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ID PMID Title PublicationDate abstract
32538490 Association of salivary inflammatory biomarkers with primary Sjögren's syndrome. 2020 Oct BACKGROUND: Primary Sjogren's syndrome (pSS) is an autoimmune disease that leads to salivary and lacrimal gland dysfunction. The adaptive immune response associated with T helper-2 lymphocytes appears to be altered in these patients. Therefore, the objective of this study was to determine the salivary levels of interleukin (IL)-6, IL-5, and IL-4 in patients with pSS when compared to a healthy control (HC) group. The secondary objectives were to study whether ILs levels in pSS patients were associated with salivary flow, patient-reported outcomes (PROMs) for xerostomia and oral health quality of life (Oral Health Impact Profile-14 [OHIP-14]), pSS classification criteria and presence of extraglandular manifestations. METHODS: A case-control study was conducted in 36 patients with pSS and 35 HCs. Cytokine levels were measured using high-sensitivity multiplex map human immunoassays. Unstimulated and stimulated whole saliva were collected and patients filled out questionnaires. The Mann-Whitney U test, chi-squared test, and Spearman correlation test were used. RESULTS: Interleukin-6 was significantly higher in pSS patients than in HCs (P = .0001). IL-6 was significantly higher in pSS patients with a positive salivary gland biopsy (P = .04), whole stimulated saliva hyposalivation (P = .02), and presence of musculoskeletal disorders (P = .03). There was a non-significant positive correlation between IL-6 levels and PROMs for xerostomia (r = .31; P = .06) and OHIP-14 (r = .07; P = .68) in pSS patients. Levels of IL-4 and IL-5 were not detected in both pSS and HCs patients. CONCLUSIONS: Salivary IL-6 levels are significantly associated with pSS patients, and therefore, it is hypothesized that this biomarker may be useful in the diagnosis and follow-up of this disease.
32458243 Diagnostic utility of submandibular and labial salivary gland biopsy in IgG4-related siala 2020 Dec OBJECTIVE: The diagnostic utility of labial salivary gland (LSG) biopsy for IgG4-related sialadenitis remains undetermined. The purpose of the present study was to determine whether submandibular gland biopsy could be replaced by LSG biopsy for diagnosing IgG4-RS. PATIENTS AND METHODS: Medical records of two groups of patients were reviewed. Group A contained 45 patients suspected to have IgG4-RS who underwent both SMG and LSG biopsies. Group B contained 25 patients who were clinically and pathologically diagnosed with Sjögren syndrome (SS). Biopsy samples were stained using hematoxylin and eosin (HE) and immunohistochemical techniques and observed under an optical microscope. Relevant data describing histopathological characteristics were collected and analyzed. RESULTS: SMG of IgG4-RS patients presented typical histopathological characteristics of fibrosis and IgG4-positive plasmacytic infiltration, while LSG showed varied characteristics. The sensitivity and accuracy of SMG for diagnosing IgG4-RS were greater than those of LSG (100% and 100% versus 55.3% and 75.7%, respectively, P < 0.05). CONCLUSIONS: Biopsy of SMG showed greater sensitivity and specificity, whereas LSG biopsy showed varied histopathological and immunohistochemical characteristics; thus, SMG biopsy cannot be replaced by LSG biopsy for diagnosis of IgG4-RS.
32023755 [Primary thymic mucosa-associated lymphoid tissue lymphoma: 7 clinical cases report and a 2020 Jan 14 Objective: To reveal clinical features, pathological diagnosis, treatment and prognosis of primary thymic mucosa-associated lymphoid tissue (MALT) lymphoma and review literatures. Methods: The clinical characteristics, pathological diagnosis, laboratory texts, treatment and prognosis of 7 cases of primary thymic MALT lymphoma identified at the First Affiliated Hospital of Nanjing Medical University from November 2017 to January 2019 were collected and analyzed. Results: Of 7 primary thymic MALT lymphoma cases, six were female. Patients were often asymptomatic and were found mediastinal mass by chest CT. After mediastinal mass resection, pathologist reported a primary thymic MALT lymphoma. Laboratory tests showed all patients were positive for anti-nuclear antibody, anti-Ro52 antibodies and anti-Sjogren's syndrome A antibodies, and increased erythrocyte sedimentation rate (ESR) . Four were diagnosed with Sjogren's syndrome (SS) . After surgery, the patients were given the positron emission tomography computed tomography (PET-CT) scans. All cases received "watch and wait" approach. Up to now, all cases showed good prognoses and none of them relapsed. Conclusion: Primary thymic MALT lymphoma was rare, and it was often associated with autoimmune diseases. Such patients who usually had good prognoses should be followed up closely and avoided excessive treatments if there were no indications of intervention.
