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  1. Article ; Online: Psychological features of primary Sjögren syndrome

    V Módis, László / Szántó, Antónia / Bugán, Antal

    Orvosi hetilap

    2021  Volume 162, Issue 39, Page(s) 1558–1566

    Abstract: Összefoglaló. A primer Sjögren-szindróma (pSS) krónikus autoimmun betegség, melynek elsődleges tünetei az exokrin mirigyeket érintő autoimmun folyamat következtében létrejövő szem- és szájszárazság, szisztémás manifesztációi között pedig a leggyakoribbak ...

    Title translation A primer Sjögren-szindróma pszichológiai vonatkozásai.
    Abstract Összefoglaló. A primer Sjögren-szindróma (pSS) krónikus autoimmun betegség, melynek elsődleges tünetei az exokrin mirigyeket érintő autoimmun folyamat következtében létrejövő szem- és szájszárazság, szisztémás manifesztációi között pedig a leggyakoribbak a szellemi és fizikai fáradtság és az ízületi fájdalmak. A betegség lélektani vonatkozásait nagy érdeklődés övezi; közleményünk a vonatkozó kutatási eredményeket foglalja össze a hátterükben húzódó pszichoneuroimmunmodulációs háttér rövid bemutatásával. A pSS pszichológiai tényezői közt a legrégebben vizsgáltak a depresszió és a szorongás. Ezek jelentős hatással vannak a betegek életminőségére, jóllétére, kognitív funkcióira, sőt a betegség aktivitására is. Bemutatjuk továbbá a stressz és a megküzdési stratégiák jellegzetes mintázatait pSS-ben, amelyek közt a betegségre jellemző maladaptív stratégiák a legjelentősebbek a patogenezis megértése és a kezelés szempontjából. Ilyenek a tagadás, a hasítás és a helyettesítés. Kitérünk a Sjögren-szindrómát kísérő személyiségjellemzőkre is, amely ígéretes terület, de kevés adat áll rendelkezésünkre; az eddigi vizsgálatok alapján a neuroticismus a leginkább pSS-re jellemző tényező. A kognitív funkciók érintettségének összefoglalása és az egyéb pszichológiai tényezők (szellemi fáradtság, szexualitás, testképzavar, életminőség) összegzése után egyértelműen levonható a következtetés, hogy a Sjögren-szindróma nemcsak biológiai, hanem pszichés, pszichoszociális és szociális jelenségszinteken is okoz zavarokat, tüneteket. Ezért rendkívül fontos a betegség biopszichoszociális szemléletű kezelése, a betegek aktív, intencionális részvétele a pSS-sel való megküzdésben. Orv Hetil. 2021; 162(39): 1558-1566. Summary. Primary Sjögren syndrome (pSS) is a chronic, autoimmune disorder. Primary symptoms are ocular and oral dryness as a of an autoimmune process affecting the exocrine glands. The most common systemic manifestations of the disease are mental and physical fatigue and arthralgia. Psychological features of pSS are studied with great interest; the present publication reviews the results of the related investigations alongside with the possible psychoneuroimmunomodulatory background. Among psychological factors in pSS, depression and anxiety have been studied the longest. These impact significantly the quality of life, wellbeing, cognitive functions and disease activity of the patients. Afterwards, we introduce the characteristic patterns of stress and coping mechanisms in pSS, among which maladaptive strategies, typical for the disorder are the most important regarding the pathogenesis and the therapy. These pSS-linked maladaptive strategies are denial, disengagement and focus and venting of emotions. As next, we give a summary about personality characteristics in pSS, which is a promising field to study, and yet very few related data are available; based on them, neuroticism seems to be the most common personality factor in Sjögren's. After summarizing briefly cognitive functions and other psychological features (mental fatigue, sexuality, body image disturbance and quality of life), it is evident that pSS is determined not only by biological but also by psychological, psychosocial and social disturbances. Hence, treating pSS patients with a biopsychosocial perspective is crucial and so is the active and intentional participation of patients in their recovery. Orv Hetil. 2021; 162(39): 1558-1566.
    MeSH term(s) Adaptation, Psychological ; Anxiety ; Anxiety Disorders ; Humans ; Quality of Life ; Sjogren's Syndrome
    Language Hungarian
    Publishing date 2021-09-26
    Publishing country Hungary
    Document type Journal Article
    ZDB-ID 123879-6
    ISSN 1788-6120 ; 0030-6002
    ISSN (online) 1788-6120
    ISSN 0030-6002
    DOI 10.1556/650.2021.32234
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  2. Article ; Online: Novel aspects of muscle involvement in immune-mediated inflammatory arthropathies and connective tissue diseases.

