLC. The AUC for k sFLC and λ sFLC, for renal involvement was 0.78 and 0.72, respectively. Active IgG4-RD had higher levels of κ sFLC and more frequently a high κ/λ ratio. The AUC for k sFLC and k/λ ratio for predicting active IgG4-RD was 0.67 and 0.70, respectively. sFLC correlated positively with IgG1 and IgG4 levels.Conclusions: sFLC may be useful as a biomarker of disease activity as well as multiorgan and renal involvement. In particular, a high κ/λ ratio may identify patients with active disease.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Marlon Vladimir Vázquez Aguirre ◽  
Juan Pablo Godoy-Alonso ◽  
Germán González-de la Cruz ◽  
Andrea Rocha-Haro ◽  
Karla Krystel Ordaz-Candelario ◽  
...  

Abstract Background: Hypophysitis is an inflammatory process of the pituitary gland with different origins. Infundibulum may be also involved, called infundibulum-hypophysitis. Sometimes, enlargement of pituitary gland causes mass effect and anterior or posterior hormonal dysfunction, including hypopituitarism, and diabetes insipidus (DI), respectively. Hypophysitis is a rare autoimmune disease, however, the number of cases have been recently increasing due to higher detection related with more magnetic resonance imaging (MRI) studies. In addition, hypophysitis may be related with autoimmune rheumatologic disease (ARD) such as generalized lupus erythematosus (GLE), granulomatosis with polyangeitis (GPA), IgG4-related disease, and rheumatoid arthritis (RA). Aim: to compare the clinical and biochemical findings among patients with hypophysitis grouped depending on the presence of coexistent ARD activity. Methodology: it is a comparative and cross-sectional study. We registered all data from cases followed-up in Neuroendocrinology and Rheumatology units, from January 1987 to July 2019. Results: 24 patients showed confirmed diagnosis of hypophysitis. Majority of them (n=17, 71%) do not have coexistent diagnosis of ARD. However, 7 cases (29%) presented pituitary involvement with coexistent activity of GPA (n=4, 17%), GLE (n=1, 4%), RA (n=1, 4%) and IgG4-related disease (n=1, 4%). Female gender predominate in hypophysitis cases with and without ARD (p=0.9). Interestingly, age of hypophysitis diagnosis was significantly younger in cases without (38±14) vs. with (49±5) ARD (p=0.01). MRI results showed similar and typical findings related with hypophysitis independently of the presence of ARD. Hypopituitarism was present in the majority of cases, however, none of cases with hypophysitis and ARD showed hypogonadism (p=0.02). DI was present in 15 patients (63%), three of them with ARD (all with GPA). All cases received only medical therapy (i.e., glucocorticoids, rituximab, or azathioprine). Surgery or radiotherapy was not necessary in any case. Conclusions: Almost a 30% of cases with hypophysitis may have coexistent ARD. Pituitary function should be evaluated in cases with previous ARD diagnosis. However, hypogonadism was not present in cases with ARD and hypophysitis. Patients with ARD presented hypophysitis at older age. The remaining clinical and radiological data were similar between groups. References: 1) Clinical Diabetes and Endocrinology. December 2016;2(1). 2) Clin Rheumatol. 2019 Aug 24. doi: 10.1007/s10067-019-04735-7


PLoS ONE ◽  
2019 ◽  
Vol 14 (10) ◽  
pp. e0224042
Author(s):  
Daisuke Yoneoka ◽  
Shuhei Nomura ◽  
Kayo Kurotani ◽  
Shiori Tanaka ◽  
Keiji Nakamura ◽  
...  

2020 ◽  
Author(s):  
Rupa Shiwakoti ◽  
Yogendra Bahadur Gurung ◽  
Ram Sharan Pathak ◽  
Ram Chandra Poudel ◽  
Sandesh Neupane ◽  
...  