31615914 Comparison of Effect on Sicca Symptoms of Anticentromere Antibody-positive Sjögren Syndro 2020 Jun 1 OBJECTIVE: To determine whether positive anticentromere antibody (ACA) serology affects the severity of sicca symptoms in patients with primary Sjögren syndrome (pSS). METHODS: Evaluation to detect subjective and objective sicca symptoms included questionnaires, physical examination, and pathology. Cases of pSS were classified according to the 2002 American-European Consensus Group (AECG) criteria. All patients were evaluated for presence of anti-Ro, anti-La, and ACA serology. Patients with pSS were categorized into ACA+ SS and ACA-SS. The groups were compared for measures of severity of oral and ocular sicca. RESULTS: The pSS group had 446 patients, of whom 26 were ACA+ SS. Subjective ocular sicca measured 7.0 ± 2.4 (out of 10) in ACA+ SS and 6.4 ± 2.6 in ACA-SS (p = 0.197). Objective ocular sicca measured 3.2 mm ± 1.8 mm/5 min in ACA+ SS and 4.2 mm ± 4.4 mm/5 min in ACA-SS (p = 0.038). Subjective oral sicca measured 8.5 ± 1.4 in ACA+ SS and 6.7 ± 2.4 in ACA-SS (p < 0.001). Objective oral sicca measured 0.1 ml ± 0.2 ml/15 min in ACA+ SS and 0.4 ml ± 1.0 ml/15 min in ACA-SS (p < 0.001). Only 35% of ACA+ patients with SS were anti-Ro-positive or anti-La-positive compared with 77% of the ACA-patients with SS (p < 0.001). There was no significant difference in minor salivary gland fibrosis or focus scores between ACA+ SS and ACA-patients with SS. CONCLUSION: ACA+ SS is associated with more severe objective ocular sicca and more severe subjective and objective oral sicca compared to ACA-SS. The majority of ACA+ patients with SS meet AECG criteria for pSS despite negative serology for anti-Ro/La antibodies.
33415880 [CAI Sheng-chao's experience in treatment of Sjögren's syndrome with moxibustion for prom 2020 Dec 12 Professor CAI Sheng-chao's clinical experience is summarized in the treatment of Sjögren's syndrome with moxibustion for promoting meridian circulation and warming yang and the typical case is introduced. Professor CAI believes that Sjögren's syndrome refers to zao (dry) bi syndrome. It is the insufficiency of yin and body fluid for the primary and yang qi deficiency for the root. On the base of traditional long-snake moxibustion, the moxibustion therapy for promoting meridian circulation and warming yang is adopted to warm up and tonify zangfu organs. When the function of yang qi is restored, body fluid (yin) will be generated naturally. Hence, the diagnosis and treatment system is developed by the integration of the theory as "moxibustion applicable for yin deficiency" and the characteristic moxibustion therapy.
33095152 Systemic phenotype related to primary Sjögren's syndrome in 279 patients carrying isolate 2020 Jul OBJECTIVES: To evaluate the systemic phenotype associated with the presence of isolated anti-La/SSB antibodies in a large international registry of patients with primary Sjögren's syndrome (pSS) fulfilling the 2002 classification criteria. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. Baseline clinical information from leading centres on clinical research in SS of the 5 continents was collected. Combination patterns of anti-Ro/SSA-La/SSB antibodies at the time of diagnosis defined the following four immunological phenotypes: double positive (combined Ro/SSA and La/SSB,) isolated anti-Ro/SSA, isolated anti-La/SSB, and immunonegative. RESULTS: The cohort included 12,084 patients (11,293 females, mean 52.4 years) with recorded ESSDAI scores available. Among them, 279 (2.3%) had isolated anti-La/SSB antibodies. The mean total ESSDAI score at diagnosis of patients with pSS carrying isolated anti-La/SSB was 6.0, and 80.4% of patients had systemic activity (global ESSDAI score ≥1) at diagnosis. The domains with the highest frequency of active patients were the biological (42.8%), glandular (36.8%) and articular (31.2%) domains. Patients with isolated anti-La/SSB showed a higher frequency of active patients in all ESSDAI domains but two (articular and peripheral nerve) in comparison with immune-negative patients, and even a higher absolute frequency in six clinical ESSDAI domains in comparison with patients with isolated anti-Ro/SSA. In addition, patients with isolated anti-La/SSB showed a higher frequency of active patients in two ESSDAI domains (pulmonary and glandular) with respect to the most active immunological subset (double-positive antibodies). Meanwhile, systemic activity detected in patients with isolated anti-La/SSB was overwhelmingly low. Even in ESSDAI domains where patients with isolated anti-La/SSB had the highest frequencies of systemic activity (lymphadenopathy and muscular), the percentage of patients with moderate or high activity was lower in comparison with the combined Ro/SSA and La/SSB group. CONCLUSIONS: Patients carrying isolated La/SSB antibodies represent a very small subset of patients with a systemic SS phenotype characterised by a significant frequency of active patients in most clinical ESSDAI domains but with a relative low frequency of the highest severe organ-specific involvements. Primary SS still remains the best clinical diagnosis for this subset of patients.
