Seminars in arthritis and rheumatism最新文献

{"title":"Clinically relevant differences between primary Raynaud's phenomenon and secondary to connective tissue disease","authors":"Stefano Di Donato ,&nbsp;Suiyuan Huang ,&nbsp;John D Pauling ,&nbsp;Francesco Del Galdo ,&nbsp;Maya Sabbagh ,&nbsp;Dinesh Khanna ,&nbsp;Michael Hughes","doi":"10.1016/j.semarthrit.2024.152521","DOIUrl":"10.1016/j.semarthrit.2024.152521","url":null,"abstract":"<div><h3>Objectives</h3><p>Raynaud's phenomenon (RP) is a symptom complex associated with digital vascular compromise. Our aim was to examine for clinically relevant differences between primary RP (PRP) and secondary RP (SRP) to connective tissue disease.</p></div><div><h3>Methods</h3><p>We report cross-sectional results from the Patient Survey of experiences of Raynaud's Phenomenon (PASRAP), which aimed to explore the broad-ranging impact of RP. The survey was widely distributed online including via social medial. Participation was voluntary and responses were anonymous.</p></div><div><h3>Results</h3><p>1229 respondents completed PASRAP with self-reported RP: PRP 218 (17.7 %) and SRP 1011 (82.3 %) of which 903 (92.9 %) Systemic Sclerosis. The mean (SD) age was significantly lower in respondents with PRP (41.7 [11.8] vs 54.2 [12.4] years, <em>P</em>&lt;0.0001). During attacks, more subjects with SRP reported cyanotic colour changes (92.2 % vs 86.5 %, <em>P</em>=0.0089). Patients with PRP experienced more pain (72.1 % vs 55.9 %, <em>P</em>&lt;0.0001), numbness (80.3 % vs 69.4 %, <em>P</em>=0.0016), stinging/throbbing (93.4 % vs 80.8 %, <em>P</em>&lt;0.0001), and tingling (84.0 % vs 77.5 %, <em>P</em>=0.0345). Only half of respondents’ symptoms were adequately controlled by their current medication(s), more commonly in SRP (55.2 % vs 45.2 %, <em>P</em>=0.0084). There were important differences in the triggers, number, and seasonal variation of RP attacks.</p></div><div><h3>Conclusion</h3><p>There are clinically relevant differences between PRP and SRP concerning the multifaceted lived patient experience of RP. Neurosensory symptoms are more common in PRP. Patients with SRP are older and present with more colour changes, overrepresented by cyanosis, and with less complete resolution of symptoms between attacks. These data provide novel insights for future RP clinical trial design.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"68 ","pages":"Article 152521"},"PeriodicalIF":4.6,"publicationDate":"2024-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141846274","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Muscle biopsy practices in the evaluation of idiopathic inflammatory myopathies: An international survey of expert clinicians","authors":"Samuel Maxwell ,&nbsp;Laura Ross ,&nbsp;Shereen Oon ,&nbsp;Ian P. Wicks ,&nbsp;Jessica Day","doi":"10.1016/j.semarthrit.2024.152519","DOIUrl":"10.1016/j.semarthrit.2024.152519","url":null,"abstract":"<div><h3>Background</h3><p>Muscle biopsy is an important test in the evaluation of individuals with suspected myopathy, including those with suspected idiopathic inflammatory myopathy (IIM). Various approaches, including open surgical biopsy, needle biopsy and conchotome forceps, have been reported. However the real-world utilisation of these approaches remains unclear. There are no established guidelines for the use of muscle biopsy, or selection of biopsy technique, in investigating IIM and international practices are not well-documented. This study describes current approaches to muscle biopsy amongst clinicians with expertise in IIM.</p></div><div><h3>Methods</h3><p>A survey regarding muscle biopsy practices was disseminated among members of the International Myositis Assessment and Clinical Studies (IMACS) group. Data were analysed using descriptive statistics.</p></div><div><h3>Results</h3><p>One-hundred and sixteen clinicians completed the survey, primarily rheumatologists. Open surgical biopsy was the most commonly employed technique (74.5 %), followed by needle (11.3 %) and conchotome (9.4 %) approaches. Clinical examination was the most common method of muscle selection, with 85.2 % of respondents reporting they ‘always or almost always’ relied on it. MRI and electromyography were also frequently utilised for muscle selection (51.9 %, 45.4 % respectively). There was variability in the perceived utility of muscle biopsy in certain clinical contexts, such as presence of myositis specific antibodies or cutaneous manifestations of dermatomyositis. While respondents generally reported low complication rates following muscle biopsy, non-diagnostic histopathology was commonly reported, regardless of procedural approach.