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ID PMID Title PublicationDate abstract
34434435 Hepatic Hodgkin Lymphoma Presenting as Solitary Hepatic Mass Following Other Iatrogenic Im 2021 Feb Lymphoproliferative disorders (LPDs) occur frequently in patients with rheumatoid arthritis (RA) under methotrexate treatment. Some LPDs spontaneously regressed after methotrexate discontinuation, but classic Hodgkin lymphoma (CHL)-type LPDs frequently relapse, and chemotherapy is usually required for the treatment. CHL usually spreads in contiguous lymph nodes and then infiltrates in organs at an advanced stage. Thus, hepatic Hodgkin lymphoma (HHL) without lymphadenopathy is extremely rare at diagnosis. We present a case of methotrexate-associated LPDs associated with systemic lymphadenopathy and hepatosplenic mass in a 71-year-old woman with RA under methotrexate treatment over 10 years. Although spontaneous remission occurred after methotrexate discontinuation, she developed HHL presenting as a solitary hepatic mass without lymphadenopathy 3 years after spontaneous regression. She received brentuximab vedotin (BV) combination chemotherapy without bleomycin to avoid pulmonary toxicity. Complete metabolic response was achieved after four courses of BV combination chemotherapy, and the activity of RA was kept to be in remission. Our case suggested that the recurrence lesions of LPDs may present at unexpected site, which is not coincide with the primary site, and BV combination chemotherapy is a promising regimen for limited-stage CHL-type LPDs in patients with RA owing to its anti-lymphoma effect on CHL-type LPDs and a possible targeted therapy for RA.
32472461 COVID-19, chronic inflammatory rheumatic disease and anti-rheumatic treatments. 2020 Jul Since December 2019, the pandemic caused by coronavirus disease 2019 (COVID-19) raises a real public health problem. COVID-19 appeared in Wuhan (Hubei province) in China. Drugs that have been used in rheumatology for decades seem to be effective in this infection and are for the most part being studied. The rational use of these anti-rheumatic drugs is based on the cytokinic storm (hyperproduction of IL1, IL6, TNF α) in the body by COVID-19 in its severe form. In this review, the authors make the difference between the infectious and auto-inflammatory part of COVID-19; the disease does not seem to be a risk factor for admission to the intensive care unit for patients suffering from inflammatory rheumatism; however, the poverty of studies on this subject should be noted. The authors also review anti-rheumatic drugs while studying COVID-19 treatment.
32545708 Urinary Dimethylamine (DMA) and Its Precursor Asymmetric Dimethylarginine (ADMA) in Clinic 2020 Jun 12 Asymmetric protein-arginine dimethylation is a major post-translational modification (PTM) catalyzed by protein-arginine methyltransferase (PRMT). Regular proteolysis releases asymmetric dimethylarginine (ADMA). Of the daily produced ADMA, about 10% are excreted unchanged in the urine. The remaining 90% are hydrolyzed by dimethylarginine dimethylaminohydrolase (DDAH) to L-citrulline and dimethylamine (DMA), which is readily excreted in the urine. The PRMT/DDAH pathway is almost the exclusive origin of urinary ADMA and the major source of urinary DMA. Dietary fish and seafood represent additional abundant sources of urinary DMA. The present article provides an overview of urinary ADMA and DMA reported thus far in epidemiological, clinical and pharmacological studies, in connection with the L-arginine/nitric oxide (NO) pathway and beyond, in neonates, children and adolescents, young and elderly subjects, males and females. Discussed diseases mainly include those relating to the renal and cardiovascular systems such as peripheral arterial occlusive disease, coronary artery disease, chronic kidney disease, rheumatoid arthritis, Becker muscular disease, Duchenne muscular disease (DMD), attention deficit hyperactivity disorder (ADHD), and type I diabetes. Under standardized conditions involving the abstinence of DMA-rich fresh and canned fish and seafood, urinary DMA and ADMA are useful as measures of whole-body asymmetric arginine-dimethylation in health and disease. The creatinine-corrected excretion rates of DMA range from 10 to 80 µmol/mmol in adults and up to 400 µmol/mmol in children and adolescents. The creatinine-corrected excretion rates of ADMA are on average 10 times lower. In general, diseases are associated with higher urinary DMA and ADMA excretion rates, and pharmacological treatment, such as with steroids and creatine (in DMD), decreases their excretion rates, which may be accompanied by a decreased urinary excretion of nitrate, the major metabolite of NO. In healthy subjects and in rheumatoid arthritis patients, the urinary excretion rate of DMA correlates positively with the excretion rate of dihydroxyphenylglycol (DHPG), the major urinary catecholamines metabolite, suggesting a potential interplay in the PRMT/DDAH/NO pathway.