    Mogyoróssy, Sándor / Nagy-Vincze, Melinda / Griger, Zoltán / Dankó, Katalin / Szabó, Nóra Anna / Szekanecz, Zoltán / Szűcs, Gabriella / Szántó, Antónia / Bodoki, Levente

    Autoimmunity reviews

    2023  Volume 22, Issue 5, Page(s) 103311

    Abstract: Myalgia, myopathy and myositis are the most important types of muscle impairment in immune-mediated inflammatory arthropathies and connective tissue diseases. Multiple pathogenetic and histological changes occur in the striated muscles of these patients. ...

    Abstract Myalgia, myopathy and myositis are the most important types of muscle impairment in immune-mediated inflammatory arthropathies and connective tissue diseases. Multiple pathogenetic and histological changes occur in the striated muscles of these patients. Clinically, the most important muscle involvement is the one that causes complaints to the patients. In everyday practice, insidious symptoms present a serious problem for the clinician; in many cases, it is difficult to decide when and how to treat the muscle symptoms that are often present only subclinically. In this work, authors review the international literature on the types of muscle problems in autoimmune diseases. In scleroderma histopathological picture of muscle shows a very heterogeneous picture, necrosis and atrophy are common. In rheumatoid arthritis and systemic lupus erythematosus, myopathy is a much less defined concept, further studies are needed to describe it. According to our view, overlap myositis should be recognized as a separate entity, preferably with distinct histological and serological characteristics. More studies are needed to describe muscle impairment in autoimmune diseases which may help to explore this topic more in depth and be of clinical use.
    MeSH term(s) Humans ; Connective Tissue Diseases/complications ; Myositis ; Autoimmune Diseases/complications ; Muscular Diseases/etiology ; Lupus Erythematosus, Systemic/complications ; Arthritis, Rheumatoid/complications ; Muscle, Skeletal/pathology ; Scleroderma, Systemic/complications
    Language English
    Publishing date 2023-03-07
    Publishing country Netherlands
    Document type Journal Article ; Review
    ZDB-ID 2144145-5
    ISSN 1873-0183 ; 1568-9972
    ISSN (online) 1873-0183
    ISSN 1568-9972
    DOI 10.1016/j.autrev.2023.103311
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  3. Article ; Online: Efficacy and safety of remibrutinib, a selective potent oral BTK inhibitor, in Sjögren's syndrome: results from a randomised, double-blind, placebo-controlled phase 2 trial.

    Dörner, Thomas / Kaul, Martin / Szántó, Antónia / Tseng, Jui-Cheng / Papas, Athena S / Pylvaenaeinen, Ilona / Hanser, Malika / Abdallah, Nasri / Grioni, Andrea / Santos Da Costa, Aida / Ferrero, Enrico / Gergely, Peter / Hillenbrand, Rainer / Avrameas, Alexandre / Cenni, Bruno / Siegel, Richard M

    Annals of the rheumatic diseases

    2024  Volume 83, Issue 3, Page(s) 360–371

    Abstract: Objectives: To evaluate the safety and efficacy of remibrutinib in patients with moderate-to-severe Sjögren's syndrome (SjS) in a phase 2 randomised, double-blind trial (NCT04035668; LOUiSSE (LOU064 in Sjögren's Syndrome) study).: Methods: Eligible ... ...

