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Managing fatigue in patients with primary Sjögren's syndrome: challenges and solutions.

Primary Sjögren's syndrome (pSS) patients identify fatigue as their most important symptom and the one most difficult to cope with, but there are still many challenges and few solutions to manage this debilitating symptom. Promising pharmacological treatments, such as rituximab, have failed in more stringent tests including randomized controlled trials (RCTs) and meta-analysis. While non-pharmacological interventions may be safer, less costly, and address other common comorbidities, to date only aerobic exercise seems to be effective at reducing fatigue in pSS. All interventions, pharmacologic

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… Ann Rheum Dis . 1998 ; 16 : 291 – 295 . doi: 10.1136/ard.57.5.291 PMC1752605 9741313 13. Segal B , Thomas W , Rogers T , et al. Prevalence, severity, and predictors of fatigue in subjects with primary Sjögren’s syndrome . … Ann Rheum Dis . 2008 ; 67 ( 1 ): 91 – 97 . doi: 10.1136/ard.2007.071563 17545193 60. Virkki LM , Porola P , Forsblad-D’Elia H , Valtysdottir S , Solovieva SA , Konttinen YT . …

Diagnostic spectrum and 2-year outcome in a cohort of patients with very early arthritis.

OBJECTIVES: To describe the diagnostic spectrum, arthritis persistency and clinical outcomes after 2 years in patients with inflammatory art … antibodies positive. The most common final diagnoses were undifferentiated arthritis (UA) (41.7%), rheumatoid arthritis (RA) (24.1%) and reactive arthritis (18.1%). After 2 years, the arthritis had resolved in 59% of the patients. The remaining 41.0% had persistent disease defined by disease modifying antirheumatic drug (DMARD) use (32.1%) or persisten …

A comprehensive review of Sjögren's syndrome: Classification criteria, risk factors, and signaling pathways.

Sjögren's syndrome (SS) is a chronic autoimmune disease that affects the exocrine glands and may lead to a range of systemic symptoms that impact various organs. Both innate and adaptive immune pathways might trigger the disease. Studying the signaling pathways underlying SS is crucial for enhancing diagnostic and therapeutic effectiveness. SS poses an ongoing challenge for medical professionals owing to the limited therapeutic options available. This review offers a comprehensive understanding of the intricate nature of SS, encompassing disease classification criteria, risk factors, and signa

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… Rheum. Dis. 61 6 2002 554 558 10.1136/ard.61.6.554 12006334 PMC1754137 29 Shiboski S.C. Shiboski C.H. Criswell L. … Rheum. Dis. 72 2 2013 286 292 10.1136/annrheumdis-2012-201511 22904262 115 Wang S. Shen H. Bai B. …

Safety and efficacy of low-dose intravenous arsenic trioxide in systemic lupus erythematosus: an open-label phase IIa trial (Lupsenic).

BackgroundLupus animal model has shown that arsenic trioxide (ATO), a treatment of acute promyelocytic leukaemia, could be effective in SLE. This is the first clinical study to determine the safety and efficacy of a short course of intravenous ATO in patients with active SLE.MethodsThis phase IIa, open-label, dose-escalating study enrolled 11 adult SLE patients with a non-organ threatening disease, clinically active despite conventional therapy. Patients received 10 IV infusions of ATO within 24 days. The first group received 0.10 mg/kg per injection, with dose-escalating to 0.15 mg/kg in a se

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… Arthritis Rheum. 1997;40:1725. doi: 10.1002/art.1780400928. 18. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25:1271–1277. doi: 10.1002/art.1780251101. 19. Gladman DD, Ibañez D, Urowitz MB. … Definition and initial validation of a Lupus Low Disease Activity State (LLDAS) Ann Rheum Dis. 2016;75:1615–1621. doi: 10.1136/annrheumdis-2015-207726. 29. Morand EF, Trasieva T, Berglind A, et al. …

Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations

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… Role of resident mast cell-derived tumor necrosis factor alpha Arthritis Rheum. 1995 38 3 406 412 7533494 10.1002/art.1780380318 8. … Provost TT Watson R Cutaneous manifestations of Sjogren’s syndrome Rheum Dis Clin North Am. 1992 18 3 609 616 1496164 20. …

Mixed cryoglobulinemia.