32015751 Deciphering the Active Ingredients and Molecular Mechanisms of Tripterygium hypoglaucum (L 2020 Tripterygium hypoglaucum (Levl.) Hutch (THH) shows well clinical effect on rheumatoid arthritis (RA), but the active ingredients and molecular mechanisms remain unclear. This work was designed to explore these issues by network pharmacology. Compounds from THH were gathered by retrieving literatures. Compound-related and RA-related genes were identified using databases, and the overlapping genes were identified by Venn diagram. The active ingredients and genes of THH against RA were confirmed by dissecting interactions between overlapping genes and compounds using Cytoscape. SystemsDock website was used to further verify the combining degree of key genes with active ingredients. Pathway enrichment analysis was performed to decipher the mechanisms of THH against RA by Database for Annotation, Visualization and Integrated Discovery. A total of 123 compounds were collected, and 110 compounds-related and 1871 RA-related genes were identified, including 64 overlapping genes. The target genes and active ingredients of THH against RA comprised 64 genes and 17 compounds, the focus of which was PTGS2, triptolide, and celastrol. SystemsDock website indicated that the combing degree of PTGS2 with triptolide or celastrol was very good. The mechanisms of THH against RA were linked to 31 signaling pathways, and the key mechanism was related to inhibition of inflammation response through inactivating TNF and NF-kappa B signaling pathways. This work firstly explored the active ingredients and mechanisms of THH against RA by network pharmacology and provided evidence to support clinical effects of THH on RA.
31879042 Endoplasmic reticulum stress in autoimmune diseases. 2020 Mar If the body's immune system is disordered and begins to attack "self" and therefore, its own tissues this is considered to be an autoimmune pathology. The specific mechanisms vary between the different diseases and have not always been elucidated but chronic, non-resolving inflammation is a common theme in the pathogenesis of autoimmune diseases. Interestingly, it has been shown that development and occurrence of various inflammatory responses are closely correlated to endoplasmic reticulum stress. Therefore, this review discusses the current progress of research about the relationship between autoimmune diseases and endoplasmic reticulum stress, specifically the unfolded protein response (UPR).
33318970 Influenza H1N1 virus-associated pneumonia often resembles rapidly progressive interstitial 2020 PURPOSE: To describe computed tomography (CT) findings of influenza H1N1 virus-associated pneumonia (IH1N1VAP), and to correlate CT findings to pathological ones. METHODS: The study included 24 patients with IH1N1VAP. Two observers independently evaluated the presence, distribution, and extent of CT findings. CT features were divided into either classical form (C-form) or non-classical form (NC-form). C-form included: A.) broncho-bronchiolitis and bronchopneumonia type, whereas NC-forms included: B.) diffuse peribronchovascular type, simulating subacute rheumatoid arthritis-associated (RA) interstitial lung disease (ILD) and C.) lower peripheral and/or peribronchovascular type, resembling dermatomyositis-associated ILD and COVID-19 pneumonia. In 10 cases with IH1N1VAP where lung biopsy was performed, CT and pathology findings were correlated. RESULTS: The most common CT findings were ground-glass opacities (24/24, 100 %) and airspace consolidation (23/24, 96 %). C-form was found in 11 (46 %) patients while NC-form in 13 (54 %). Types A, B, and C were seen in 11(46 %), 4 (17 %), and 9 (38 %) patients, respectively. The lung biopsy revealed organizing pneumonia in all patients and 6 patients (60 %) showed incorporated type organizing pneumonia that was common histological findings of rapidly progressive ILD. CONCLUSION: In almost half of patients of IH1N1VAP, CT images show NC-form pneumonia pattern resembling either acute or subacute RA or dermatomyositis-associated ILD and COVID-19 pneumonia.
