Serum granzyme B is associated with otorhinolaryngological, pulmonary, and renal involvement of antineutrophil cytoplasmic antibody-associated vasculitis

2020 ◽  
Vol 69 (1) ◽  
pp. 91-95
Author(s):  
Taejun Yoon ◽  
Juyoung Yoo ◽  
Sung Soo Ahn ◽  
Jason Jungsik Song ◽  
Yong-Beom Park ◽  
...  

We investigated whether serum granzyme B (GrB) can reflect the inflammatory burden such as cross-sectional disease activity and organ-specific involvement in immunosuppressive drug-naïve patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Seventy-eight immunosuppressive drug-naïve patients with AAV were included in this study. At the time of the first classification, whole blood was obtained from each patient and sera was immediately isolated and stored at – 80℃. On the day of the blood sampling, we performed routine laboratory tests including antineutrophil cytoplasmic antibody tests and collected both clinical and laboratory data. AAV-specific indices included Birmingham Vasculitis Activity Score (BVAS) and Five-Factor Score (FFS). The median age of patients with AAV was 62 years and 26 patients were men. Serum GrB was not associated with the cross-sectional BVAS; however, patients with serum GrB positivity exhibited higher frequencies of otorhinolaryngological manifestation than those without (p=0.037). When serum GrB levels were compared after dividing the patients into two groups based on the presence of organ-specific involvement, patients with pulmonary involvement exhibited a significantly higher serum GrB than those without (p=0.042). On the other hand, patients with renal involvement showed a significantly lower serum GrB than those without (p=0.023). In addition, serum GrB was inversely correlated with the cross-sectional FFS (r=−0.249, p=0.028). Even though serum GrB could not reflect the inflammatory burden of AAV, serum GrB was associated with otorhinolaryngological, pulmonary, and renal involvement in immunosuppressive drug-naïve patients with AAV.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1535.2-1536
Author(s):  
J. Fernandes Serodio ◽  
S. Prieto-González ◽  
G. Espígol-Frigolé ◽  
M. Alba ◽  
J. Marco-Hernández ◽  
...  

Background:Classification of ANCA-associated vasculitis (AAV) has emerged in order to identify more homogenous subgroups of patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). However, the exact value of classifying patients according to antibody specificity (proteinase 3 [PR3] or myeloperoxidase [MPO]) is still unclear.Objectives:To assess demographic, clinical and prognostic differences among subgroups of AAV patients, according to clinicopathological classification (GPAvs. MPA) and antibody specificity (PR3vs. MPO) in a single-centre cohort.Methods:A clinical retrospective (1990-2019) observational analysis was performed. Among all patients with ANCA positivity, we analysed patients with GPA and MPA diagnosed according to 2018 Draft Classification Criteria for AAV1, who were homogeneously treated and followed by the authors. Demographic, clinical and laboratory data, as well as disease outcomes, particularly BVAS, disease relapses and survival, were reviewed.Results:Among a total 140 patients with any form of AAV, 32 were excluded for a diagnosis of isolated interstitial lung disease (n=10), cocaine-induced AAV (n=3), ANCA negative or undetermined disease (n=16), atypical ANCA or double PR3/MPO positivity (n=3). Finally, 108 patients with MPA (n=66) or GPA (n=42) were included (83 MPO, 25 PR3). GPA was associated with PR3 in 55% and MPO 45% of patients. MPA was associated with MPO in 97% and PR3 in 3% of patients. GPA patients with PR3 or MPO presented with similar clinical features, disease extent and BVAS. However, compared with GPA/PR3, GPA/MPO were more frequently women (p=0.002). MPA patients presented more frequent with renal involvement (p=0.008) and GPA patients with ENT/ocular involvement (p<0.001). Patients with MPO were older (p=0.028) and more frequently women (p=0.001) than PR3 patients. When antibody specificity was compared, differences on organ-specific manifestations were less clear than between clinical phenotypes (GPA vs. MPA), and were only seen in ENT/ocular involvement (more frequent in PR3 than in MPO patients) and in muscle biopsies disclosing vasculitis (more frequent in MPO than in PR3 patients). GPA and PR3 patients presented more frequent relapsing disease than MPA and MPO patients, respectively (GPA 60% vs. MPA 36%; p=0.018 / PR3 60% vs. MPO 41%; p=0.094). While GPA tended to have a better survival rate than MPA patients (p=0.066) (Graph1), the MPO-associated disease (GPA or MPA) had clearly worse survival prognosis than PR3-AAV (p=0.008) (Graph2), similarly to what occurred in GPA-MPO (compared with GPA-PR3).Conclusion:A high proportion of GPA patients with MPO-ANCA (45%) is observed in our series. GPA is associated with a more frequent relapsing disease than MPA. MPA and presence of MPO were more frequent in females and older patients. Clinical features were similar in GPA patients with PR3 or MPO. The presence of MPO (in GPA or MPA) seems to be the main factor associated with mortality in AAV.Table 1.Symptomatology and ultrasound findings in the patients examined. PMR: Polymyalgia RheumaticaUltrasoundSymptomsCranial(n=17)PMR only(n=17)Non-specific symptoms (n=18)PMR (+) (n=7)PMR (-) (n=10)Temporal (+)7301Facial (+)2100Axilliary (+)0031References:[1]Robson JC, Grayson PC, Ponte C, et al.Draft classification criteria for the ANCA associated vasculitides. Ann Rheum Dis 2018;77 (suppl 2):60-1.Disclosure of Interests:João Fernandes Serodio: None declared, Sergio Prieto-González: None declared, Georgina Espígol-Frigolé: None declared, Marco Alba: None declared, Javier Marco-Hernández: None declared, Maria C. Cid Grant/research support from: Kiniksa Pharmaceuticals, Consultant of: Janssen, Abbvie, Roche, GSK, Speakers bureau: Vifor, José Hernández-Rodríguez: None declared


2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Taejun Yoon ◽  
Sung Soo Ahn ◽  
Jung Yoo Pyo ◽  
Lucy Eunju Lee ◽  
Jason Jungsik Song ◽  
...  