Mixed cryoglobulinemia (MC), type II and type III, refers to the presence of circulating cryoprecipitable immune complexes in the serum and manifests clinically by a classical triad of purpura, weakness and arthralgias. It is considered to be a rare disorder, but its true prevalence remains unknown. The disease is more common in Southern Europe than in Northern Europe or Northern America. The prevalence of 'essential' MC is reported as approximately 1:100,000 (with a female-to-male ratio 3:1), but this term is now used to refer to a minority of MC patients only. MC is characterized by variable

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… (modified from [ 24 ], Ferri C et al , Sem Arthritis Rheum 2004, 33:355–74.). Cryoglobulinemia is usually classified into three subgroups [ 4 ] according to Ig composition (Table 1 ): type I cryoglobulinemia is composed of only one isotype or subclass of immunoglobulin. … Ann Rheum Dis 2008 67 283 7 17644544 10.1136/ard.2006.065565 Ferri C Moriconi L Gremignai G Migliorini P Paleologo G Fosella PV Bombardieri S Treatment of renal involvement in essential mixed cryoglobulinemia with prolonged plasma exchance Nephron 1986 43 246 51 3736735 10.1159/000183849 Moriconi L …

Evolving cognition of the JAK-STAT signaling pathway: autoimmune disorders and cancer.

The Janus kinase (JAK) signal transducer and activator of transcription (JAK-STAT) pathway is an evolutionarily conserved mechanism of transmembrane signal transduction that enables cells to communicate with the exterior environment. Various cytokines, interferons, growth factors, and other specific molecules activate JAK-STAT signaling to drive a series of physiological and pathological processes, including proliferation, metabolism, immune response, inflammation, and malignancy. Dysregulated JAK-STAT signaling and related genetic mutations are strongly associated with immune activation and c

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… Rheum. Dis. 2015;74:1311–1316. doi: 10.1136/annrheumdis-2014-206028. 33. Palmroth M, et al. Tofacitinib suppresses several JAK-STAT pathways in rheumatoid arthritis in vivo and baseline signaling profile associates with treatment response. Front. … Rheum. Dis. 2022;81:1504–1514. doi: 10.1136/ard-2022-222605. 100. Pelham SJ, et al. STAT5B restrains human B-cell differentiation to maintain humoral immune homeostasis. J. Allergy Clin. Immunol. 2022;150:931–946. doi: 10.1016/j.jaci.2022.04.011. 101. Duetsch G, et al. …

Implications of Angiogenesis Involvement in Arthritis.

Angiogenesis, the growth of new blood vessels, is essential in the pathogenesis of joint inflammatory disorders such as rheumatoid arthritis (RA) and osteoarthritis (OA), facilitating the invasion of inflammatory cells and increase in local pain receptors that contribute to structural damage and pain. The angiogenic process is perpetuated by various mediators such as growth factors, primarily vascular endothelial growth factor (VEGF) and hypoxia-inducible factors (HIFs), as well as proinflammatory cytokines, various chemokines, matrix components, cell adhesion molecules, proteases, and others.…

Ангиография оптической согласованности — полезное средство для отличия первичного феномена Рейно от системного склероза и/или очень ранней болезни системного склероза.