32532019 Folate Receptor β (FRβ) Expression in Tissue-Resident and Tumor-Associated Macrophages A 2020 Jun 10 As macrophages exhibit a huge functional plasticity under homeostasis and pathological conditions, they have become a therapeutic target for chronic inflammatory diseases. Hence, the identification of macrophage subset-specific markers is a requisite for the development of macrophage-directed therapeutic interventions. In this regard, the macrophage-specific Folate Receptor β (FRβ, encoded by the FOLR2 gene) has been already validated as a target for molecular delivery in cancer as well as in macrophage-targeting therapeutic strategies for chronic inflammatory pathologies. We now show that the transcriptome of human macrophages from healthy and inflamed tissues (tumor; rheumatoid arthritis, RA) share a significant over-representation of the "anti-inflammatory gene set", which defines the gene profile of M-CSF-dependent IL-10-producing human macrophages (M-MØ). More specifically, FOLR2 expression has been found to strongly correlate with the expression of M-MØ-specific genes in tissue-resident macrophages, tumor-associated macrophages (TAM) and macrophages from inflamed synovium, and also correlates with the presence of the PU.1 transcription factor. In fact, PU.1-binding elements are found upstream of the first exon of FOLR2 and most M-MØ-specific- and TAM-specific genes. The functional relevance of PU.1 binding was demonstrated through analysis of the proximal regulatory region of the FOLR2 gene, whose activity was dependent on a cluster of PU.1-binding sequences. Further, siRNA-mediated knockdown established the importance of PU.1 for FOLR2 gene expression in myeloid cells. Therefore, we provide evidence that FRβ marks tissue-resident macrophages as well as macrophages within inflamed tissues, and its expression is dependent on PU.1.
32919253 Interleukin-15 in autoimmunity. 2020 Dec Interleukin-15 (IL-15) is a member of the IL-2 family of cytokines, which use receptor complexes containing the common gamma (γ(c)) chain for signaling. IL-15 plays important roles in innate and adaptative immune responses and is implicated in the pathogenesis of several immune diseases. The IL-15 receptor consists of 3 subunits namely, the ligand-binding IL-15Rα chain, the β chain (also used by IL-2) and the γ(c) chain. IL-15 uses a unique signaling pathway whereby IL-15 associates with IL-15Rα during biosynthesis, and this complex is 'trans-presented' to responder cells that expresses the IL-2/15Rβγ(c) receptor complex. IL-15 is subject to post-transcriptional and post-translational regulation, and evidence also suggests that IL-15 cis-signaling can occur under certain conditions. IL-15 has been implicated in the pathology of various autoimmune diseases such as rheumatoid arthritis, autoimmune diabetes, inflammatory bowel disease, coeliac disease and psoriasis. Studies with pre-clinical models have shown the beneficial effects of targeting IL-15 signaling in autoimmunity. Unlike therapies targeting other cytokines, anti-IL-15 therapies have not yet been successful in humans. We discuss the complexities of IL-15 signaling in autoimmunity and explore potential immunotherapeutic approaches to target the IL-15 signaling pathway.
32141836 Perceived Treatment Satisfaction and Effectiveness Facilitators Among Patients With Chroni 2020 Mar 6 BACKGROUND: Approximately 50% of patients are nonadherent to prescribed medications. Patient perception regarding medication effectiveness has been linked to improved adherence. However, how patients perceive effectiveness is poorly understood. OBJECTIVE: The aim of this study was to elucidate factors associated with perceived treatment satisfaction and effectiveness among patients with chronic health conditions. METHODS: We conducted a descriptive study using a cross-sectional survey design. We administered a Web-based survey to participants with migraine, multiple sclerosis (MS), or rheumatoid arthritis (RA). Patients were recruited from established online communities of Health Union. Descriptive statistics, correlations, and comparison tests were used to examine outcomes. RESULTS: Data were collected from 1820 patients: 567 with migraine, 717 with MS, and 536 with RA. The majority of participants were female (1644/1820, 90.33%), >40 years old (1462/1820, 80.33%), and diagnosed >5 years ago (1189/1820, 65.33%). Treatment satisfaction and perceived medication effectiveness were highly correlated (r=0.90, P<.01). Overall, three temporal factors were positively correlated with satisfaction or perceived effectiveness: time on current medication (satisfaction r(s)=0.22, P<.01; effectiveness r(s)=0.25, P<.01), time since diagnosis (satisfaction r(s)=0.07, P<.01; effectiveness r(s)=0.09, P<.01), and time on treatment (effectiveness r(s)=0.08, P<.01). CONCLUSIONS: Findings validated the strong relationship between treatment satisfaction and perceived effectiveness. Understanding the (1) positive relationship between time and treatment satisfaction and effectiveness and (2) factors associated with determining medication effectiveness can help clinicians better understand the mindset of patients regarding treatment. Clinicians may be better prepared to elicit patient beliefs, which influence medication adherence, for people diagnosed with chronic health conditions.
