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34333949 [Clinical study on the effect of etoposide-containing induction regimen for adult-onset St 2021 Aug 3 Objective: To investigate the efficacy of an etoposide-containing regimen in the treatment of adult-onset Still's disease related hemophagocytic syndrome(AOSD-HLH). Methods: This study adopted the method of retrospective analysis to collect clinical data of 43 AOSD-HLH patients, including the clinical characteristics, laboratory indexes, treatment regimen and prognosis. There were 7 males and 36 females, aged 24-40 years, with a median age of 30. All of them were diagnosed and treated in Beijing Friendship Hospital of Capital Medical University from December 2014 to December 2019. According to whether or not etoposide (VP-16) was included in the initial therapy, patients were divided into group 1 (VP-16 was not administrated in the initial treatment, n=31) and group 2 (the initial treatment included etoposide, n=12). Patients in group 1 who did not respond to the initial treatment were retreated with VP-16-containing regimen, and the effect of initial treatment was compared between the 2 groups. Similarly, according to whether the VP-16-containing regimen was applied or not, patients achieving remission of HLH were divided into group a (not applied, n=6) and group b (applied, n=33), and the laboratory indicators of the two groups were compared. Results: The overall response rate (ORR, 6/31 vs 11/12) and complete response rate (CRR, 1/31 vs 5/12) of patients in group 1 were significantly lower than those in group 2 (both P<0.05). Patients in group 1 who did not respond to the initial treatment were retreated with a VP-16-containing regimen, and we found that the ORR reached 22/24. Among patients in remission, the natural killer cell activity [16.3(14.2, 17.5)% vs 13.1(12.2, 13.8)%] and granulocyte counts [5.6(3.4, 9.3) ×10(9)/L vs 3.9(2.3, 4.7) ×10(9)/L] of patients was significantly higher in group B than that in group A(both P<0.05). There was no statistically significant difference in haemoglobin [103.0 (97.0, 109.5) g/L vs 91.5 (70.0, 118.0) g/L] and platelet counts [(212.2±74.2)×10(9)/L vs (226.0±114.9)×10(9)/L] between the two groups(both P>0.05). Conclusion: The remission status of HLH has an impact on the prognosis of patients. The use of VP-16 in initial treatment can significantly increase the ORR and CRR of AOSD-HLH patients. The application of VP-16 does not cause bone marrow suppression.
33886787 The role of platelet to mean platelet volume ratio in the identification of adult-onset st 2021 OBJECTIVES: Inflammatory factors exert a significant role in the development of adult-onset Still's disease (AOSD) and sepsis. Although platelet counts and platelet parameters have long served as indicators for inflammatory diseases, their role in the differential diagnosis between adult-onset stilĺs disease and sepsis remains unclear. We designed this retrospective study to explore whether the platelet to mean platelet volume (MPV) ratio (PMR) can help to distinguish AOSD from sepsis. METHODS: A total of 110 AOSD patients and 84 sepsis patients were enrolled in the study. Seventy-three AOSD patients and 56 sepsis patients between January 2010 and June 2017 were enrolled in the test cohort to analyze PMR values, which was then validated in the validation cohort (37 AOSD patients and 28 sepsis patients between June 2017 and December 2019). RESULTS: The values of PMR were significantly higher in AOSD patients than in sepsis patients (test cohort, validation cohort, and entire cohort), In the test cohort, logistic regression analysis showed that PMR was an independent risk factor of AOSD (odds ratios [OR]: 9.22, 95% confidence interval [CI] 2.15-39.46, p=0.003). Further receiver operating characteristic curve (ROC) analysis showed that the area under the ROC curve was 0.735 (95% CI 0.631-0.839, p<0.001) for PMR alone and 0.925 (95% CI 0.869-0.980, p<0.001) for the combination of PMR and serum ferritin. Consistently, the validation cohort exhibited analogous results. CONCLUSIONS: PMR could be used as a single indicator or a complementary indicator to distinguish AOSD from sepsis.
