The Experts below are selected from a list of 174 Experts worldwide ranked by ideXlab platform

Nadia Vazirpanah - One of the best experts on this subject based on the ideXlab platform.

  • cytometry by time of flight identifies distinct signatures in patients with systemic sclerosis systemic lupus erythematosus and Sjogrens Syndrome
    European Journal of Immunology, 2020
    Co-Authors: Maarten Van Der Kroef, Lucas L Van Den Hoogen, Jorre S Mertens, Sofie L M Blokland, Scott Haskett, Abhinandan Devaprasad, Tiago Carvalheiro, Eleni Chouri, Nadia Vazirpanah
    Abstract:

    Systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and primary Sjogrens Syndrome (pSS) are clinically distinct systemic autoimmune diseases (SADs) that share molecular pathways. We quantified the frequency of circulating immune-cells in 169 patients with these SADs and 44 healty controls (HC) using mass-cytometry and assessed the diagnostic value of these results. Alterations in the frequency of immune-cell subsets were present in all SADs compared to HC. Most alterations, including a decrease of CD56hi NK-cells in SSc and IgM+ Bcells in pSS, were disease specific; only a reduced frequency of plasmacytoid dendritic cells was common between all SADs Strikingly, hierarchical clustering of SSc patients identified 4 clusters associated with different clinical phenotypes, and 9 of the 12 cell subset-alterations in SSc were also present during the preclinical-phase of the disease. Additionally, we found a strong association between the use of prednisone and alterations in B-cell subsets. Although differences in immune-cell frequencies between these SADs are apparent, the discriminative value thereof is too low for diagnostic purposes. Within each disease, mass cytometry analyses revealed distinct patterns between endophenotypes. Given the lack of tools enabling early diagnosis of SSc, our results justify further research into the value of cellular phenotyping as a diagnostic aid.

A J Silman - One of the best experts on this subject based on the ideXlab platform.

  • weak association between subjective symptoms of and objective testing for dry eyes and dry mouth results from a population based study
    Annals of the Rheumatic Diseases, 1998
    Co-Authors: Elaine Thomas, Ali H Hajeer, H Chambers, A J Silman
    Abstract:

    OBJECTIVES To determine associations between symptoms of dry eyes and dry mouth and objective evidence of lacrimal and salivary gland dysfunction in a population based sample. To determine associations between these elements and the presence of autoantibodies. METHODS A cross sectional population based survey. Subjects were interviewed and examined (Schirmer-1 test and unstimulated salivary flow) for the presence of dry eyes and mouth. Antibodies (anti-Ro [SS-A], anti-La [SS-B], rheumatoid factor, antinuclear antibody) were measured. RESULTS 341 subjects were examined. Twenty four per cent had dry eye symptoms, 29% dry mouth symptoms, and 14% both. There was only a weak association between the presence of oral or ocular symptoms and their respective test results. Associations were strongest between dry mouth symptoms and positive test results, and in subjects under 55 years of age. There was no association between the presence of autoantibodies and either symptoms or signs of dry eyes or dry mouth. CONCLUSION Only weak associations were found between self reported symptoms of dry eyes and dry mouth and objective measures said to define Sjogrens Syndrome in the general population. The clinical significance of these symptoms in the community needs reappraisal.

Jorre S Mertens - One of the best experts on this subject based on the ideXlab platform.

  • cytometry by time of flight identifies distinct signatures in patients with systemic sclerosis systemic lupus erythematosus and Sjogrens Syndrome
    European Journal of Immunology, 2020
    Co-Authors: Maarten Van Der Kroef, Lucas L Van Den Hoogen, Jorre S Mertens, Sofie L M Blokland, Scott Haskett, Abhinandan Devaprasad, Tiago Carvalheiro, Eleni Chouri, Nadia Vazirpanah
    Abstract:

    Systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and primary Sjogrens Syndrome (pSS) are clinically distinct systemic autoimmune diseases (SADs) that share molecular pathways. We quantified the frequency of circulating immune-cells in 169 patients with these SADs and 44 healty controls (HC) using mass-cytometry and assessed the diagnostic value of these results. Alterations in the frequency of immune-cell subsets were present in all SADs compared to HC. Most alterations, including a decrease of CD56hi NK-cells in SSc and IgM+ Bcells in pSS, were disease specific; only a reduced frequency of plasmacytoid dendritic cells was common between all SADs Strikingly, hierarchical clustering of SSc patients identified 4 clusters associated with different clinical phenotypes, and 9 of the 12 cell subset-alterations in SSc were also present during the preclinical-phase of the disease. Additionally, we found a strong association between the use of prednisone and alterations in B-cell subsets. Although differences in immune-cell frequencies between these SADs are apparent, the discriminative value thereof is too low for diagnostic purposes. Within each disease, mass cytometry analyses revealed distinct patterns between endophenotypes. Given the lack of tools enabling early diagnosis of SSc, our results justify further research into the value of cellular phenotyping as a diagnostic aid.

Dag Leonard - One of the best experts on this subject based on the ideXlab platform.