32091008 Detecting Migration and Infiltration of Neutrophils in Mice. 2020 Feb 6 Neutrophils are a major member of the innate immune system and play pivotal roles in host defense against pathogens and pathologic inflammatory reactions. Neutrophils can be recruited to inflammation sites via the guidance of cytokines and chemokines. Overwhelming infiltration of neutrophils can lead to indiscriminate tissue damage, such as in rheumatoid arthritis (RA). Neutrophils isolated from peritoneal exudate respond to a defined chemoattractant, N-formyl-Met-Leu-Phe (fMLP), in vitro in Transwell or Zigmond chamber assays. The air pouch experiment can be used to evaluate the chemotaxis of neutrophils towards lipopolysaccharide (LPS) in vivo. The adjuvant-induced arthritis (AA) mouse model is frequently used in RA research, and immunohistochemical staining of joint sections with anti-myeloperoxidase (MPO) or anti-neutrophil elastase (NE) antibodies is a well-established method to measure neutrophil infiltration. These methods can be used to discover promising therapies targeting neutrophil migration.
31325552 Comorbid diseases of vitiligo: A 10-year cross-sectional retrospective study of an urban U 2020 Mar BACKGROUND: Vitiligo is associated with medical conditions, primarily autoimmune disorders; however, only a few studies in the United States have investigated these associations. OBJECTIVE: Our purpose was to investigate the diseases associated with vitiligo in the New York, New York, population and evaluate if these associations differ by race/ethnicity and sex. METHODS: In this retrospective study, we analyzed data collected from the medical records of 1487 vitiligo patients seen at New York University during a 10-year period. RESULTS: Vitiligo patients had a statistically significant higher prevalence of hypothyroidism, multiple sclerosis, rheumatoid arthritis, idiopathic thrombocytopenic purpura, seronegative arthritis, pernicious anemia, myasthenia gravis, inflammatory bowel disease, lymphoma, and systemic lupus erythematosus. Rates of comorbid autoimmune diseases varied by race and sex. LIMITATIONS: Medical charts did not consistently report race/ethnicity, type of vitiligo, and total body surface area affected. Information from nondermatology medical visits was also included. CONCLUSION: This study revealed multiple new disease associations for vitiligo, including multiple sclerosis, idiopathic thrombocytopenic purpura, and lymphoma, as well as confirmed previously reported associations with other autoimmune diseases, the most common being hypothyroidism followed by rheumatoid arthritis. Associations did vary by race/ethnicity and sex.
33193430 Impact of Janus Kinase Inhibition on the Treatment of Axial Spondyloarthropathies. 2020 Many immune cells and effector molecules (e.g. cytokines, Interferons, growth factors) utilize different combinations of Janus kinase (JAK) and signal transducer and activator of transcription (STAT) molecules to transduce signals from the cell surface to the nucleus, where they regulate transcription. This pathway is basically involved in almost all inflammatory diseases and also in the interleukin (IL)-23/IL-17 cascade, which is an essential part of the pathogenesis of spondyloarthropathies (SpA). Upon evidence from in vitro and in vivo experiments indicating disease-modifying effects of JAK inhibition in inflammatory joint disease, numerous inhibitors of the JAK/STAT pathway (= JAKinibs) with different selectivity against the four members of the JAK family [JAK1, JAK2, JAK3, and tyrosine kinase 2 (TYK2)] were developed. Trials in rheumatoid arthritis were successful with respect to efficacy and safety, and currently, three JAKinibs are approved for the treatment of rheumatoid arthritis in the European Union. Although new treatment options (anti-IL-23, anti-IL-17, and phosphodiesterase 4 inhibitors) have become available for spondyloarthritis and especially psoriatic arthritis (PsA) within the last years, most of them are biologics and do not address all disease manifestations equally. Therefore, multiple trials were initiated to evaluate JAKinibs in PsA and axial spondyloarthritis (axSpA). A trial of Tofacitinib (OPAL) was successful in PsA and has led to the inclusion of JAKinibs into the treatment algorithm. Currently many trials with JAKinibs are ongoing for PsA and axSpA, with one phase III trial of upadacitinib (selective JAK1 inhibitor) showing good therapeutic response in active radiographic axSpA.