Abstract Background Despite Sustainable Development Goals explicit call for ensuring “Universal access to Sexual and Reproductive Health(SRH)”, and specific articles in the United Nations Convention on the Rights of Persons with Disabilities, women with disabilities(WwD) continue to experience barriers to access SRH services in Nepal. This study assessed factors affecting utilization of SRH services among WwD in Ilam district, Nepal. Methods A mixed method cross-sectional study of 384 WwD of reproductive age was conducted in Ilam district, Nepal. Face-to-face interviews were conducted using structured questionnaire. Relationships between utilization of SRH services and associated factors were explored using multivariate logistic regression. In-depth interviews of WwD, key informant interviews of health workers and local political leaders, and focus group discussions with Female Community Health Volunteers were used to collect qualitative data and underwent thematic analysis. Data were analyzed using Health Belief Model. Results One in five (15%) WwD had ever utilized SRH services. No need of (57%) and unaware of SRH services (24%) were main reasons for not utilizing SRH services. Majority (81%) of them reported that nearest health facility was not disability-friendly (73%) specifically referring to road (48%). A multivariate analysis showed factors positively associated with utilization of SRH services that included being ever-married (AOR = 121.7, 95% CI: 12.206-1214.338), perceived need of SRH services (AOR = 5.5; 95% CI: 1.419–21.357) and perceived susceptibility to SRH related disease/condition (AOR = 6.0; 95% CI:1.978–18.370). Qualitative findings revealed illiteracy, low economic and social class, lack of information, severity of disability, lack of disability friendly environment (no family support, distant health facility, inaccessible-infrastructure, stigmatization, bad behavior and attitude of health care providers), perception that SRH is needed only for married, and negative individual perception towards SRH (no need of SRH services, not susceptible to SRH related disease/condition and SRH disease/condition is not severe) as barriers to utilization of SRH services among WwD. Conclusions Utilization of SRH services among WwD was very low in Ilam district, Nepal. Promoting awareness raising program, ensuring disability-friendly environment, and prioritizing SRH services irrespective of marital status could increase SRH service utilization among WwD.


2020 ◽  
Author(s):  
Naomi Yagi ◽  
Yoshitada Sakai ◽  
Naoko Kawamura ◽  
Hitoshi Maezawa ◽  
Yutaka Hata ◽  
...  

Abstract It has recently been shown that the aging population is refractory to the maintenance of swallowing function, which can seriously affect quality of life. Singing and vocal training contribute to mastication, swallowing and respiratory function. Previous studies have shown that singers have better vocal cord health. No consensus has been reached as to how vocal training affects swallowing function. Our study was designed to establish evidence that singers are statistically superior at swallowing function. In an effort to test our hypothesis we undertook a clinical trial on 55 singers and 141 non-singers (mean age: 60.1 ± 11.7 years). This cross-sectional study with propensity score matching resulted in significant differences in the repetitive saliva swallowing test among singers: 7.1 ± 2.4, n =53 vs non-singers: 5.9 ± 1.9, n =53, p < 0.05. We therefore conclude that singing can serve an important role in stabilizing the impact of age-related disease on speech.


2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Alenka Franko ◽  
Katja Goricar ◽  
Metoda Dodic Fikfak ◽  
Viljem Kovac ◽  
Vita Dolzan

Abstract Background The study investigated the influence of GCLC, GCLM, GSTM1, GSTT1 and GSTP1 polymorphisms, as well as the influence of interactions between polymorphism and interactions between polymorphisms and asbestos exposure, on the risk of developing pleural plaques, asbestosis and malignant mesothelioma (MM). Subjects and methods The cross sectional study included 940 asbestos-exposed subjects, among them 390 subjects with pleural plaques, 147 subjects with asbestosis, 225 subjects with MM and 178 subjects with no asbestos-related disease. GCLC rs17883901, GCLM rs41303970, GSTM1 null, GSTT1 null, GSTP1 rs1695 and GSTP1 rs1138272 genotypes were determined using PCR based methods. In statistical analysis, logistic regression was used. Results GSTT1 null genotype was associated with the decreased risk for pleural plaques (OR = 0.63; 95% CI = 0.40–0.98; p = 0.026) and asbestosis (OR = 0.51; 95% CI = 0.28–0.93; p = 0.028), but not for MM. A positive association was found between GSTP1 rs1695 AG + GG vs. AA genotypes for MM when compared to pleural plaques (OR = 1.39; 95% CI = 1.00–1.94; p = 0.049). The interactions between different polymorphisms showed no significant influence on the risk of investigated asbestos-related diseases. The interaction between GSTT1 null polymorphism and asbestos exposure decreased the MM risk (OR = 0.17; 95% CI = 0.03–0.85; p = 0.031). Conclusions Our findings suggest that GSTT1 null genotype may be associated with a decreased risk for pleural plaques and asbestosis, may modify the association between asbestos exposure and MM and may consequently act protectively on MM risk. This study also revealed a protective effect of the interaction between GSTP1 rs1695 polymorphism and asbestos exposure on MM risk.


Sign in / Sign up

Export Citation Format

Share Document