  • 264 damage accrual in swedish systemic lupus erythematosus secondary Sjogrens Syndrome is among the factors associated with increased risk
    Lupus science & medicine, 2019
    Co-Authors: Martina Frodlund, Sarah Reid, Jonas Wettero, Orjan Dahlstrom, Christopher Sjowall, Dag Leonard
    Abstract:

    Background Although the expected survival of patients with systemic lupus erythematosus (SLE) has improved during the last 50 years, accrual of damage remains a critical concern. Acquired damage is tightly linked to decreased quality-of-life and premature death, and could be due to raised disease activity, drug-related side-effects or co-morbidities. Methods Accumulation of organ damage was studied in 543 well-characterized and from 1998 and onwards consecutively recruited prevalent/incident SLE cases meeting the 1982 American College of Rheumatology (ACR82) and/or the 2012 SLICC criteria. The SLICC/ACR damage index (SDI) was used to estimate damage. Disease variables were evaluated regarding association with damage accrual, and time to first damage. Detailed information on clinical and immunological features, as well as damage in each SDI domain, where at hands. Standardized mortality rate was calculated and causes of death recorded. Comparisons between groups were performed using Chi-square- or Mann-Whitney U-tests, p-values Results 59% of the patients had an SDI score 1% and 25% had extensive damage defined as SDI 3. Patients with presence of damage (SDI 1) were significantly older at disease onset, had longer SLE duration and fulfilled further classification criteria. Caucasian ethnicity was more common among cases with damage. Having ACR82-defined neurologic disorder, antiphospholipid Syndrome (APS), any antiphospholipid antibody (positive IgG anti-2-GPI or a lupus anticoagulant test, separately) as well as concomitant co-morbidities such as hypertension, hyperlipidemia, diabetes, depression and secondary Sjogrens Syndrome were associated with presence of damage (SDI 1). In addition to factors associated with SDI 1, serositis, renal and hematological disorder as well as interstitial lung disease and positive IgG anti-cardiolipin were associated with extensive damage (SDI 3). Time to first damage was significantly shorter for males and for cases with a positive lupus anticoagulant test, whereas APS patients were borderline significant. Cases with malar rash and anti-La/SSB antibodies had significantly longer time to first damage. Malignancy was the most common cause of death. Conclusions Despite that Swedish health care is tax-funded and offers universal access, a considerable number of patients are affected by irreversible damage over time. We confirm previous observations for several damage associations and report secondary Sjogrens Syndrome to be associated with an increased risk of organ damage in SLE. Funding Source(s): This work was supported by grants from the Swedish Rheumatism Association, the County Council of Ostergotland, the Swedish Society of Medicine, the King Gustaf Vs 80 year anniversary foundation and the King Gustaf V and Queen Victorias Freemasons foundation.

Servet Akar - One of the best experts on this subject based on the ideXlab platform.

  • 269 coexistence of axial spondyloarthritis systemic lupus erythematosus Sjogrens Syndrome and secondary antiphospholipid Syndrome case report tarhan f keser g celik o klnc rm akar s
    Lupus science & medicine, 2019
    Co-Authors: Emne Fgen Tarhan, Gokhan Keser, Ozgur Ilhan Celik, Raba Mhrban Kilic, Servet Akar
    Abstract:

    Background Axial spondyloarthritis (AxSpA) is a chronic inflammatory disease of the axial skeleton which manifests as inflammatory back pain and progressive stiffness of the spine. Patients with axial disease and other features of SpA but no unequivocal sacroiliitis in conventional X-ray now termed as non-radiographic axial spondyloarthritis (nr-AxSpA). Those patients can be diagnosed on the basis of the presence of active inflammation in magnetic resonance imaging (MRI) or human leukocyte antigen B27 (HLA-B27).Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown etiology that may affect the skin, joints, kidneys, lungs, nervous system, sereous membranes, and/or other organs of the body. Sjogrens Syndrome (SS) is the second most common autoimmune rheumatic condition characterized by lymphocytic infiltrate of the exocrine glands, resulting in dysfunction and destruction of them. Antiphospholipid Syndrome (APS) is the association of thrombosis and/or pregnancy morbidity with antiphospholipid antibodies (aPL) (lupus anticoagulant [LA], anticardiolipin antibodies (aCL), and/or anti-2 -glycoprotein-I antibodies (a2 GPI). Methods Case Presentation We report a 55-year-old female patient having the association of nr-AxSpA, SLE, secondary APS, and SjS. Diagnosis of nr-AxSpA was made based upon the presence inflammatory low-back pain, human leukocyte antigen B27 positivity, and presence of sacroiliitis only in MRI. SLE was diagnosed with butterfly-shaped rash on her cheeks, inflammatory arthritis, photosensitivity, erythema involving dorsal inter-joint area of hand fingers, alopecia together with antinuclear antibody (ANA) and anti-dsDNA positivity, low serum complement levels, leucopenia and thrombocytopenia. Additional presence of sicca symptoms, low Schirmer I test, anti SSA/Ro and anti-SSB/La positivity, supported by positive labial salivary gland biopsy led to the diagnosis of SjS. Results Furthermore, this patient also had miscarriage at 16th week and cerebral vascular disease at 33 years. Besides, IgG and IgM anticardiolipin antibodies were found to be positive twice. Therefore, she was also diagnosed as secondary APS. She fulfilled the relevant criteria for AxSpA, SLE, SjS and APS. Conclusions To our knowledge, this is the first case report showing the association of these four diseases, with different genetic, etiopathogenetic and clinical systemic inflammatory diseases. Funding Source(s): No