</p></div><div><h3>Conclusion</h3><p>Clinicians managing IIM report muscle biopsy to be well tolerated however, non-diagnostic results are common. Substantial heterogeneity regarding perceived indications for biopsy, procedural approaches, and muscle selection strategies were observed within this expert group. Future research is needed to establish best practice and determine the role of muscle biopsy in the context of continued advancements in serological profiling of IIM.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"68 ","pages":"Article 152519"},"PeriodicalIF":4.6,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0049017224001598/pdfft?md5=c37a42ae131cc948916a9214c57b31d7&pid=1-s2.0-S0049017224001598-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141701238","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk of incident gout following exposure to recombinant zoster vaccine in US adults aged ≥50 years","authors":"Chengchen Zhang ,&nbsp;Alejandro Amill-Rosario ,&nbsp;Abree Johnson ,&nbsp;Haeyoung Lee ,&nbsp;O'Mareen Spence ,&nbsp;Driss Oraichi ,&nbsp;Harry Seifert ,&nbsp;Valentine Franck ,&nbsp;Susan Gamble ,&nbsp;Huifeng Yun ,&nbsp;Susan dosReis","doi":"10.1016/j.semarthrit.2024.152515","DOIUrl":"10.1016/j.semarthrit.2024.152515","url":null,"abstract":"<div><h3>Objective</h3><p>Assess the risk of incident gout following exposure to recombinant zoster vaccine (RZV).</p></div><div><h3>Methods</h3><p>This case-only, self-controlled risk interval study included a cohort of US fee-for-service Medicare (Part A, B, and D) beneficiaries aged ≥65 years. The exposure was receipt of at least one dose of the two-dose RZV regimen in 2018 or 2019. The risk and control windows were days 1–30 and days 31–60, respectively, following vaccination. Incident gout was defined as the first episode of gout during the risk or control window, with no evidence of gout in the last 365 days. We estimated the relative risk (RR) and 95 % confidence interval (CI) of incident gout in the risk window relative to the control window, using conditional Poisson regression models. Sensitivity analyses included a dose-compliant subanalysis of individuals who received dose 2 60–183 days after dose 1; dose-specific analysis; seasonality adjustment; and COVID-19 adjustment for potential detection bias due to the pandemic.</p></div><div><h3>Results</h3><p>The 1290 RZV-exposed individuals with incident gout were primarily White (86.98 %), male (61.16 %), and aged 70–79 years (55.82 %). The RR of incident gout was 1.00 (95 % CI 0.90, 1.12). In the dose-compliant sensitivity analysis (<em>n</em> = 959 cases of incident gout), the RR of incident gout was 0.99 (95 % CI 0.87, 1.13). The findings were unchanged in the dose-specific, seasonality, and COVID-19 sensitivity analyses.</p></div><div><h3>Conclusion</h3><p>The findings suggest that RZV is not significantly associated with an increased risk of incident gout in the Medicare population aged ≥65 years.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"68 ","pages":"Article 152515"},"PeriodicalIF":4.6,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141693562","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Real-world application of the pediatric Glucocorticoid Toxicity Index in childhood-onset lupus","authors":"Emily Zhang ,&nbsp;Sarah Capponi ,&nbsp;Rebecca Scobell ,&nbsp;Gabrielle Alonzi ,&nbsp;Madeline Hlobik ,&nbsp;Ankana Daga ,&nbsp;Esra Meidan ,&nbsp;Holly Wobma ,&nbsp;Liyoung Kim ,&nbsp;Lauren A. Henderson ,&nbsp;Siobhan Case ,&nbsp;Peter A. Nigrovic ,&nbsp;John H. Stone ,&nbsp;Karen H. Costenbader ,&nbsp;Mary Beth F. Son ,&nbsp;Joyce C. Chang","doi":"10.1016/j.semarthrit.2024.152516","DOIUrl":"10.1016/j.semarthrit.2024.152516","url":null,"abstract":"<div><h3>Objectives</h3><p>The pediatric Glucocorticoid Toxicity Index (pGTI) is a new, pediatric-specific tool to quantify glucocorticoid (GC)-related morbidity in children. We evaluated the feasibility and construct validity of retrospective pGTI scoring in patients with pediatric-onset systemic lupus erythematosus (pSLE) and identified risk factors for cumulative toxicity.