    Abstract Objectives: To evaluate the safety and efficacy of remibrutinib in patients with moderate-to-severe Sjögren's syndrome (SjS) in a phase 2 randomised, double-blind trial (NCT04035668; LOUiSSE (LOU064 in Sjögren's Syndrome) study).
    Methods: Eligible patients fulfilling 2016 American College of Rheumatology/European League Against Rheumatism (EULAR) criteria for SjS, positive for anti-Ro/Sjögren's syndrome-related antigen A antibodies, with moderate-to-severe disease activity (EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) (based on weighted score) ≥ 5, EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) ≥ 5) received remibrutinib (100 mg) either one or two times a day, or placebo for the 24-week study treatment period. The primary endpoint was change from baseline in ESSDAI at week 24. Key secondary endpoints included change from baseline in ESSDAI over time, change from baseline in ESSPRI over time and safety of remibrutinib in SjS. Key exploratory endpoints included changes to the salivary flow rate, soluble biomarkers, blood transcriptomic and serum proteomic profiles.
    Results: Remibrutinib significantly improved ESSDAI score in patients with SjS over 24 weeks compared with placebo (ΔESSDAI -2.86, p=0.003). No treatment effect was observed in ESSPRI score (ΔESSPRI 0.17, p=0.663). There was a trend towards improvement of unstimulated salivary flow with remibrutinib compared with placebo over 24 weeks. Remibrutinib had a favourable safety profile in patients with SjS over 24 weeks. Remibrutinib induced significant changes in gene expression in blood, and serum protein abundance compared with placebo.
    Conclusions: These data show preliminary efficacy and favourable safety of remibrutinib in a phase 2 trial for SjS.
    MeSH term(s) Humans ; Sjogren's Syndrome/drug therapy ; Sjogren's Syndrome/complications ; Proteomics ; Antibodies ; Severity of Illness Index ; Pyrimidines
    Chemical Substances remibrutinib ; Antibodies ; Pyrimidines
    Language English
    Publishing date 2024-02-15
    Publishing country England
    Document type Randomized Controlled Trial ; Clinical Trial, Phase II ; Journal Article
    ZDB-ID 7090-7
    ISSN 1468-2060 ; 0003-4967
    ISSN (online) 1468-2060
    ISSN 0003-4967
    DOI 10.1136/ard-2023-224691
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  4. Article ; Online: A novel way to evaluate autoantibody interference in samples with mixed antinuclear antibody patterns in the HEp-2 cell based indirect immunofluorescence assay and comparison of conventional microscopic and computer-aided pattern recognition.

    Nagy, Gábor / Földesi, Róza / Csípő, István / Tarr, Tünde / Szűcs, Gabriella / Szántó, Antónia / Bubán, Tamás / Szekanecz, Zoltán / Papp, Mária / Kappelmayer, János / Antal-Szalmás, Péter

    Clinica chimica acta; international journal of clinical chemistry

    2023  Volume 553, Page(s) 117747

    Abstract: Background: A major challenge of the HEp-2 cell-based indirect immunofluorescence (IIF) assays is the correct identification of the individual anti-nuclear antibodies (ANAs) if more than one is present in a sample. We created artificial mixes by pooling ...

    Abstract Background: A major challenge of the HEp-2 cell-based indirect immunofluorescence (IIF) assays is the correct identification of the individual anti-nuclear antibodies (ANAs) if more than one is present in a sample. We created artificial mixes by pooling two different samples with a single autoantibody in different titers. Comparison of the expected and observed patterns and titers clarifies the interference between the two tested ANAs.
    Methods: Serum samples with a single homogeneous or speckled ANA pattern were serially diluted and mixed in 16 combinations, providing end-point titers of 1:5,120 to 1:80 for both patterns. These mixes were tested by a HEp-2 IIF assay and were evaluated by conventional evaluation, the EUROPattern (EPa) system and on-screen analysis.
    Results: Homogeneous pattern can alter the identification of the speckled pattern much more than vice versa, but both has an interfering effect on the other. The effect of the interfering on the tested pattern was higher if the titer of the former one was higher. The pattern recognition efficacy of conventional and the on-screen evaluation was similar and superior compared to the EPa analysis.
    Conclusions: The application of artificial mixed samples can help the evaluation of the efficacy of manual and computer-aided ANA HEp-2 pattern recognition.
    MeSH term(s) Humans ; Antibodies, Antinuclear ; Autoantibodies ; Fluorescent Antibody Technique, Indirect ; Computers ; Autoimmune Diseases
    Chemical Substances Antibodies, Antinuclear ; Autoantibodies
    Language English
    Publishing date 2023-12-26
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 80228-1
    ISSN 1873-3492 ; 0009-8981
    ISSN (online) 1873-3492
    ISSN 0009-8981
    DOI 10.1016/j.cca.2023.117747
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  5. Article ; Online: The Imbalance of Circulating Follicular T Helper Cell Subsets in Primary Sjögren's Syndrome Associates With Serological Alterations and Abnormal B-Cell Distribution.