34907023 Genetic variants at the RTP4/MASP1 locus are associated with fatigue in Scandinavian patie 2021 Dec OBJECTIVES: Fatigue is common and severe in primary Sjögren's syndrome (pSS). The aim of this study was to identify genetic determinants of fatigue in pSS through a genome-wide association study. METHODS: Patients with pSS from Norway, Sweden, UK and USA with fatigue and genotype data available were included. After genotype imputation and quality control, 682 patients and 4 966 157 genetic markers were available. Association analysis in each cohort using linear regression with fatigue as a continuous variable and meta-analyses between the cohorts were performed. RESULTS: Meta-analysis of the Norwegian and Swedish cohorts identified five polymorphisms within the same linkage disequilibrium block at the receptor transporter protein 4 (RTP4)/MASP1 locus associated with fatigue with genome-wide significance (GWS) (p<5×10(-8)). Patients homozygous for the major allele scored 25 mm higher on the fatigue Visual Analogue Scale than patients homozygous for the minor allele. There were no variants associated with fatigue with GWS in meta-analyses of the US/UK cohorts, or all four cohorts. RTP4 expression in pSS B cells was upregulated and positively correlated with the type I interferon score. Expression quantitative trait loci effects in whole blood for fatigue-associated variants at RTP4/MASP1 and levels of RTP4 and MASP1 expression were identified. CONCLUSION: Genetic variations at RTP4/MASP1 are associated with fatigue in Scandinavian pSS patients. RTP4 encodes a Golgi chaperone that influences opioid pain receptor function and MASP1 is involved in complement activation. These results add evidence for genetic influence over fatigue in pSS.
34663713 Sjogren's syndrome associated with bilateral peripheral facial paralysis. 2021 Oct Sjogren's Syndrome (SS) is a chronic autoimmune disorder that may be complicated by neurological dysfunctions. The involvement of cranial nerves in SS was described as a very rare complication. Moreover, bilateral peripheral facial paralysis associated with SS has been described only in 3 patients in the literature and the first case was described by Henrik Sjogren himself in 1935. We report a 59-year-old female with bilateral peripheral facial paralysis associated with Sjogren's syndrome. She was treated with 5-day IVIG consecutively and continued oral methylprednisolone 16mg/day and almost fully recovered at 2 months of follow-up examination. Acute bilateral peripheral facial palsy in SS is a very rare condition and Lyme disease, Guillain-Barré syndrome, HIV infection, and central nervous system lymphoma should be considered in the differential diagnosis. As a result, SS should be considered as an underlying cause of bilateral facial paralysis.
34165270 Translation and validation of a Turkish version of the Xerostomia Inventory XI in patients 2021 Oct 21 BACKGROUND/AIM: The aim of this study was to assess the reliability and validity of Turkish version of the Xerostomia Inventory XI in patients with primary Sjögren’s syndrome (pSS). MATERIALS AND METHODS: A cross-sectional survey study design and analysis were used to assess the reliability and validity of the Xerostomia Inventory XI. A total of 69 patients with pSS (5 males, 64 females; mean age = 54.81 ± 8.77 years) were included. The Xerostomia Inventory XI (TR) was applied twice at an interval of 15 days. The test-retest reliability was assessed with the intraclass correlation coefficient (ICC), and the internal consistency of multiitem subscales by calculating Cronbach’s alpha values. The correlations between ESSPRI, basal and stimulated salivary flow (BSF-SSF), Oral Health Impact Profile-14 (OHIP-14) and Oral Health-Related Quality of Life-UK (OHRQoL-UK) Questionnaire were evaluated to determine the construct validity. RESULTS: The ICC value for test/retest reliability of the Xerostomia Inventory XI (TR) was 0.993. The internal consistency was 0.869. There were low to high correlations between Xerostomia Inventory XI (TR) and ESSPRI, BSF, SSF, OHIR-14 total and OHRQoL-UK total. CONCLUSION: The Turkish version of the Xerostomia Inventory XI was found to be clinically valid and reliable to be used in clinical evaluations and rehabilitation interventions in patients with pSS.