32874480 Hyperbaric oxygen in animal model of rheumatoid arthritis: Analysis Of HIF-1α, ACPA and I 2020 Jul 7 Antigen and collagen-induced arthritis (ACIA) is animal model of rheumatoid arthritis. The aim of this study was to identify the effect of different doses of hyperbaric oxygen (HBO) exposure in reducing inflammation on ACIA through analysis hypoxia inducible factor-1α (HIF-1α), anticyclic citrullinated peptide antibody (ACPA) and interleukine 17a (IL-17a). 24 male Balb/C mice were divided into 3 groups, 8 mice did not receive HBO exposure as a control group (G1) and 16 mice received HBO exposure as treatment group (G2 and G3). G2 was ACIA which was exposed to HBO 2.4 ATA O(2) 100% 90 minutes divided by 3 each 30 minutes intervals 2 times 5 minutes breathing with normal air for 10 consecutive days. G3 was ACIA which was exposed to HBO 2.4 ATA O(2) 100% 90 minutes divided by 3 each 30 minutes intervals 2 times 5 minutes breathing with normal air for 5 consecutive days, break 5 days, 5 consecutive days.ACPA and IL-17a were measured by enzyme-linked immunosorbent assay (ELISA) technique. The expression of HIF-1α was measured by immunohistochemistry technique. There was significant decrease of ACPA levels, IL-17a levels and HIF-1α expression (P< 0.05) in G2 and G3 compared to G1. There was not significant decrease of ACPA levels (P> 0.05), there was significant decrease of IL-17a levels and HIF-1α expression (P<0.05) in G2 compared to G3. G2 was a group with a higher oxygen partial pressure than G3. HBO 2.4 ATAO(2) 100% 90 minutes divided by 3 each 30 minutes intervals 2 times 5 minutes breathing with normal air for 10 consecutive days more effective in reducing inflammation than exposure 5 consecutive days, break 5 days, 5 consecutive days in ACIA. HBO has therapeutic potential for the treatment of RA.
30794003 Association of anti-cyclic citrullinated peptide antibody with clinical features in patien 2020 Mar Background: Although anti-cyclic citrullinated peptide antibody (anti-CCP Ab) is reported to be found in 5-20% of patients with psoriatic arthritis (PsA), its clinical significance has not been elucidated.Objective: To clarify the association of anti-CCP Ab with clinical features in PsA.Methods: Patients were enrolled who fulfilled the classification criteria for psoriatic arthritis (CASPAR) criteria and visited our hospital. We retrospectively compared clinical characteristics between those who were positive and negative for anti-CCP Ab and further compared changes in disease activity in the patients treated with biological disease-modifying anti-rheumatic drugs (DMARDs).Results: We examined 41 patients (11 females), seven were anti-CCP Ab-positive and 34 were negative. Age (55.0 ± 15.1 years old) and frequency of lung involvements (71.4%) in the anti-CCP Ab-positive group were significantly higher than those (40.0 ± 16.0 and 0%, respectively) in the negative group (p  <  .05). Rheumatoid factor (RF) titer (749.4 ± 860.7 U/mL) and MMP-3 (604.8 ± 1060.6) in the anti-CCP Ab-positive group was significantly higher than that (3.6 ± 4.4 U/mL and 111.2 ± 77.4, respectively) in the negative group (p  <  .05). Five patients were treated with tumor necrosis factor (TNF) inhibitors (infliximab (IFX): 3 and adalimumab (ADA): 2) in the anti-CCP Ab-positive group, while in the negative group there were 11 (IFX: 6, ADA: 4, and etanercept (ETN): 1). Within 6 months of treatment, arthritis did not improve with TNF inhibitors in the anti-CCP Ab-positive group, whereas it improved significantly in the negative group.Conclusion: In patients with PsA, anti-CCP Ab might be related to lung involvements, elderly onset, RF and MMP-3 titers, and resistance to TNF inhibitor.