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.
33342022 Pilot study of a machine-learning tool to assist in the diagnosis of hand arthritis. 2020 Dec 20 BACKGROUND: Arthritis is a common condition, which frequently involves the hands. Patients with inflammatory arthritis have been shown to experience significant delays in diagnosis. AIM: To develop and test a screening tool combining an image of a patient's hands, a short series of questions and a single examination technique to determine the most likely diagnosis in a patient presenting with hand arthritis. Machine learning techniques were used to develop separate algorithms for each component, which were combined to produce a diagnosis. METHODS: A total of 280 consecutive new patients presenting to a rheumatology practice with hand arthritis were enrolled. Each patient completed a nine-part questionnaire, had photographs taken of each hand and had a single examination result recorded. The rheumatologist diagnosis was recorded following a 45-min consultation. The photograph algorithm was developed from 1000 previous hand images and machine learning techniques were applied to the questionnaire results, training several models against the diagnosis from the rheumatologist. RESULTS: The combined algorithms in the present study were able to predict inflammatory arthritis with an accuracy, precision, recall and specificity of 96.8%, 97.2%, 98.6% and 90.5% respectively. Similar results were found when inflammatory arthritis was subclassified into rheumatoid arthritis and psoriatic arthritis. The corresponding figures for osteoarthritis were 79.6%, 85.9%, 61.9% and 92.6%. CONCLUSION: The present study demonstrates a novel application combining image processing and a patient questionnaire with applied machine-learning methods to facilitate the diagnosis of patients presenting with hand arthritis. Preliminary results are encouraging for the application of such techniques in clinical practice.
35415510 Coronoid Impingement Causing Early Failure of Total Elbow Arthroplasty. 2020 Sep Total elbow arthroplasty (TEA) is a well-established treatment for end-stage rheumatoid arthritis of the elbow. With improved surgical techniques and implant designs, TEA is also effective in treating elbow osteoarthritis, posttraumatic arthritis, distal humerus nonunion, and comminuted distal humerus fractures in the elderly population. There have been multiple reports of greater than 90% survival rate, free of reoperation at 10 years. We present a case of early failure of TEA caused by coronoid impingement, to provide a surgical pearl for others to avoid this complication.
32616069 Correction to: Temporary interruption of baricitinib: characterization of interruptions an 2020 Jul 2 An amendment to this paper has been published and can be accessed via the original article.
31129869 A multidisciplinary approach to reproductive healthcare in women with rheumatic disease. 2020 Feb INTRODUCTION: Rheumatic disease (RD) patients when family planning must consider fertility, disease activity, and management from preconception to lactation. A clear understanding is necessary, especially for those receiving disease-modifying antirheumatic medications. Previous studies have highlighted unmet needs in the care of women with RDs with reproductive healthcare needs. This study describes the first published standardized reproductive care pathway for women with RDs and the outcomes of this approach. MATERIAL AND METHODS: We developed the care pathway with multidisciplinary input from rheumatologists, rheumatology nurse specialists, obstetricians, midwives, maternal medicine specialists, and pharmacists. We identified patients' emotional and healthcare needs, ensured access to expert advice, maintenance of good disease control, and positive reproductive outcomes. We prospectively followed the patients and report the results of the service. RESULTS: Ninety-eight women with median age (range) of 35 years (19-48) were assessed. The majority had an inflammatory arthritis. Seventy-six babies were born to 62 mothers. There were 12 miscarriages and one perinatal death. Breastfeeding rates at 6 weeks were low (28%). CONCLUSION: We describe the first published evidence-based integrated multidisciplinary reproductive care pathway for women with RDs and the results of this approach. Seventy percent of women successful in trying to conceive delivered a healthy baby, and 90% of patients were 'very satisfied' with the service.