34781621 Acute Flaccid Quadriparesis Because of a Rare Systemic Cause. 2021 Nov Sjogren's syndrome is a chronic slowly progressive autoimmune disease characterized by lymphocytic infiltration of exocrine glands resulting in xerostomia and dry eyes. The syndrome has wide clinical spectrum from organ specific exocrionopathy to systemic manifestation. The disease can present alone or with other autoimmune diseases like RA, SLE, Scleroderma, autoimmune thyroid disease etc. Prevalence of primary Sjogren's is 0.5-1% and of secondary Sjogren's is 5-20%. Renal involvement is rare and can either be tubulointerstial or glomerular. Based on biopsy reports in the available literature, tubulointerstitial nephritis (TIN) is the most common histological abnormality, followed by glomerulonephritis as a distant second.1 Distal Renal tubular Acidosis is the most common manifestation of TIN. We report a case of a 35 year female with acute onset motor weakness (quadriparesis) with hypokalemia with NAGMA with distal RTA. Patient was diagnosed with Secondary Sjogren's and managed accordingly.
34254339 Efficacy of hydroxychloroquine for treating annular erythema associated with Sjögren's sy 2021 Oct Annular erythema is one of the cutaneous manifestations of Sjögren's syndrome (SS). Topical corticosteroids and tacrolimus, and oral corticosteroids, have been used as treatments for this condition. However, the safety and efficacy of these treatments remains unsatisfactory, and further development of therapies are desired. In this study, we performed a retrospective analysis of 16 annular erythema associated with SS (AESS) patients treated with hydroxychloroquine (HCQ). Disease activity was assessed using a modified version of the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI), which we termed the modified CLASI (m-CLASI). HCQ treatment improved AESS lesions in all 16 patients. The mean m-CLASI score was reduced by 85.6% at the 12-week follow-up relative to baseline (p < 0.01). Notably, 60% (6/10 cases) of patients with AESS lesions limited to the facial area achieved complete remission within 4 weeks. In the analysis of six patients who had taken oral prednisolone before starting HCQ, all were able to reduce the dose within 52 weeks without relapse. Particularly, 75% (3/4 cases) of patients with prednisolone dose of more than 5 mg/day could reduce their dose to less than 5 mg/day in combination with HCQ. For the safety concerns, two patients experienced grade 1 diarrhea during the 52-week observation period. However, neither serious adverse events nor adverse events requiring discontinuation of treatment occurred. The results of the present study suggest that HCQ may not only be highly effective as a single agent, but may also be useful as a steroid-sparing agent in refractory case requiring long-term steroid administration, making it a good treatment option for AESS.
34091740 Resilience in women with primary Sjögren's syndrome. 2021 Nov To assess the relationship between resilience and several diseases and individual features in primary Sjӧgren's Syndrome (SS) patients. Resilience was assessed using the Resilience Scale (RS-14). Disease activity, damage, and reported symptoms were assessed by means of ESSDAI (EULAR Sjögren's syndrome disease activity index), SSDDI (Sjӧgren's Syndrome Disease Damage Index) and ESSPRI (EULAR Sjӧgren's Syndrome Patient Reported Index). EuroQol, HADS (Hospital Anxiety and Depression Scale), SF-12 (Short-form 12 health survey), FAS (Fatigue Assessment Scale), FACIT-F (Functional Assessment of Chronic Illness Therapy - Fatigue), and IPAQ (International Physical Activity Questionnaire) questionnaires were submitted to evaluate physical and mental well-being of the recruited patients. Data about the autoimmune profile, systemic manifestations, and current therapy were collected. Educational qualifications and work activities were also considered. Descriptive, correlational, and linear regression analysis were performed. 74 consecutive women with primary SS and 74 sex and age-matched healthy subjects as a control group were recruited. SS patients displayed a moderate value of resilience (median 78.5) with no significant difference compared to controls (p = 0.38). An inverse relationship was found between resilience and mood disorders such as anxiety (p = 0.038) and depression (p < 0.001). Greater resilience was associated with a better perception of the quality of life (p = 0.02) and general health (p < 0.001), as well as with less fatigue (p = 0.008) and a more physically active lifestyle (p = 0.001). No significant relationship was found neither between resilience and age, socio-demographic and disease characteristics, nor with ESSDAI (p = 0.26), ESSPRI (p = 0.83) and SSDDI (p = 0.67). This is the first study assessing resilience in a large group of unselected primary SS patients. Most resilient primary SS patients are less depressed and show a better perception of their health. Greater resilience tends to correlate with less anxiety, physical and mental fatigue, and a more active lifestyle, while no association with disease activity and duration, damage, and socio-demographic features was detected. Considering the well-known role of resilience in helping to better cope with chronic illnesses, its assessment in SS patients should not be overlooked and the possible strategies for its improvement should be better explored and further implemented.