32693559 Mitochondria disease due to humidifier disinfectants: diagnostic criteria and its evidence 2020 Jun Humidifier disinfectant damages caused by the misuse of humidifier disinfects, such as polyhexamethylene guanidine (PHMG), resulted in chemical disasters in South Korea in 2011. About four million people were exposed to humidifier disinfectants (HDs) in the 17 years between 1994 and 2011. Although fatal lung damage was initially reported, investigations into the victims' injuries revealed that the damage was not limited to the lungs, but that systemic damage was also confirmed. Considering the spread of HD from the lungs to the whole body, the toxic effects of PHMG from reactive oxygen species (ROS), NOTCH signaling pathways, and mitochondrial dysfunction resulted in endothelial damage in the lungs, blood vessels, liver, kidneys, bone marrow, nerves, and muscles. The main toxic mechanisms involved in HD damage may be the NOTCH pathway and mitochondrial damage. There are many case reports which include neurologic disorders (ADHD, depression, posttraumatic stress disorder), muscular disorder (exercise intolerance, myalgia), energy metabolism disorder (chronic fatigue syndrome), and immunologic disorder (rheumatoid arthritis) in HDs victims. These case reports involve multi-system involvement in HDs victims. Further well-designed study is needed to clarify whether mitochondrial dysfunction is associated with multi-organs involvement in HDs victims.
31644467 Common and rare forms of vasculitis associated with Sjögren's syndrome. 2020 Jan PURPOSE OF REVIEW: Although uncommon, systemic vasculitis is one of the most severe extraglandular manifestations of primary Sjögren's syndrome (pSS) accounting for the increased morbidity and mortality of the disease. This review aims to describe major previous and recent reports regarding the clinical presentation, prognosis and treatment of systemic vasculitis associated with pSS. RECENT FINDINGS: Both older and recent pSS cohort studies performed over the past several and recent years, have clearly shown that cryoglobulinaemic vasculitis is the most frequent type of systemic vasculitis accompanying pSS. Antineutrophil cytoplasmic antibody-associated, large and medium vessel vasculitis are described only in sporadic cases. In addition to the overt clinical manifestations of cryoglobulinaemic vasculitis, type II cryoglobulinaemia, glomerulonephritis and purpura have been correlated with increased risk for B-cell non-Hodgkin lymphoma (NHL) in pSS. SUMMARY: pSS is characterized by autoreactive B and T-cell infiltrates around the epithelial structures of the affected organs, as well as, B-cell hyperreactivity. The latter, is attested by the increased production of autoantibodies, directed against many different organ and nonorgan self-antigens. Vasculitis is a significant and potentially life-threatening complication of the disease depending on the size, localization, histologic type and the pathogenetic mechanisms of the inflammatory process. The potentially irreversible tissue damage, as well as the increased risk for NHL development, prompts the need for early diagnosis and treatment of cryoglobulinaemic vasculitis in pSS.