</p></div><div><h3>Methods</h3><p>We conducted a retrospective cohort study of patients with pSLE treated with GCs at two pediatric centers (1999–2023). GC exposure was estimated using interval-averaged oral prednisone-equivalent dose and cumulative prednisone-equivalent dose. We scored change in GC toxicity every 6 months (±2) using a modified pGTI including 7 of 10 domains. We calculated the Cumulative Worsening Score (CWS), a continuous summation of toxicity accrued. Mixed effects linear regression was used to identify factors associated with CWS.</p></div><div><h3>Results</h3><p>There were 126 patients with pSLE, including 88 with nephritis, with a median of 6 visits/patient. Nearly half (47 %) experienced toxicity in the Blood Pressure domain. Other common toxicities were mood disturbance (25 %), followed by increased body mass index (BMI), striae, and sleep disturbance (21 % each). Decreased growth velocity was observed in 18 %. There was modest correlation between cumulative GC dose and CWS (rho 0.3; <em>p</em> &lt; 0.01). Greater cumulative toxicity was associated with younger age, elevated BMI, and rituximab use at the time of GC initiation, albeit indications for the latter were not captured.</p></div><div><h3>Conclusions</h3><p>Patients with pSLE experience a high burden of GC toxicity, particularly related to blood pressure, BMI, sleep, and growth. Standardized, pediatric-specific GC toxicity assessment is feasible in real-world settings and can facilitate evaluation of strategies to reduce morbidity in children requiring chronic GC treatment.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"68 ","pages":"Article 152516"},"PeriodicalIF":4.6,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141708440","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Anti-HMGCR myopathy: Diversity of clinical presentations in a national cohort in New Zealand","authors":"Ke Li Chow ,&nbsp;Paula Elizabeth Keating ,&nbsp;Kamal Solanki ,&nbsp;Mark Sapsford ,&nbsp;Karen Lindsay ,&nbsp;John Liston O'Donnell","doi":"10.1016/j.semarthrit.2024.152522","DOIUrl":"10.1016/j.semarthrit.2024.152522","url":null,"abstract":"<div><h3>Aims</h3><p>We describe the varied clinical presentations, barriers in diagnosis and outcomes of anti-HMGCR myopathy in a large national cohort.</p></div><div><h3>Methods</h3><p>Adults found positive for serum anti-HMGCR autoantibodies via line blot or enzyme-immunoassay followed by immunoprecipitation were included in the study.</p></div><div><h3>Results</h3><p>Of 75 patients identified, the records of 72 (96 %) described weakness as the presenting symptom. The records of 65 gave a reliable description of proximal weakness. In 22/65 (33.8 %) the weakness was described as predominantly or solely lower limb weakness. Forty-five of 75 (60 %) presented with a subacute onset (duration of symptoms &gt;4 weeks -≤6 months), whilst 22/75 (29.3 %) presented with a more indolent chronic onset (duration of symptoms &gt;6 months). Eighteen of 75 (24 %) suffered falls and 2/75 (2.7 %) had “general decline”. In three patients no weakness was described: two presented with myalgia and one with a skin rash characterized as Jessner lymphocytic skin rash. Median creatine kinase at presentation was 7337 U/L (range 1050–25,500). Muscle biopsy was performed in 38 (50.7 %). Associated malignancy was infrequent. Four patients recovered without immunosuppression. Five-year and 10-year survival was 92.7 % (95 % CI 80.6–97.4 %), and 82.5 % (95 % CI 61.2–92.8 %) respectively.</p></div><div><h3>Conclusion</h3><p>Recurrent falls, a long prodrome and dominant lower limb proximal weakness were common in this anti-HMGCR myopathy cohort. These features overlap with frailty syndrome and sporadic inclusion body myositis emphasizing the importance of considering anti-HMGCR myopathy in that clinical context. A minority of patients recover after statin withdrawal alone.