33841398 Peripheral Nervous System Involvement in Sjögren's Syndrome: Analysis of a Cohort From th 2021 PURPOSE: The prevalence of peripheral nervous system (PNS) involvement in primary Sjögren's syndrome (pSS) has been reported to range from 2% to over 50%. Bias in study designs, including low number of patients and unclearly defined rheumatological and neurological diagnosis could explain such variability. Consequently, the exact depiction of PNS involvement in pSS is still lacking. This study aimed at analyzing the prevalence and the clinical and laboratory factors associated with PNS involvement in a very large cohort of well-characterized pSS patients with a clearly defined neurological diagnosis. METHODS: Clinical and serological data of 1,695 pSS patients with specific and accurate information on PNS involvement were analyzed. Comparisons between patients with and without PNS involvement and between patients with distinct subsets of PNS manifestations were performed. RESULTS: Prevalence of PNS involvement was 3.7%. The most frequent types observed were pure sensory neuropathies and axonal sensorimotor polyneuropathies (SMP). Patients with PNS involvement exhibited a more active disease profile and were more frequently treated with immunosuppressants. Intriguingly, clinical and serological negative prognostic factors, including purpura, extra-glandular manifestations, leukopenia, low complement and cryoglobulinemia, principally characterized patients with SMP, while subjects with pure sensory neuropathy displayed a milder phenotype. CONCLUSION: Our results highlight that PNS involvement is rather rare, but prognostically relevant in pSS. Main adverse prognostic features characterize patients with SMP, while pure sensory neuropathies are usually associated with a mild clinical picture. These findings, useful for patient stratification, may suggest protean pathogenic pathways underlying different types of PNS manifestations in pSS.
34535869 The effectiveness of tocilizumab in treating refractory adult-onset Still's disease with d 2022 Feb OBJECTIVE: Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder with clinical heterogeneity. Although tocilizumab (TCZ), an interleukin (IL)-6 receptor inhibitor, is an effective treatment for AOSD, the evidence regarding its efficacy on systemic or articular subtypes is conflicting. Furthermore, the predictors of therapeutic response are still elusive and worthy of exploration. METHODS: This two-center retrospective study analyzed the effectiveness and safety profile of TCZ treatment in 28 patients with refractory AOSD. The 28-joint disease activity score (DAS28) and systemic activity score were assessed before and during TCZ treatment period at weeks 12, 24, 36, and 48. Plasma levels of proinflammatory cytokines at baseline were determined using ELISA method. RESULTS: Among the systemic subtype patients, 10 (58.8%), 13 (76.5%), 14 (82.4%), and 15 (88.2%) patients achieved complete remission at week 12, 24, 36, and 48, respectively, in comparison to 2 (22.2%), 5 (55.6%), 6 (66.7%), and 7 (77.8%) who achieved disease remission (DAS28 < 2.6) at weeks 12, 24, 36, and 48, respectively, among articular subtype patients. The systemic activity scores and inflammatory parameters were significantly decreased after 12-week TCZ therapy, and TCZ could significantly reduce corticosteroid dose in AOSD patients. Multivariate analysis reveals that baseline IL-18 level is a significant predictor of poor therapeutic response at week 24 (odds ratio 7.86, p < 0.05). CONCLUSION: AOSD patients refractory to high-dose corticosteroids and methotrexate may respond well to TCZ treatment with a steroid-sparing effect and an acceptable safety. A high baseline IL-18 level may be a predictor of poor therapeutic response. Key Points • Tocilizumab may be effective and well-tolerated in refractory AOSD patients regardless of disease subtypes. • High plasma levels of IL-18 may predict poor response to tocilizumab in AOSD patients.