32921079 Adult-onset Still's disease presenting as fever of unknown origin: a single-center retrosp 2020 Sep BACKGROUND: The aim of this study is to establish a clinical diagnosis model as a new evaluation indicator for the differentiation of adult-onset Still's disease (AOSD) and other fever of unknown origin disease (FUO). METHODS: This is an observational case-control study between January 2010 and December 2018. Laboratory parameters of AOSD group (N=91), FUO group (N=89) and control group (N=81) including procalcitonin, C-reactive protein (CRP), ferritin, leukocyte, lymphocyte, neutrophil, lymphocyte proportion, neutrophil proportion, red blood cell distribution width (RDW), platelet and platelet parameters were collected. Descriptive statistics and logistic regression were performed to establish a model based on these laboratory variables. RESULTS: After univariate screening, the variables including CRP, leukocyte, neutrophil, lymphocyte proportion, neutrophil proportion, ferritin and mean platelet volume (MPV) showed significant difference between AOSD and FUO groups, then a stepwise regression analysis was performed to establish a model based on these screened variables, at last ferritin, neutrophil and MPV were significantly different in the model. The results suggested that the higher value of ferritin and neutrophil, the lower value of MPV in the model indicated the higher risk to diagnose AOSD. Area under the curve (AUC) of the model was 0.909 (95% CI: 0.855-0.947), which showed high differential diagnostic value (sensitivity: 86.6%, specificity: 82.0%). CONCLUSIONS: The diagnosis model of AOSD and other FUO was established, with an outstanding performance for differential diagnosis.
32093745 Unstimulated whole saliva flow for diagnosis of primary Sjögren's syndrome: time to revis 2020 Feb 24 BACKGROUND: Unstimulated whole saliva (UWS) flow rate is one of the ACR/EULAR 2016 criteria for primary Sjögren's syndrome (pSS). With a single threshold of ≤ 0.1 mL/min, UWS flow does not take into account the age- and sex-related physiological variations. Furthermore, it has a low sensitivity for the diagnosis of pSS (about 50%), contrary to the screening test for xerophthalmia, Schirmer's test (sensitivity of about 70%). We aimed to identify UWS thresholds allowing better performances for a screening test for pSS comparable to Schirmer's test, and considering age- and sex-related variations. METHODS: A prospective cohort of 185 patients with oral and/or ocular dryness was classified into 3 groups: men, women < 50 (< 50 years old), and women ≥ 50 (≥ 50 years old). The diagnostic performances of UWS flow rate in these groups were compared in terms of sensitivity, specificity, positive and negative predictive values, and ROC curves. The identification of thresholds that optimize diagnostic performances was carried out using Youden's index. RESULTS: The diagnostic performances of UWS flow rate varied according to age and sex. UWS had poor diagnostic performances whatever the threshold in the women ≥ 50 group. The threshold of 0.2 mL/min had a sensitivity of ≥ 70% and a specificity of ≥ 50% in both men and women < 50 groups. In the whole population and compared to the current cutoff, a threshold of 0.2 mL/min increased sensitivity (+ 19.8%) and positive (+ 2.3%) and negative (+ 7.0%) predictive values, with a better specificity (65.2%) than Schirmer's test. CONCLUSION: For objective assessment of xerostomia, raising the threshold of the UWS flow rate to 0.2 mL/min would optimize its screening performances for pSS.
31708548 Elderly-onset Still's Disease Complicated by Macrophage Activation Syndrome: A Case Report 2020 Mar 1 Adult-onset Still's disease (AOSD) usually affects young adults. Some cases of elderly-onset Still's disease (EOSD) have been reported, but its clinical features are unclear. We herein report a 74-year-old woman who developed AOSD with macrophage activation syndrome (MAS). We also reviewed 24 previous EOSD cases in patients over 70 years old and compared the findings with overall AOSD. While the clinical features were similar between the two, including the presence of MAS, disseminated intravascular coagulation was more frequent in EOSD than in AOSD. Furthermore, despite a similar frequency of glucocorticoid use, immunosuppressants and biologics were less frequently administered in EOSD than in AOSD. This report highlights the fact that typical AOSD can develop in elderly patients with some characteristic features.
32990854 The Role of Polymorphic Markers rs1478604, rs2292305, and rs2228262 in THBS1 Gene in the D 2020 Sep An association of polymorphic marker rs2228262 in the THBS1 gene with the risk of developing dry eye in Sjögren syndrome was revealed. Confocal microscopy data suggest that this polymorphic marker is responsible for the high probability of corneal nerve fiber lesion in Sjögren syndrome even in the absence of clinical and functional signs of dry eye syndrome. A significant correlation was established between polymorphic markers rs1478604, rs2228262 in THBS1 gene and the coefficients of anisotropy and orientation symmetry of corneal nerve fibers. These results allow considering these polymorphic markers as a genetic factor of predisposition to dry eye syndrome in patients with Sjögren syndrome.