    Szabó, Krisztina / Jámbor, Ilona / Szántó, Antónia / Horváth, Ildikó Fanny / Tarr, Tünde / Nakken, Britt / Szodoray, Peter / Papp, Gábor

    Frontiers in immunology

    2021  Volume 12, Page(s) 639975

    Abstract: Since B-cell hyperactivity and pathologic antibody response are key features in the immunopathogenesis of primary Sjögren's syndrome (pSS), the role of follicular T helper ( ... ...

    Abstract Since B-cell hyperactivity and pathologic antibody response are key features in the immunopathogenesis of primary Sjögren's syndrome (pSS), the role of follicular T helper (T
    MeSH term(s) Adult ; Aged ; Autoantibodies/immunology ; B-Lymphocytes/immunology ; CD40 Antigens/immunology ; Cell Differentiation/immunology ; Female ; Flow Cytometry/methods ; Humans ; Interleukins/immunology ; Male ; Middle Aged ; Sjogren's Syndrome/immunology ; T-Lymphocytes, Helper-Inducer/immunology ; T-Lymphocytes, Regulatory/immunology
    Chemical Substances Autoantibodies ; CD40 Antigens ; Interleukins ; interleukin-21 (MKM3CA6LT1)
    Language English
    Publishing date 2021-03-19
    Publishing country Switzerland
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2021.639975
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  6. Article ; Online: Interactions between the NLRP3-Dependent IL-1β and the Type I Interferon Pathways in Human Plasmacytoid Dendritic Cells.

    Bencze, Dóra / Fekete, Tünde / Pfliegler, Walter / Szöőr, Árpád / Csoma, Eszter / Szántó, Antónia / Tarr, Tünde / Bácsi, Attila / Kemény, Lajos / Veréb, Zoltán / Pázmándi, Kitti

    International journal of molecular sciences

    2022  Volume 23, Issue 20

    Abstract: Generally, a reciprocal antagonistic interaction exists between the antiviral type I interferon (IFN) and the antibacterial nucleotide-binding oligomerization domain (NOD)-like receptor pyrin domain containing 3 (NLRP3)-dependent IL-1β pathways that can ... ...

    Abstract Generally, a reciprocal antagonistic interaction exists between the antiviral type I interferon (IFN) and the antibacterial nucleotide-binding oligomerization domain (NOD)-like receptor pyrin domain containing 3 (NLRP3)-dependent IL-1β pathways that can significantly shape immune responses. Plasmacytoid dendritic cells (pDCs), as professional type I IFN-producing cells, are the major coordinators of antiviral immunity; however, their NLRP3-dependent IL-1β secretory pathway is poorly studied. Our aim was to determine the functional activity of the IL-1β pathway and its possible interaction with the type I IFN pathway in pDCs. We found that potent nuclear factor-kappa B (NF-κB) inducers promote higher levels of pro-IL-1β during priming compared to those activation signals, which mainly trigger interferon regulatory factor (IRF)-mediated type I IFN production. The generation of cleaved IL-1β requires certain secondary signals in pDCs and IFN-α or type I IFN-inducing viruses inhibit IL-1β production of pDCs, presumably by promoting the expression of various NLRP3 pathway inhibitors. In line with that, we detected significantly lower IL-1β production in pDCs of psoriasis patients with elevated IFN-α levels. Collectively, our results show that the NLRP3-dependent IL-1β secretory pathway is inducible in pDCs; however, it may only prevail under inflammatory conditions, in which the type I IFN pathway is not dominant.
    MeSH term(s) Humans ; NLR Family, Pyrin Domain-Containing 3 Protein/metabolism ; Interferon Type I/metabolism ; NF-kappa B/metabolism ; Signal Transduction ; Interleukin-1beta/metabolism ; Dendritic Cells ; Interferon-alpha/metabolism ; Antiviral Agents/metabolism ; Interferon Regulatory Factors/metabolism ; Anti-Bacterial Agents/metabolism ; Nucleotides/metabolism ; Inflammasomes/metabolism
    Chemical Substances NLR Family, Pyrin Domain-Containing 3 Protein ; Interferon Type I ; NF-kappa B ; Interleukin-1beta ; Interferon-alpha ; Antiviral Agents ; Interferon Regulatory Factors ; Anti-Bacterial Agents ; Nucleotides ; Inflammasomes
    Language English
    Publishing date 2022-10-12
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms232012154
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  7. Article ; Online: Regulation of RLR-Mediated Antiviral Responses of Human Dendritic Cells by mTOR.