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"68 ","pages":"Article 152522"},"PeriodicalIF":4.6,"publicationDate":"2024-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141692391","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk of incident gout following exposure to recombinant zoster vaccine in US adults aged ≥50 years","authors":"Sheryl A. Kluberg ,&nbsp;Andrew L. Simon ,&nbsp;Sarah M. Alam ,&nbsp;Alexander Peters ,&nbsp;Casie Horgan ,&nbsp;Dongdong Li ,&nbsp;Erick Moyneur ,&nbsp;Elizabeth Messenger-Jones ,&nbsp;Richard Platt ,&nbsp;Cheryl N. McMahill-Walraven ,&nbsp;Djeneba Audrey Djibo ,&nbsp;Kimberly Daniels ,&nbsp;Aziza Jamal-Allial ,&nbsp;Claire H. Pernar ,&nbsp;Najat J. Ziyadeh ,&nbsp;Qianli Ma ,&nbsp;Mano Selvan ,&nbsp;O'Mareen Spence ,&nbsp;Driss Oraichi ,&nbsp;Harry Seifert ,&nbsp;Huifeng Yun","doi":"10.1016/j.semarthrit.2024.152518","DOIUrl":"10.1016/j.semarthrit.2024.152518","url":null,"abstract":"<div><h3>Objective</h3><p>To assess whether recombinant zoster vaccine (RZV) is associated with an increased risk of new-onset gout among US adults aged ≥50 years.</p></div><div><h3>Methods</h3><p>We conducted a real-world, retrospective safety study with a self-controlled risk interval (SCRI) design using administrative claims data. We included health plan members aged ≥50 years with RZV exposure, followed by incident gout within 60 days. Days 1−30 following RZV exposure were considered the risk window (RW), and days 31−60 were considered the control window (CW). We estimated the risk ratio (RR) of gout in the RW versus CW, using a conditional Poisson model. The primary analysis estimated the risk of incident gout following any RZV dose. Sensitivity analyses evaluated dose 1- and dose 2-specific risks, risk among patients compliant with recommended dose spacing of 60−183 days, adjustment for seasonality, and restriction to the pre-COVID-19 era (before December 1, 2019).</p></div><div><h3>Results</h3><p>A total of 461,323 individuals received ≥1 RZV dose; we included 302 individuals (mean age 72.5 years; 66 % male) with evidence of new-onset gout within 60 days in SCRI analyses. A total of 153 (50.7 %) individuals had gout events in the RW and 149 (49.3 %) in the CW (RR 1.03; 95 % confidence interval 0.81, 1.29). All sensitivity analyses had consistent results, with no association of RZV with incident gout.</p></div><div><h3>Conclusion</h3><p>In a population of US adults aged ≥50 years, there was no statistically significant increase in the risk of gout during the 30 days immediately after RZV exposure, compared with a subsequent 30-day CW.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"68 ","pages":"Article 152518"},"PeriodicalIF":4.6,"publicationDate":"2024-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0049017224001586/pdfft?md5=032644a2a38a35b39debcbe16c27c772&pid=1-s2.0-S0049017224001586-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141701155","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Improvement of bone mineral density and new vertebral fractures during 8 years of TNF-α inhibition in patients with axial spondyloarthritis","authors":"Mark Siderius ,&nbsp;Freke Wink ,&nbsp;Stan Kieskamp ,&nbsp;Fiona Maas ,&nbsp;Reinhard Bos ,&nbsp;Frans G.M. Kroese ,&nbsp;Anneke Spoorenberg ,&nbsp;Suzanne Arends","doi":"10.1016/j.semarthrit.2024.152523","DOIUrl":"10.1016/j.semarthrit.2024.152523","url":null,"abstract":"<div><h3>Objective</h3><p>In our prospective cohort with standardized bi-annual measurements of bone mineral density (BMD) and spinal radiographs, we evaluated the long-term course of BMD and the development of radiographic vertebral fractures (VFs) during 8 years of TNFi treatment in patients with radiographic axial spondyloarthritis (r-axSpA).</p></div><div><h3>Methods</h3><p>Consecutive axSpA patients from the GLAS cohort receiving TNFi for ≥8 years were included. Patients who received anti-osteoporotic treatment were excluded. Lumbar spine (LS) BMD was assessed at baseline, 1 year and bi-annually using DEXA. Radiographic VFs were evaluated using the Genant classification.</p></div><div><h3>Results</h3><p>126 axSpA patients were included; 75 % male, mean age 42 ± 11 years, ASDAS 3.8 ± 0.8, median LS BMD Z-score -0.5 (IQR -1.4–0.7) and 20 % had radiographic VFs at baseline. Disease activity improved rapidly and sustained. LS BMD Z-score improved significantly up to 4 years compared to the previous time point and sustained thereafter. Median percentage of improvement compared to baseline was 8.9 % (2.8–15.8) and 7.2 % (2.2–14.7) after 4 and 8 years, respectively. Of 90 patients with baseline and 8-year radiographs, 14 (16 %) developed new VFs and 5 (6 %) showed an increase in severity of existing VFs. Of all 44 VFs present at 8 years, 30 % were grade 2 (<em>n</em> = 12) or grade 3 (<em>n</em> = 1).