Цель этого межсекторального исследования заключалась в сопоставлении результатов оптической ангиографии, полученной пациентами, с первичным явлением Рейно (PRP; n = 22), очень раннее заболевание системного склероза (VEDOS; n = 19), и системный склероз (SSc; 25 пациентов с ограниченным количеством SSc (lcSSc) и 13 - пациентов с диффузным резиновым SSc (dcSSc)). Целые, парафовиальные и перифовенные поверхностные капиллярные сплексы (ПХФ) плотность судна (ДГ), глубокие капиллярные сплетения VD и целые, внутренние и перипапольные VD были значительно выше в группе ПРП (p) < 0.001). В группе IcSSc п

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… Rheum. Dis. 2011;70:476–481. doi: 10.1136/ard.2010.136929. 8. Ushiyama O., Ushiyama K., Yamada T., Koarada S., Tada Y., Suzuki N., Ohta A., Nagasawa K. Retinal findings in systemic sclerosis: A comparison with nailfold capillaroscopic patterns. Ann. Rheum. … Rheum. Dis. 1977;36:152–156. doi: 10.1136/ard.36.2.152. 16. Rommel F., Siegfried F., Kurz M., Brinkmann M.P., Rothe M., Rudolf M., Grisanti S., Ranjbar M. …

Identification of a variant in NLRP3 gene in a patient with Muckle-Wells syndrome: a case report and review of literature.

BackgroundCryopyrin-associated periodic syndrome (CAPS), a rare genetic autoimmune disease, is composed of familial cold autoinflammatory syndrome (FCAs), Muckle-Wells syndrome (MWS), and neonatal onset multisystem inflammatory disease (NOMID). MWS is caused by dominantly inherited or de novo gain-of-function mutations in the NOD-like receptor 3 (NLRP3) gene. At present, there is no report about the variation of R262W in China.Case presentationWe reported a 3-year-old Chinese boy who had recurrent fever without obvious inducement, bilateral conjunctival congestion, and urticarial-like rash. La

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… Kümmerle-Deschner JB Tyrrell PN Reess F Kötter I Lohse P Girschick H Risk factors for severe Muckle-Wells syndrome Arthritis Rheum 2010 62 12 3783 3791 10.1002/art.27696 20722029 10. … Ter Haar NM Oswald M Jeyaratnam J Anton J Barron KS Brogan PA Recommendations for the management of autoinflammatory diseases Ann Rheum Dis 2015 74 9 1636 1644 10.1136/annrheumdis-2015-207546 26109736 30. …

ANCA-Associated Glomerulonephritis: Diagnosis and Therapy Proceedings of the Henry Shavelle Lectureship.

BackgroundAnti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) frequently affects the kidney. Glomerulonephritis (GN) in AAV, ANCA-GN, not only dictates therapeutic decisions but is also of relevance for overall survival influencing the risk of cardiovascular disease and serious infections.SummaryA diagnosis of ANCA-GN includes laboratory investigations including urinalysis and a thorough assessment of potential organ involvement. A kidney biopsy can be performed to ascertain the diagnosis but has an additional prognostic relevance and tools have been established to predict

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… Semin Arthritis Rheum . 2022 ; 55 : 152010 . 35486995 10.1016/j.semarthrit.2022.152010 83. Patel NJ , Jayne DRW , Merkel PA , Bekker P , Zhang Y , Yue H , . … Ann Rheum Dis . 2018 ; 77 ( 8 ): 1150 – 6 . 29724729 10.1136/annrheumdis-2017-212768 93. Charles P , Terrier B , Perrodeau É , Cohen P , Faguer S , Huart A , . …

Higher mortality risk from gynaecological neoplasms and non-Hodgkin's lymphoma in patients with systemic lupus erythematosus: an observational study from the Spanish National Registry.

OBJECTIVE: To evaluate the impact of the different types of neoplasms and lineages on mortality of patients with SLE. METHODS: Retrospective and observational comparison of the neoplasm-related deaths in patients with SLE and the general Spanish population reported in the Spanish Hospital Discharge Database. To determine the impact of SLE on the risk of dying from each neoplasm lineage, a binary logistic regression considering age, female sex, tobacco and alcohol consumption, was performed. RESULTS: During 2016-2019, 139 531 in-hospital deaths from neoplasms were certified in Spain (91 in pa