32266326 Healthcare resource utilization and costs associated with inflammatory bowel disease among 2020 BACKGROUND: Chronic inflammatory diseases (CIDs; ankylosing spondylitis [AS], psoriatic arthritis [PsA], psoriasis [PsO], or rheumatoid arthritis [RA]) and inflammatory bowel disease (IBD; Crohn's disease and ulcerative colitis) are associated with substantial economic burden. The relative increased costs among patients with CIDs and concomitant IBD compared to those without IBD is an important consideration when deciding on the clinical management of patient symptoms. Given the increasing use of novel agents for the treatment of CIDs, including those that may increase the risk of IBD in patients with CIDs, the objective of the study was to describe the incidence of IBD and to quantify healthcare resource utilization (HRU) and costs associated with IBD among patients with CIDs. METHODS: The IBM MarketScan® Research Databases (1/2010-7/2017) were used to identify adult patients with ≥2 claims with a diagnosis of either AS/PsA/PsO/RA (index date was a random claim for AS/PsA/PsO/RA). The one-year incidence rate of IBD was calculated following the index date. HRU and healthcare costs were compared between patients developing and not developing IBD in the year following the index date, adjusting for baseline characteristics. RESULTS: A total of 537,450 patients with CIDs (mean age = 54.0 years; 63.1% female) were included in the study. The 1-year incidence rate of IBD was 0.52% (range = 0.39% in patients with PsO but without PsA to 1.73% in patients with AS). Patients who developed IBD (N = 2778) had significantly higher rates of inpatient, outpatient, and emergency room visits (incidence rate ratios [IRR] = 2.91, 1.35, 1.81; all P < 0.0001), compared to patients without IBD (N = 534,672). Patients who developed IBD had $18,500 (P < 0.0001) higher total costs per year, including $15,121 (P < 0.0001) higher medical costs and $3380 higher pharmacy costs (P < 0.0001). CONCLUSION: Higher HRU and costs were observed in patients with concomitant CID and IBD compared to patients with CID alone. Consideration should be given to treatment decisions that adequately manage CID and IBD to ensure optimal clinical and economic outcomes.
32218732 Synergistic Effects of Erzhi Pill Combined With Methotrexate on Osteoblasts Mediated via t 2020 Rheumatoid arthritis (RA) is a chronic systemic autoimmune disease characterized by chronic synovitis, bone erosion, and bone loss. Erzhi Pill (EZP), a classic Chinese patent medicine, is often used to treat osteoporosis and shows a capacity for bone metabolism regulation. Methotrexate (MTX), an essential drug for RA treatment, has been reported to inhibit generalized bone loss in RA patients. However, the combined therapeutic effects and mechanism of EZP and MTX in RA have not been fully elucidated. The aim of this study was to investigate the synergistic effect of EZP and MTX on RA and to explore the underlying mechanism through network pharmacological prediction and experimental verification. Chemical compounds of EZP, human target proteins of EZP and MTX, and RA-related human genes were identified in the Encyclopedia of Traditional Chinese Medicine database, PubChem database, and NCBI database, respectively. The molecular network of EZP and MTX in RA was generated and analyzed with Ingenuity Pathway Analysis software according to the datasets. Then, MTX monotherapy, EZP monotherapy, and combined MTX and EZP therapy were administered to collagen-induced arthritis rats, followed by assessment of pathological score, bone damage, bone alkaline phosphatases (BALP), and tartrate-resistant acid phosphatase (TRACP), and of gene levels related to the Wnt1/LRP5/β-catenin pathway according to network pharmacological analysis. Finally, serum samples from MTX-, EZP- and MTX+EZP-treated rats were used to treat the rat osteoblast (OB)-like UMR-106 cell line to evaluate gene levels related to Wnt1/LRP5/β-catenin. Network pharmacological analysis showed that the Wnt/β-catenin signaling pathway was the top signaling pathway shared among MTX, EZP, and RA. The results from in vivo experiments indicated that EZP combined with MTX reduced arthritis severity, alleviated ankle bone damage, increased BALP and decreased TRACP serum levels, and regulated the mRNA expression of Wnt1, LRP5, β-catenin, Runx2, BALP, and BGP in the ankles. In vitro experiments showed that EZP combined with MTX could also improve the expression of genes related to the Wnt1/LRP5/β-catenin pathway. This study demonstrated that EZP in combination with MTX played a synergistic role in regulating OBs in RA, which was connected to the modulatory effect of EZP and MTX on the Wnt1/LRP5/β-catenin signaling pathway.