</p></div><div><h3>Conclusion</h3><p>In r-axSpA patients treated with TNFi for 8 years, LS BMD Z-score increased significantly, especially during the first 4 year of treatment. Radiographic VFs continued to develop or progressed, irrespective of improvement in BMD. Therefore, clinical attention for trabecular bone loss is important in daily clinical practice.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"68 ","pages":"Article 152523"},"PeriodicalIF":4.6,"publicationDate":"2024-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S004901722400163X/pdfft?md5=81d25b871991a960a392b060f9f955f3&pid=1-s2.0-S004901722400163X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141630206","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Commentary on IgG4-related uveitis. A French cohort and literature review","authors":"Mustafa Ekici ,&nbsp;Sibel Kadayıfçılar ,&nbsp;Figen Bezci ,&nbsp;Ömer Karadağ","doi":"10.1016/j.semarthrit.2024.152513","DOIUrl":"10.1016/j.semarthrit.2024.152513","url":null,"abstract":"","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"69 ","pages":"Article 152513"},"PeriodicalIF":4.6,"publicationDate":"2024-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141634485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Response to commentary on IgG4-related uveitis. A French cohort and literature review","authors":"Emily Stuchfield-Denby ,&nbsp;Benjamin De Sainte Marie ,&nbsp;Nicolas Schleinitz","doi":"10.1016/j.semarthrit.2024.152514","DOIUrl":"10.1016/j.semarthrit.2024.152514","url":null,"abstract":"","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"69 ","pages":"Article 152514"},"PeriodicalIF":4.6,"publicationDate":"2024-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141699018","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Whole phenotype of patients with systemic sclerosis and sicca manifestations: Comparison with sicca manifestations from other causes","authors":"François Zimmermann ,&nbsp;François Robin ,&nbsp;Elisabeth Diot ,&nbsp;Aurore Bleuzen ,&nbsp;Sandrine Jousse-Joulin ,&nbsp;Claire de Moreuil ,&nbsp;Nicolas Belhomme ,&nbsp;Claire Cazalets ,&nbsp;Ronan Garlantézec ,&nbsp;Agnès Gazzola ,&nbsp;Francisco Llamas-Gutierrez ,&nbsp;Romain Muraz ,&nbsp;Antoinette Perlat ,&nbsp;Guillaume Coiffier ,&nbsp;Alain Lescoat","doi":"10.1016/j.semarthrit.2024.152480","DOIUrl":"10.1016/j.semarthrit.2024.152480","url":null,"abstract":"<div><h3>Introduction &amp; objectives</h3><p>This study aimed to characterize the whole phenotype of Systemic sclerosis (SSc) patients with sicca symptoms, using major salivary glands Ultrasound (SGUS) parameters, minor salivary glands biopsies (mSGB) and clinical findings, and to compare these characteristics with those from patients with Sjogren's Disease (SjD), and patients with sicca manifestations from other causes.</p></div><div><h3>Methods</h3><p>Sixty SSc patients fulfilling the 2013 ACR/EULAR classification criteria and with subjective self-declared sicca symptoms were consecutively recruited and had SGUS and mSGB. Fifteen SSc patients without subjective sicca symptoms and 65 patients with sicca symptoms from other causes (including 37 SjD with no SSc).</p></div><div><h3>Results</h3><p>SSc patients with subjective sicca symptoms had frequent objective clinical (up to 83 %), histological (44 % of Focus score≥1/ mm2) and US anomalies (63 % of OMERACT ≥2). 53 % patients without subjective clinical complaint also had abnormal objective tests, suggesting the existence of a sub clinical involvement of salivary glands in SSc. SjD-SSc patients had more severe glandular involvement as compared to patients with isolated SjD and isolated Sicca-SSc patients (70%, 48,6 % and 38% of patients with OMERACT ≥2 respectively) suggesting additive impact of both diseases on glandular physiology and structure.</p></div><div><h3>Conclusion</h3><p>SjD-SSc overlap have more severe sicca features as compared to isolated sicca-SSc and isolated SjD, suggesting a specific impact of SSc on salivary gland physiology. Further translational studies are needed to identify the underlying pathways that could serve as therapeutic targets.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"68 ","pages":"Article 152480"},"PeriodicalIF":4.6,"publicationDate":"2024-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141564258","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}