    Fekete, Tünde / Ágics, Beatrix / Bencze, Dóra / Bene, Krisztián / Szántó, Antónia / Tarr, Tünde / Veréb, Zoltán / Bácsi, Attila / Pázmándi, Kitti

    Frontiers in immunology

    2020  Volume 11, Page(s) 572960

    Abstract: To detect replicating viruses, dendritic cells (DCs) utilize cytoplasmic retinoic acid inducible gene-(RIG) I-like receptors (RLRs), which play an essential role in the subsequent activation of antiviral immune responses. In this study, we aimed to ... ...

    Abstract To detect replicating viruses, dendritic cells (DCs) utilize cytoplasmic retinoic acid inducible gene-(RIG) I-like receptors (RLRs), which play an essential role in the subsequent activation of antiviral immune responses. In this study, we aimed to explore the role of the mammalian target of rapamycin (mTOR) in the regulation of RLR-triggered effector functions of human monocyte-derived DCs (moDCs) and plasmacytoid DCs (pDCs). Our results show that RLR stimulation increased the phosphorylation of the mTOR complex (mTORC) 1 and mTORC2 downstream targets p70S6 kinase and Akt, respectively, and this process was prevented by the mTORC1 inhibitor rapamycin as well as the dual mTORC1/C2 kinase inhibitor AZD8055 in both DC subtypes. Furthermore, inhibition of mTOR in moDCs impaired the RLR stimulation-triggered glycolytic switch, which was reflected by the inhibition of lactate production and downregulation of key glycolytic genes. Blockade of mTOR diminished the ability of RLR-stimulated moDCs and pDCs to secret type I interferons (IFNs) and pro-inflammatory cytokines, while it did not affect the phenotype of DCs. We also found that mTOR blockade decreased the phosphorylation of Tank-binding kinase 1 (TBK1), which mediates RLR-driven cytokine production. In addition, rapamycin abrogated the ability of both DC subtypes to promote the proliferation and differentiation of IFN-y and Granzyme B producing CD8 + T cells. Interestingly, AZD8055 was much weaker in its ability to decrease the T cell proliferation capacity of DCs and was unable to inhibit the DC-triggered production of IFN-y and Granyzme B by CD8 + T cells. Here we demonstrated for the first time that mTOR positively regulates the RLR-mediated antiviral activity of human DCs. Further, we show that only selective inhibition of mTORC1 but not dual mTORC1/C2 blockade suppresses effectively the T cell stimulatory capacity of DCs that should be considered in the development of new generation mTOR inhibitors and in the improvement of DC-based vaccines.
    MeSH term(s) Antineoplastic Agents/pharmacology ; CD8-Positive T-Lymphocytes/immunology ; Cell Differentiation ; Cell Line ; Cell Proliferation ; Cells, Cultured ; DEAD Box Protein 58/metabolism ; Dendritic Cells/immunology ; Humans ; Interferon Type I/metabolism ; Mechanistic Target of Rapamycin Complex 1/metabolism ; Monocytes/immunology ; Morpholines/pharmacology ; Protein Serine-Threonine Kinases/metabolism ; Receptors, Immunologic/metabolism ; Signal Transduction ; TOR Serine-Threonine Kinases/metabolism ; Vaccines/immunology ; Virus Diseases/immunology
    Chemical Substances Antineoplastic Agents ; Interferon Type I ; Morpholines ; Receptors, Immunologic ; Vaccines ; (5-(2,4-bis((3S)-3-methylmorpholin-4-yl)pyrido(2,3-d)pyrimidin-7-yl)-2-methoxyphenyl)methanol (970JJ37FPW) ; Mechanistic Target of Rapamycin Complex 1 (EC 2.7.11.1) ; Protein Serine-Threonine Kinases (EC 2.7.11.1) ; TBK1 protein, human (EC 2.7.11.1) ; TOR Serine-Threonine Kinases (EC 2.7.11.1) ; RIGI protein, human (EC 3.6.1.-) ; DEAD Box Protein 58 (EC 3.6.4.13)
    Keywords covid19
    Language English
    Publishing date 2020-09-11
    Publishing country Switzerland
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2020.572960
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  8. Article ; Online: Characterization and Comparison of Patient Subgroups Suspicious for IgG4-Related Disease and Malignant Lymphoma in Patients Followed-up for Sjögren's Syndrome.