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… Ann Rheum Dis 2007;66:815–7. 10.1136/ard.2006.067777 34. Furer V, Rondaan C, Heijstek MW, et al. Update of EULAR recommendations for vaccination in adult patients with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis 2020;79:39–52. 10.1136/annrheumdis-2019-215882 35. … Ann Rheum Dis 2023;10:ard-2023-224416. 10.1136/ard-2023-224416 37. Curry SJ, Krist AH, Owens DK, et al. Screening for cervical cancer. JAMA 2018;320:674. 10.1001/jama.2018.10897 38. Markowitz LE, Dunne EF, Saraiya M, et al. …

Taming renal inflammation: signaling pathways and therapeutic advances in lupus nephritis.

Lupus nephritis (LN), a serious complication of systemic lupus erythematosus (SLE), involves complex immune dysregulation that leads to chronic renal inflammation and progressive tissue damage. Despite decades of use of standard immunosuppressive therapy, treatment responses remain variable, and many patients experience relapses or develop end-stage renal disease. This review synthesizes emerging insights into the immunopathogenesis of LN, drawing on studies from single-cell transcriptomics, signaling pathway analyses and renal tissue immunology. It examines the role of both innate and adaptiv

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… Arthritis Rheum. 2010;62(1):222–33. 20039413 10.1002/art.27233 PMC4548300 46. … Ann Rheum Dis. 2013;72(8):1280–6. 23740227 10.1136/annrheumdis-2012-202844 48. …

Integrative multi-omics analysis reveals CXCL10-driven inflammation and TREM2 + macrophage-plasma cell survival niche as hallmarks of late-stage rheumatoid arthritis.

BACKGROUND: Rheumatoid arthritis (RA) is characterized by persistent synovial inflammation, yet the molecular mechanisms distinguishing early from late-stage disease remain incompletely elucidated. Identifying stage-specific biomarkers and pathogenic cellular interactions is crucial for precision medicine. OBJECTIVE: To comprehensively characterize the transcriptomic landscape and cellular composition of early versus late RA synovium, identify diagnostic biomarkers, and elucidate key pathogenic cell–cell interactions driving disease chronicity. METHODS: Synovial tissues from 51 RA patients (13…

Peritoneal Carcinomatosis in an Adult With Sjögren's Syndrome: A Diagnostic Dilemma Revealing a Rare Association With Primary Gastric Adenocarcinoma.

Sjögren's syndrome (SS) is a systemic autoimmune disease that mainly affects middle-aged women. It is rarely associated with solid neoplasms. We report a 60-year-old woman with a past medical history relevant for SS who was diagnosed with advanced-stage gastric adenocarcinoma upon evaluating for peritoneal carcinomatosis and succumbed two months after her original diagnosis. This case highlights the significance of considering gastrointestinal (GI) malignancy as an essential differential, particularly when evaluating patients with SS who fail conservative treatment for their GI symptoms.

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… Human Ethics Consent was obtained by all participants in this study References 1 Incidence of cancer in a nationwide population cohort of 7852 patients with primary Sjogren's syndrome in Taiwan Ann Rheum Dis Weng MY Huang YT Liu MF Lu TH 524 527 71 2012 22072014 10.1136/annrheumdis-2011-200402 2 … , retrospective, clinical study by the European Concerted Action on Sjögren's Syndrome Arthritis Rheum Voulgarelis M Dafni UG Isenberg DA Moutsopoulos HM 1765 1772 42 1999 10446879 10.1002/1529-0131(199908)42:8<1765::AID-ANR28>3.0.CO;2-V 11 Adenocarcinoma of the lung and Sjogren's syndrome: an unlikely …

Spatiotemperal Dynamics of Osteoarthritis: Преобразование значений от стенда до Бедсайда.