34519280 Optimizing reproductive health management in lupus and Sjogren's syndrome. 2021 Nov 1 PURPOSE OF REVIEW: People with childbearing capacity who are diagnosed with systemic lupus erythematosus (SLE) and Sjogren's syndrome (SS) have specific and important reproductive health considerations. RECENT FINDINGS: Recommendations from the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) provide rheumatologists and other clinicians with guidance for reproductive health management of patients with rheumatic diseases. Patient-centered reproductive health counseling can help clinicians to operationalize the EULAR and ACR guidelines and enhance patient care. SUMMARY: Disease activity monitoring, risk factor stratification, and prescription of pregnancy-compatible medications during pregnancy help to anticipate complications and enhance pregnancy outcomes in SLE and SS. Assisted reproductive technologies are also safe among people with well-controlled disease. Safe and effective contraceptive methods are available for patients with SLE and SS, and pregnancy termination appears to be safe among these patients.
34332148 Tear metabolomics highlights new potential biomarkers for differentiating between Sjögren 2021 Oct PURPOSE: The lacrimal exocrinopathy of primary Sjögren's syndrome (pSS) is one of the main causes of severe dry eye syndrome and a burden for patients. Early recognition and treatment could prevent irreversible damage to lacrimal glands. The aim of this study was to find biomarkers in tears, using metabolomics and data mining approaches, in patients with newly-diagnosed pSS compared to other causes of dry eye syndrome. METHODS: A prospective cohort of 40 pSS and 40 non-pSS Sicca patients with dryness was explored through a standardized targeted metabolomic approach using liquid chromatography coupled with mass spectrometry. A metabolomic signature predictive of the pSS status was sought out using linear (logistic regression with elastic-net regularization) and non-linear (random forests) machine learning architectures, after splitting the studied population into training, validation and test sets. RESULTS: Among the 104 metabolites accurately measured in tears, we identified a discriminant signature composed of nine metabolites (two amino acids: serine, aspartate; one biogenic amine: dopamine; six lipids: Lysophosphatidylcholine C16:1, C18:1, C18:2, sphingomyelin C16:0 and C22:3, and the phoshatidylcholine diacyl PCaa C42:4), with robust performances (ROC-AUC = 0.83) for predicting the pSS status. Adjustment for age, sex and anti-SSA antibodies did not disrupt the link between the metabolomic signature and the pSS status. The non-lipidic components also remained specific for pSS regardless of the dryness severity. CONCLUSION: Our results reveal a metabolomic signature for tears that distinguishes pSS from other dry eye syndromes and further highlight nine key metabolites of potential interest for early diagnosis and therapeutics of pSS.
33389133 The use of Schirmer strips to measure salivary and lacrimal flow in non-Sjögren patients. 2021 Jun INTRODUCTION: Oral and ocular dryness are the most common symptoms reported during ophthalmological and dental examinations. It is becoming a serious and growing problem due to the huge variety of affecting factors and with population aging. OBJECTIVES: The purpose was to demonstrate an application of the Schirmer test for xerostomia. Subsequently, to compare and correlate the results achieved from the lacrimal Schirmer test and salivary Schirmer test in non-Sjögren patients. METHODS: Study group consisted of 642 patients with/without subjective or/and objective symptoms of dry eye or mouth who did not fulfill the criteria for diagnosis of Sjögren syndrome. The lacrimal Schirmer test (lST) and the salivary Schirmer tests (sST) were performed (sSTm was put on the floor of the mouth, sSTp in front of the parotid gland duct). The results were recorded after 1 min (sSTm), 3 min (sSTp), and 5 min (lST). RESULTS: The lST and sST test scores were considerably higher in the healthy group than in others, p < 0.001. The results of sST1 and sST2 decreased with the appearance of subjective and objective symptoms, p < 0.001. There were positive correlations between lST and sSTm outcomes between the groups, p < 0.001. CONCLUSIONS: We present the Schirmer test adapted to measure salivary gland hypofunction that is a time-saving tool in our daily practice. Results of this study reveal an excellent correlation between the eye Schirmer test and the salivary Schirmer tests. CLINICAL RELEVANCE: The salivary Schirmer tests seem to be rapid, convenient, and reliable objective screening tools for salivary gland hypofunction in non-Sjögren patients.