31562738 Analysis of intestinal immunity and flora in a collagen-induced mouse arthritis model: dif 2020 Jan 9 Intestinal immunity and flora are reported to be associated with the onset of rheumatoid arthritis. However, differences in the intestinal immunity and flora dynamics between the initial peak and relapse of arthritis have not been investigated. Here we analyzed the lymphocyte populations in different lymphoid tissues, the IgA in feces, and the intestinal flora at the initial peak and the relapse phase of arthritis in a collagen-induced arthritis (CIA) mouse model. In this model compared with the control group, the percentage of RORγt+CD4+ T cells in the mesenteric lymph nodes (mLN) was increased at the initial peak but decreased at the relapse stage of arthritis, and the opposite changes were observed in the spleen. The percentage of Foxp3+CD4+ T cells was unchanged at the initial peak in both tissues but increased only in the mLN at the relapse stage. The IgA in feces increased with the progression of arthritis, and bacterial analysis revealed that some specific bacterial families were changed at the peak and relapse stages of arthritis. Finally, the immune dynamics under different arthritic conditions were examined by integrating these factors using principal component analysis (PCA). PCA showed that the immunological and intestinal flora profiles were different between the initial peak and the relapse of the arthritis. Our findings suggest that the intestinal immunity and the environment change drastically with the progress of arthritis.
32569777 Sphingosine kinase 1/sphingosine 1-phosphate/sphingosine 1-phosphate receptor 1 pathway: A 2020 Sep 1 OBJECTIVE: Rheumatoid arthritis (RA) is a common inflammatory autoimmune disease characterized by the formation of joint synovitis and pannus. Sphingosine 1-phosphate (S1P) is an important mediator related to angiogenesis, inflammation and autoimmunity. As Geniposide (GE) has potent immuno-modulation function, we investigated the effects on the dynamic balance of angiogenesis-related factors and Sphingosine kinase 1 (SphK1)-S1P-S1P receptor 1 (S1PR1) signal transduction in adjuvant-induced arthritis (AA) rats. METHOD: The model evaluation was performed from paw swelling degree, arthritis index and movement score. The immunohistochemistry and enzyme-linked immunosorbent assay were used to study the microvascular density (MVD) and pro/anti-angiogenic factors levels. The cell viability was examined by cell counting kit-8 assay. SphK1, S1PR1 mRNA and protein levels in fibroblast-like synoviocytes (FLSs) were detected by quantitative real-time polymerase chain reaction and Western blotting. RESULTS: The results showed that GE can apparently suppressed the inflammatory pathological status. The arthritis index, paw swelling and MVD of AA rats were decreased with dose dependence ((⁎)P < 0.05, (⁎⁎)P < 0.01). In addition, GE can reduce the secretion of vascular endothelial growth factor (VEGF) and angiopoietin-1 (Ang-1), promote the secretion of endostatin (ES) and inhibit excessive proliferation of FLSs ((⁎)P < 0.05, (⁎⁎)P < 0.01). Importantly, GE can significantly inhibit the activity of SphK1, the level of S1P and the expression of SphK1 and S1PR1 in FLSs ((⁎)P < 0.05, (⁎⁎)P < 0.01). CONCLUSION: It indicated that GE reduces the activity of SphK1 by restoring the dynamic balance between pro/anti-angiogenic factors, thereby interfering with SphK1-S1P-S1PR1 signal transduction, reducing the formation of synovial microvessels and exerting anti-angiogenesis effect of RA.