    Szántó, Antónia / Szabó, Katalin / Nagy, Gábor / Molnár, Csaba / Zeher, Margit

    Pathology oncology research : POR

    2016  Volume 22, Issue 3, Page(s) 579–585

    Abstract: Differential diagnosis of patients with Sjögren's syndrome (SS), IgG4-related disease (IgG4-RD) and SS patients having high risk for lymphoma (LHR) can be challenging. Some patients with IgG4-RD might be misdiagnosed as having SS. There are special ... ...

    Abstract Differential diagnosis of patients with Sjögren's syndrome (SS), IgG4-related disease (IgG4-RD) and SS patients having high risk for lymphoma (LHR) can be challenging. Some patients with IgG4-RD might be misdiagnosed as having SS. There are special symptoms of SS that raise the possibility of IgG4-RD whereas other symptoms identify patients as having LHR. The purpose of this study was to characterize and compare patients with SS, possible IgG4-RD and SS patients with LHR. Sixty-five SS patients were divided into 4 subgroups according to having possible IgG4-RD (n = 15), LHR (n = 16), eligible for both aforementioned groups (n = 20) and not eligible for either group (n = 14), respectively. Four patients fulfilled the diagnostic criteria for IgG4-RD. The serum levels of IgG4 were significantly higher in patients suspicious for IgG4-RD compared to that of LHR patients (0.46 g/l vs. 0.12 g/l, p = 0.032). Shared features of the patient groups (salivary gland swelling (SGS) and lymphadenopathy), were separately analysed: SGS patients had higher IgG4/IgG ratio (p = 0.036), lymphadenopathic patients had higher IgG4 levels (p = 0.042). Some patients may be "hidden" under the diagnosis of SS. Although patients with LHR and patients with possible IgG4-RD share some symptoms, they differ significantly regarding IgG4 levels and IgG4/IgG ratio.
    MeSH term(s) Diagnosis, Differential ; Female ; Humans ; Immunoglobulin G/metabolism ; Lymphoma/diagnosis ; Lymphoma/metabolism ; Male ; Sjogren's Syndrome/diagnosis ; Sjogren's Syndrome/metabolism
    Chemical Substances Immunoglobulin G
    Language English
    Publishing date 2016-07
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 1375979-6
    ISSN 1532-2807 ; 1219-4956
    ISSN (online) 1532-2807
    ISSN 1219-4956
    DOI 10.1007/s12253-016-0041-1
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  9. Article ; Online: Clinical course, prognosis, and cause of death in primary Sjögren's syndrome.

    Horvath, Ildiko Fanny / Szanto, Antonia / Papp, Gabor / Zeher, Margit

    Journal of immunology research

    2014  Volume 2014, Page(s) 647507

    Abstract: The aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosed with primary Sjögren's syndrome (pSS) between 1975 and 2010. The patients were followed up for 11.4± ... ...