33496869 Differences in perceived intra-oral dryness in various dry-mouth patients as determined us 2021 Jun OBJECTIVES: Recently, it was shown that the Regional Oral Dryness Inventory (RODI) could determine differences in dry-mouth perception at different intra-oral locations. The main aim of this study was to determine whether the RODI might help to discriminate between various causes of oral dryness in dry-mouth patients. The second aim was to ascertain whether the RODI could become an additional diagnostic tool in dry-mouth patients. MATERIALS AND METHODS: Data were collected retrospectively from patients who visited a specialized saliva clinic. Salivary flow rates, Xerostomia Inventory scores, and RODI scores were extracted from the medical records. Patients were stratified into subgroups according to their health status. RESULTS: Five hundred twenty-eight patients participated in this study (mean age of 59.6 ± 16.0 years; 68.4% female). Specific patient groups differed with regard to the region of the mouth they experienced as the most and least dry. The posterior palate was the area perceived as most dry by controls and Sjögren patients. In patients using limited or multiple medications, it was the anterior tongue. RODI scores also differed significantly among dry-mouth patient groups: whereas controls and patients using limited medication had the lowest RODI scores and experienced less intra-oral dryness, Sjögren patients had the highest RODI scores. CONCLUSION: Our use of the RODI questionnaire showed that perceived intra-oral dryness differed between the various dry-mouth patients. CLINICAL RELEVANCE: The RODI can be a valuable clinical diagnostic tool in dry-mouth diagnostics, in which it can be used to discriminate between the various causes of oral dryness in patients.
34796855 Differences in presentation between paediatric- and adult-onset primary Sjögren's syndrom 2021 Nov OBJECTIVES: Primary Sjögren's syndrome (pSS) is a rare disease in paediatric patients. Presenting symptoms differ from those in adult patients. The aim of this study was to evaluate presenting symptoms, classification criteria and clinical assessments, including salivary gland ultrasonography (SGUS), at disease onset in paediatric and adult patients with pSS. METHODS: Data of 23 paediatric- and 33 adult-onset patients with pSS were obtained from our standardised multidisciplinary REpSULT and RESULT cohorts, respectively. Clinical, patient-reported, serological, functional, biopsy and SGUS parameters were compared. RESULTS: In paediatric-onset pSS (pedSS) patients, recurrent parotid gland swelling (91% vs. 49%, p<0.001) and fever (30% vs. 3%, p=0.006) were more often present than in adult-onset patients. In contrast, sicca symptoms of mouth (52% vs. 79%, p=0.046) and eyes (26% vs. 73%, p<0.001) were less common in pedSS patients. In paediatric patients, the entry criteria of the ACR/EULAR classification were most often met due to activity in the glandular domain of the ESSDAI. When applying the ACR/EULAR classification criteria, only 78% of pedSS fulfilled these criteria compared to 100% of adult patients. Abnormal glandular function tests had a greater contribution to fulfilling the criteria in adults, while the biopsy had a greater contribution in paediatric patients. Anti-SSA/Ro serology had similar contribution for both cohorts. SGUS Hocevar score was significantly higher in paediatric compared to adult patients (median 25 vs. 18, p=0.004). CONCLUSION: PedSS has a different presentation than adult-onset pSS. Recurrent parotid gland swelling in paediatric patients should alert clinicians to the potential presence of pSS.