Остеоартрит (OA) представляет собой многогранное дегенеративное совместное расстройство, вызванное различными факторами риска, такими, как возраст, механический стресс, воспаление и метаболическое воздействие. Эти элементы способствуют его разнообразным фенотипам и эндотипам, что подчеркивает имманентную сложность болезни. Участие нескольких тканей и их взаимодействие еще больше осложняет расследование ОА. Нынешние ограничения в области технологий пространственного фенотификации в сочетании со сложной сетью многофакторных взаимодействий препятствуют обнаружению надежных ранних диагностических

Обзор различных методов совершенствования лечения пульмональной гипертензии у пациентов с системным склерозом

При системном склерозе (SSc) смертность связана главным образом с участием лёгких, для которых характерны межститиальная болезнь легких (МЛС) и легочная гипертония (PH). В SSc PH может быть вызван различными этиологиями, включая ILD, хроническую тромбоэмболическую болезнь, легочную веноклюзивную болезнь и легочную артериальную гипертонию (PAH). Основным инструментом для экранирования ПАУ является трансхуракическая эхокардиография (TTE), которая обладает чувствительностью: 90%, даже если окончательный диагноз должен быть подтвержден правильной катетеризацией сердца (RHC). Радиологическая оценка

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… Rheum. Dis. 2010 69 1809 1815 10.1136/ard.2009.114264 20551155 10. Chung L. Liu J. Parsons L. Hassoun P.M. McGoon M. Badesch D.B. Miller D.P. Nicolls M.R. Zamanian R.T. … Rheum. Dis. 2012 71 1335 1342 10.1136/annrheumdis-2011-200546 22307943 PMC3398226 29. Lammi M.R. Saketkoo L.A. Gordon J.K. Lauto P. Fagan K. Steen V.D. …

Sex Difference in Primary Sjögren Syndrome: A Medical Records Review Study.

OBJECTIVES: The aim of this study was to study clinical and biological differences between men and women with primary Sjögren syndrome (pSS) … t 4 ( p < 0.05); and higher titers of antinuclear antibody, anti-Sjögren syndrome A, anti-Ro52, and rheumatoid factor positivity ( p < 0.05) than men, whereas men with pSS had a higher prevalence of parotid enlargement and interstitial lung disease ( p < 0.05). CONCLUSIONS: Women with pSS are associated with more dryness, cytopenia, hypocomplementemia, …

What Are the Peripheral Blood Determinants for Increased Osteoclast Formation in the Various Inflammatory Diseases Associated With Bone Loss?

Local priming of osteoclast precursors (OCp) has long been considered the main and obvious pathway that takes place in the human body, where … ogenesis using peripheral blood from patients with bone loss diseases in prevalent diseases such as rheumatoid arthritis, osteoporosis, periodontitis, and cancer-related osteopenia unequivocally point toward an intrinsically increased osteoclast formation and activation. In particular, such increased osteoclastogenesis already takes place without the ad …

Relapsing polychondritis: state of the art on clinical practice guidelines.

Due to the rarity of relapsing polychondritis (RP), many unmet needs remain in the management of RP. Here, we present a systematic review of clinical practice guidelines (CPGs) published for RP, as well as a list of the most striking unmet needs for this rare disease. We carried out a systematic search in PubMed and Embase based on controlled terms (medical subject headings and Emtree) and keywords of the disease and publication type (CPGs). The systematic literature review identified 20 citations, among which no CPGs could be identified. We identified 11 main areas with unmet needs in the fie

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… Arthritis Rheum 2009;61:577–82. 10.1002/art.24366 20. Moulis G, Pugnet G, Costedoat-Chalumeau N, et al. Efficacy and safety of biologics in relapsing polychondritis: a French national multicentre study. Ann Rheum Dis 2018;77:72–78. 10.1136/annrheumdis-2017-212705 21. THT L, Far N, Young AL. … Arthritis Rheum 1713;2001:1713–5. 44. Matzkies FG, Manger B, Schmitt-Haendle M, et al. Severe septicaemia in a patient with polychondritis and sweet's syndrome after initiation of treatment with infliximab. Ann Rheum Dis 2003;62:81–2. 10.1136/ard.62.1.81 …