    Abstract The aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosed with primary Sjögren's syndrome (pSS) between 1975 and 2010. The patients were followed up for 11.4±6.2 years. We evaluated the clinical and laboratory features, and assessed their influence on the time of diagnosis, survival, and mortality ratios, and compared them within subgroups defined by gender, glandular and extraglandular manifestations (EGMs), associated diseases, and immunoserological abnormalities. The most frequent EGMs were polyarthritis, Raynaud's phenomenon, and vasculitis among our patients; the most common associated disease was thyroiditis. During the follow-up period, 51 patients died; the median survival time was 33.71 years. Our results revealed a negative effect of cryoglobulinemia on survival ratios; additionally, the presence of vasculitis and lymphoproliferative diseases at the time of diagnosis increased the risk of mortality. The development of vasculitis was the most powerful predictor of mortality. Mortality in the group of patients with extraglandular symptoms was two- to threefold higher than in the glandular group. Attention is drawn to the importance of close monitoring and targeted diagnostic approaches in those pSS subgroups with obviously increased mortality risk.
    MeSH term(s) Adolescent ; Adult ; Aged ; Cause of Death ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Male ; Middle Aged ; Prognosis ; Raynaud Disease/pathology ; Retrospective Studies ; Risk Factors ; Sjogren's Syndrome/immunology ; Sjogren's Syndrome/mortality ; Sjogren's Syndrome/pathology ; Survival Analysis ; Thyroiditis/pathology ; Vasculitis/pathology
    Language English
    Publishing date 2014-05-20
    Publishing country Egypt
    Document type Journal Article
    ZDB-ID 2817541-4
    ISSN 2314-7156 ; 2314-8861
    ISSN (online) 2314-7156
    ISSN 2314-8861
    DOI 10.1155/2014/647507
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  10. Article ; Online: Anti-neutrophil cytoplasmic antibody testing by indirect immunofluorescence: Computer-aided versus conventional microscopic evaluation of routine diagnostic samples from patients with vasculitis or other inflammatory diseases.

    Nagy, Gábor / Csípő, István / Tarr, Tünde / Szűcs, Gabriella / Szántó, Antónia / Bubán, Tamás / Sipeki, Nóra / Szekanecz, Zoltán / Papp, Mária / Kappelmayer, János / Antal-Szalmás, Péter

    Clinica chimica acta; international journal of clinical chemistry

    2020  Volume 511, Page(s) 117–124

    Abstract: Background: Detection of anti-neutrophil cytoplasmic antibodies (ANCA) by indirect immunofluorescence assays (IFA) is of diagnostic importance in vasculitides and some other inflammatory diseases. Automation of IFA may be beneficial in high-throughput ... ...

    Abstract Background: Detection of anti-neutrophil cytoplasmic antibodies (ANCA) by indirect immunofluorescence assays (IFA) is of diagnostic importance in vasculitides and some other inflammatory diseases. Automation of IFA may be beneficial in high-throughput clinical laboratories. An analytical appraisal of the EUROPattern (EPa) automated microscope and image analysis system has not been reported in a routine clinical laboratory setting testing samples from both vasculitis and non-vasculitis patients.
    Methods: Results of EPa and on-screen ANCA pattern recognition of 568 consecutive routine serum samples were compared to those of conventional visual evaluation.
    Results: Agreement of discrimination between negative and non-negative samples was 86.1% comparing EPa and conventional reading, and it increased to 96.7% after on-screen user validation. Importantly, from the 334 samples classified as negative by EPa 328 (98.2%) were also negative by conventional evaluation. Pattern recognition showed 'moderate' agreement between classical microscopic and EPa analysis (κ = 0.446) and 'very good' agreement after user validation (κ = 0.900). Misclassification by EPa was dominantly due to the presence of anti-nuclear/cytoplasmic antibodies (incorrect pattern, 80/568) and the lower fluorescence cut-off of the automated microscope (false positives, 73/568).
    Conclusions: Automated ANCA testing by EPa is a reliable alternative of classical microscopic evaluation, though classification of sera needs correction by trained personnel during on-screen validation.
    MeSH term(s) Antibodies, Antineutrophil Cytoplasmic ; Antibodies, Antinuclear ; Computers ; Fluorescent Antibody Technique, Indirect ; Humans ; Vasculitis/diagnosis
    Chemical Substances Antibodies, Antineutrophil Cytoplasmic ; Antibodies, Antinuclear
    Language English
    Publishing date 2020-09-28
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 80228-1
    ISSN 1873-3492 ; 0009-8981
    ISSN (online) 1873-3492
    ISSN 0009-8981
    DOI 10.1016/j.cca.2020.09.031
    Database MEDical Literature Analysis and Retrieval System OnLINE

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