34235806 The role of lactate dehydrogenase-to-albumin ratio in clinical evaluation of adult-onset S 2021 Oct OBJECTIVES: The aim of the study is to evaluate the importance of lactate dehydrogenase-to-albumin ratio (LAR) in patients with adult-onset Still's disease (AOSD) and to investigate the relationship between this ratio and clinical laboratory variables. METHODS: The study design was retrospective cross-sectional. The demographic and clinical data, laboratory results and imaging findings were documented. Univariate and multinomial logistic regression analyses were performed to find the predictors, which could be useful to define the ferritin level and organ involvement. Receiver-operating characteristic (ROC) analysis was used to clarify the diagnostic ability of the LAR for ferritin level and organ involvement. RESULTS: Fifty-eight patients with AOSD were evaluated. When patients were divided into two groups according to the serum ferritin level with a cut-off of 1500 ng/dL, lymphocyte count and albumin level were significantly less in patients who had higher ferritin levels (P = .015 and P = .005). In multinominal logistic regression analysis, AST, LDH and LAR were found as predictors for ferritin levels. When we compared LAR between patients with and without organ involvement, higher LAR was found in patients with HM, SM, serositis and MAS. Also, LAR was significantly higher in patients with higher ferritin levels (≥1500 ng/dL) than those without (P < .001). In ROC analysis, the cut-off point of LAR predicting the ferritin level was determined as 80.75 with 83.7% sensitivity and 80.0% specificity (AUC = 0.89, 95% CI 0.79-0.98, P < .001). CONCLUSION: LAR can be a valuable inflammatory marker to determine AOSD patients with organ involvement and higher ferritin level.
33943020 Inhibitory effect of HTLV-1 infection on the production of B-cell activating factors in es 2021 Sep INTRODUCTION: The low frequency of ectopic germinal center in labial salivary glands of patients with human T-cell leukemia virus type 1 (HTLV-1) antibody-positive Sjögren's syndrome (SS) suggests that HTLV-1 has some effects on follicular dendritic cells (FDCs). METHODS: We used flow cytometry, immunofluorescence, and enzyme-linked immunosorbent assays (ELISAs) to investigate the direct effect of HTLV-1 on B-cell activating factors produced by established FDC like cells obtained from excised human tonsils. We then measured the serum B-cell activating factor (BAFF) and C-X-C motif ligand (CXCL) 13 concentrations of the HTLV-1-seropositive SS patients and the HTLV-1-seronegative SS patients by ELISA. RESULTS: Among the 31 isolated specimens, 22 showed morphological characteristics of FDCs. Day 2-cultured specimens showed expressions of CD14, CD23, CD40, intracellular adhesion molecule-1 (ICAM-1), and vascular cell adhesion molecule-1. After 2 weeks, 12 of these specimens expressed ICAM-1, FDC, and fibroblast cell marker. Intracellular BAFF and CXCL13 were constitutively expressed regardless of stimulation. After direct coculture with the HTLV-1-infected T-cell line HCT-5 or MT-2, the BAFF and CXCL13 expressions on the FDC-like cells were decreased in accord with the increased number of HCT-5 and MT-2 cells with styliform change and without HTLV-1 Gag protein expression. Interferons upregulated the concentration of BAFF (but not CXCL13) in the culture supernatant, which showed a declining trend under the presence of HCT-5 or MT-2. The serum concentrations of BAFF and CXCL13 in the HTLV-1-seropositive SS patients were lower than those of the HTLV-1 seronegative SS patients. CONCLUSIONS: HTLV-1 partially inhibited the BAFF and CXCL13 expressions of established FDC-like cells.