34018170 Macrophage Activation Syndrome in a Case of Myasthenia Gravis with Concurrent Cytomegalovi 2020 Dec OBJECTIVE: Macrophage activation syndrome (MAS) or reactive hemophagocytic lymphohistiocytosis (HLH) refers to a set of clinical symptoms caused by the excessive activation and proliferation of macrophages. It was linked with autoimmune disease such as systemic-onset juvenile rheumatoid arthritis, systemic lupus erythematosus, rheumatoid arthritis, and dermatomyositis, etc. Herein we report a case of myasthenia gravis (MG) with concurrent cytomegalovirus (CMV) infection developed MAS. CASE REPORT: A 31-year-old female with history of MG for 2 years under stable control with azathioprine and prednisolone. She presented with persistent high fever for 2 weeks after an upper respiratory infection. Lab data revealed pancytopenia, elevated triglyceride, ferritin and C-reactive protein (CRP). A bone marrow aspiration confirmed hemophagocytosis. Investigation for occult infection revealed her plasma was positive for CMV IgG and IgM, and high for CMV viral load. She was then treated with 5 sessions of plasmapheresis and pulse steroid. Azathioprine was discontinued and replaced with cyclosporine. Gancylovir was given for her concurrent CMV infection. After 2 weeks of treatment, her fever gradually subsided, and her blood cell count, hepatobiliary enzymes, ferritin and CRP have returned to normal range. She was discharged in good recovery. CONCLUSION: MAS is a rare complication of systemic autoimmune disease with poor prognosis, which may be precipitated by concurrent infection. Early recognition of this syndrome and prompt immune modulation therapy is crucial for successful treatment.
33358993 Mapping Out Autoimmunity Control in Primary Immune Regulatory Disorders. 2021 Feb There is a growing understanding of the clinical overlap between primary immune deficiency and autoimmunity. An atypical or treatment-refractory clinical presentation of autoimmunity may in fact signal an underlying congenital condition of primary immune dysregulation (an inborn error of immunity). Detailed profiling of the family history is critical in the diagnostic process and must not be limited to the occurrence of frequent or atypical infections, but additionally should include inquiries into chronic forms of autoimmunity, hyperinflammation, and malignancy. A genetic and a functional diagnostic approach are complementary and nonoverlapping methods of identifying and validating an inborn error of immunity. Extended immune phenotyping of both affected and unaffected family members may provide insight into disease mode of inheritance, penetrance, and secondary inherited or environmentally acquired modifiers. Clinical care of a family with an inborn error of immunity may require local and national expertise in addition to cross-disciplinary care from the disciplines of pediatrics and internal medicine. Physician communication across subspecialties as well as distinct medical institutes can facilitate the appropriate disclosure of genetic testing results toward their prompt incorporation into patient care. Targeted immunomodulation based directly on genetic and functional immune phenotyping has the potential to reduce unnecessary immunosuppression and provide more exacting therapeutic benefit to our patients.
33324249 Anxiety, Reinforcement Sensitivity and Social Context in Accepting the Experience of Pain 2020 Background: Acceptance has become one of the most widely studied processes regarding chronic pain because of its ability to influence participants' adaptation and coping responses. Leading researchers have found relationships between variables such as anxiety, reinforcement sensitivity, and the responses of the participants' environment to their behavior and acceptance. In contrast, few studies have been found that investigate the variables that predict the acceptance of pain. This study has set out to explore the relationships between pain-related anxiety, sensitivity to contingencies, and the punishment responses of significant people toward pain behaviors regarding pain acceptance. Methods: With a view to fulfilling this purpose, a cohort of 62 participants with rheumatoid arthritis was chosen, and the subjects were assessed through the following self-report measures: Chronic Pain Acceptance Questionnaire, CPAQ; Pain Anxiety Symptoms Scale, PASS-20; The Sensitivity to Punishment and Sensitivity to Reward Questionnaire, SPSRQ, and The West Haven-Yale Multidimensional Pain Inventory, WHYMPI. The study's initial objectives were achieved by means of a stepwise multiple linear regression analysis. Results: The linear regression analyses revealed a negative and significant correlation between anxiety, reinforcement sensitivity, and the significant persons' responses to pain behaviors and pain acceptance. Conclusion: The results suggest that the identification of these variables might be important for addressing these participants' pain. Finally, the discussion focuses on our findings' implications as regards their use in clinical practice.
32561991 [Sonography of the foot in rheumatology : Ultrasound diagnostics of the ankle joint and fo 2020 Oct Ultrasound examination of the joints is an essential component of diagnostics in rheumatology. Due to its easy accessibility, excellent image definition of soft tissue and bone surfaces with standardized scan sections and scoring systems, ultrasound examination of the joints enables decisions to be made on early and differential diagnostics, treatment monitoring and prognosis. Involvement of the ankle and foot is a common problem in both inflammatory and degenerative rheumatological diseases. Persisting inflammatory processes and bone destruction increase the burden of disease by causing a loss of mobility due to pain. As treatment is often prolonged it is an important issue for both the quality of life of affected patients and a burden on the resources of the healthcare system. The anatomical characteristics of the foot make it difficult to draw diagnostic conclusions by physical examination only. These diagnostic gaps in the remaining rheumatological questions can often be answered by ultrasound examination of the joint.