Serum interleukin- (IL-) 27 level has been reported to increase in patients with several autoimmune diseases; however, its significance in patients with antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is unknown. In this study, we investigated the associations between serum IL-27, laboratory features, and activity of AAV and evaluate the predictive ability of serum IL-27 level for disease activity. This study included 77 AAV patients, and we collected clinical and laboratory data at blood sampling. Inflammation-related variables included white blood cell, neutrophil, lymphocyte and platelet counts, serum albumin, erythrocyte sedimentation rate, and C-reactive protein levels. Serum IL-27 and IL-18 levels were measured from stored sera using Human Magnetic Luminex® assay. High disease activity of AAV was defined as the highest tertile of Birmingham vasculitis activity score (BVAS) (≥11). The mean age of the enrolled patients was 59.9 years, and 38 (49.4%) were diagnosed as microscopic polyangiitis. In the multivariable analysis, serum albumin ( β = − 0.419 ) and serum IL-27 level ( β = 0.221 ) were significantly associated with BVAS. Furthermore, patients with renal manifestation exhibited higher serum IL-27 (mean 308.7 pg/mL vs. 105.8 pg/mL) and IL-18 levels (mean 376.7 pg/mL vs. 270.4 pg/mL) than those without. On applying the optimal cut-off of serum IL-27 level for predicting high activity, AAV patients with serum IL − 27   level ≥ 300.8  pg/mL had a significantly higher risk for having high disease activity than those with serum IL − 27   level < 300.8  pg/mL (relative risk 3.380, 95% confidence interval 1.223, 9.345, P = 0.016 ). These results suggest that serum IL-27 level is associated with the cross-sectional activity and the presence of renal manifestation and could be used to predict high disease activity in patients with AAV.


Author(s):  
J.-F. Revol ◽  
Y. Van Daele ◽  
F. Gaill

The only form of cellulose which could unequivocally be ascribed to the animal kingdom is the tunicin that occurs in the tests of the tunicates. Recently, high-resolution solid-state l3C NMR revealed that tunicin belongs to the Iβ form of cellulose as opposed to the Iα form found in Valonia and bacterial celluloses. The high perfection of the tunicin crystallites led us to study its crosssectional shape and to compare it with the shape of those in Valonia ventricosa (V.v.), the goal being to relate the cross-section of cellulose crystallites with the two allomorphs Iα and Iβ.In the present work the source of tunicin was the test of the ascidian Halocvnthia papillosa (H.p.). Diffraction contrast imaging in the bright field mode was applied on ultrathin sections of the V.v. cell wall and H.p. test with cellulose crystallites perpendicular to the plane of the sections. The electron microscope, a Philips 400T, was operated at 120 kV in a low intensity beam condition.


1960 ◽  
Vol 19 (3) ◽  
pp. 803-809
Author(s):  
D. J. Matthews ◽  
R. A. Merkel ◽  
J. D. Wheat ◽  
R. F. Cox

2017 ◽  
Vol 19 (1) ◽  
pp. 7-11
Author(s):  
Vivi Linda Fristianti ◽  
Nur Hidayat ◽  
Slamet Iskandar

Background : IDD problem is a serious problem, 33% of districts in Indonesia are endemic, 21% of endemic mild, moderate endemic 5% and 7% by weight endemic. Urine Iodine Excretion (EYU) illustrates the iodine intake of a person, because 90% of the iodine that enter the body are excreted through the urine. Blocking agents are substances certain minerals such as iron, manganese and calcium, which can bind iodine in groundwater. Objectives : This study aims to determine the relationship of Fe content in ground water sources against EYU levels in school children in the village of the District Cerme Panjatan Kulon Progo. Methods : An observational analytic research with cross sectional design. Data collection is done in May - June 2015. Location research Elementary School Cerme, District Panjatan Kulon Progo. The subjects of the study as many as 34 children in elementary school classroom Cerme 3, 4 and 5 were taken by simple random sampling. Well water and urine samples taken students then tested the iron in the Central Health Laboratory test Yogyakarta and iodine in urine in Magelang BP2GAKY Laboratory. Data analysis using Spearman Range test with significancy level : 0.05. Result : Fe content in the ground water is below the normal threshold is <0.3 mg / L. While levels EYU school children who are under the optimal level of 5.9%, the optimal level of 32.4%, and the optimum levels above 61.8% (optimal: 100 - 199μg / L). Conclusion : There was no significant relationship between the Fe content in groundwater with levels of EYU in school children in District Panjatan Kulon Progo (p = 0.447).   Keywords: Fe, EYU


2018 ◽  
Author(s):  
Sang Hoon Lee ◽  
Jeff Blackwood ◽  
Stacey Stone ◽  
Michael Schmidt ◽  
Mark Williamson ◽  
...  

Abstract The cross-sectional and planar analysis of current generation 3D device structures can be analyzed using a single Focused Ion Beam (FIB) mill. This is achieved using a diagonal milling technique that exposes a multilayer planar surface as well as the cross-section. this provides image data allowing for an efficient method to monitor the fabrication process and find device design errors. This process saves tremendous sample-to-data time, decreasing it from days to hours while still providing precise defect and structure data.


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