32689841 Characterizing Microbiota from Sjögren's Syndrome Patients. 2021 Jul OBJECTIVE: To compare the oral microbiota of Sjögren's syndrome (SS) with that of healthy subjects (HS). METHODS: Supragingival and subgingival biofilm samples were collected from the mesial-buccal tooth surfaces of SS patients (n = 57) and age- and sex-matched HS (n = 53). Unstimulated saliva and 8 oral tissue samples were taken using a buccal brush. Caries and periodontal measures were recorded. All supragingival samples and a subgroup of 24 SS and 28 HS subgingival samples, as well as 32 SS and 11 HS saliva and oral tissue samples, were analyzed for their content of 41 bacterial species using checkerboard DNA-DNA hybridization. Mean levels (×10(5) ± SEM) and percentage of DNA probe counts of each species were determined for each sample site and averaged within subjects in the 2 clinical groups. Kruskal-Wallis tests, adjusting for multiple comparisons and cluster analysis, were used for soft tissue and microbial analysis, and the Mann-Whitney test was used to compare caries and periodontal measures. RESULTS: Mean (×10(5) ± SEM) total DNA probe counts in supragingival samples were significantly lower (P < 0.001) in the SS (13.3 ± .7) compared to the HS (44.1 ± 6.8) group. In supragingival samples, Veillonella parvula, Fusobacterium nucleatum ss vincenti, and Propionibacterium acnes were markedly elevated in the SS compared to the HS group in both mean (×10(5) ± SEM) and mean (± SEM) percentage DNA probe counts (P < 0.001). In subgingival samples of SS, V. parvula was significantly different compared to HS (P < 0.05). SS was characterized by high levels of purple and low levels of orange and red complexes. Cluster analysis of oral tissues and saliva demonstrated that the mean microbial profiles for SS patients and the HS group clustered separately. Active root caries (P < 0.003) and attachment loss were significantly higher (P < 0.029) in the SS group compared to the HS group. CONCLUSION: These findings indicate that saliva is a major controlling factor of intraoral biofilm. V. parvula may be a unique microbial biomarker for Sjögren's syndrome. KNOWLEDGE TRANSFER STATEMENT: The microbiome characterized for Sjögren's syndrome in salivary hypofunction is shown to be under stress and reduced. Veillonella parvula can be a possible identification of a biomarker for Sjögren's syndrome.
33181176 Nerolidol-beta-cyclodextrin inclusion complex enhances anti-inflammatory activity in arthr 2021 Jan 15 Rheumatoid arthritis is an autoimmune inflammatory disease with progressive degradation of cartilage and joints. Additionally, gastric ulcer affects many patients who make prolonged use of non-steroidal anti-inflammatory drugs widely used in the symptomatic treatment of rheumatoid arthritis. Nerolidol, a natural sesquiterpene, has several biological activities including anti-inflammatory and antiulcerogenic action. This study aims to develop and characterize a nerolidol ß-cyclodextrin inclusion complex and to evaluate its activity in an experimental arthritis model. Inclusion complex was prepared by the lyophilization method and characterized by NMR, term analysis, XRD and SEM. Neutrophil migration assays and histopathological analysis were performed on zymosan-induced arthritis model using Swiss mice. And the gastroprotective effect was evaluated in two models of gastric ulcers: induced by ethanol and indomethacin. Inclusion complex showed no cytotoxicity and free nerolidol at a dose of 100 mg/kg (p.o.) in the arthritis model reduced neutrophil migration in 56% in relation to vehicle, and this inhibition was more expressive in the inclusion complex (67%) at the same dose. Histopathological analysis of the joint tissue confirmed the reduction of inflammatory signs. In the ethanol-induced gastric ulcer model, free nerolidol reduced the relative ulcer area more expressively (4.64%) than the inclusion complex (21.3%). However, in the indomethacin induction model, the inclusion complex showed better results in gastric protection compared to free nerolidol. The action of nerolidol complexed in beta-cyclodextrin in reducing arthritis inflammation combined with its gastroprotective action make it a potential new drug.
34966506 Pigmented Villonodular Synovitis (PVNS) of the Knee mimicking Septic Arthritis in a Paedia 2021 Nov Pigmented villonodular synovitis (PVNS) is a benign but rare proliferative disorder of the synovium. It commonly occurs in the adult population and usually presents as a monoarticular disease. There are two types of PVNS, namely the localised and diffused type. The disease is often misdiagnosed due to its rarity especially in paediatric patients. Knee involvement in PVNS is the commonest form in children although other joints such as hip, foot, ankle, hip, sacroiliac joint and concurrent multiple joint involvements have also been reported. PVNS in paediatric patients is often misdiagnosed as septic arthritis, juvenile rheumatoid arthritis and bone sarcoma, and the diagnosis is usually often made late due to its vague presentation. The majority of PVNS cases are managed by surgery either via open or arthroscopic synovectomy except in a few paediatric patients as described in the literature. This case report of PVNS is of a knee in 11-year-old boy who was initially treated as septic arthritis. The synovium appearance mimicked the features of PVNS during a knee arthrotomy washout, and histopathological examination confirmed the diagnosis. The knee symptoms had significantly improved without additional surgery, and good functional knee motion was achieved, with no sign of recurrence, after two years of follow-up.