Journal of Scleroderma and Related Disorders最新文献

筛选
英文 中文
Challenging diagnosis of renal failure associated with severe neurological symptoms in a patient with mixed connective tissue disease. 混合结缔组织病患者肾功能衰竭伴有严重神经症状的诊断具有挑战性。
IF 2
Journal of Scleroderma and Related Disorders Pub Date : 2023-02-01 Epub Date: 2022-06-13 DOI: 10.1177/23971983221099847
Clothilde Gros, Olivier Fogel, Idris Boudhabhay, Charlotte Debiais, Jean-Paul Duong Van Huyen, Aurélie Hummel, Yannick Allanore, Jérôme Avouac
{"title":"Challenging diagnosis of renal failure associated with severe neurological symptoms in a patient with mixed connective tissue disease.","authors":"Clothilde Gros, Olivier Fogel, Idris Boudhabhay, Charlotte Debiais, Jean-Paul Duong Van Huyen, Aurélie Hummel, Yannick Allanore, Jérôme Avouac","doi":"10.1177/23971983221099847","DOIUrl":"10.1177/23971983221099847","url":null,"abstract":"<p><p>We report the case of a patient followed for a mixed connective tissue disease with signs of systemic sclerosis and systemic lupus, who presented an acute renal failure with severe neurological symptoms (confusion, obnubilation) and hypertension. The distinction between scleroderma renal crisis and lupus nephritis was challenging and hence, the decision to use or not high dose of corticosteroids. Kidney biopsy was of major importance for the diagnosis and therapeutic strategy. The diagnosis of neurological symptoms was also made difficult given the clinical presentation and the results of imaging. Neurolupus, malignant hypertension, or posterior reversible encephalopathy syndrome were the evoked diagnosis.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 1","pages":"NP6-NP10"},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896196/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9648569","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The validity and reliability of the Swedish version of the Satisfaction with appearance scale for individuals with systemic sclerosis. 瑞典版系统性硬化症患者外貌满意度量表的效度与信度。
IF 2
Journal of Scleroderma and Related Disorders Pub Date : 2023-02-01 DOI: 10.1177/23971983221107858
Malin Mattsson, Roger Hesselstrand, Karin Gunnarsson, Elisabet Dyrsmeds, Monica Holmner, Annica Nordin, Carina Boström
{"title":"The validity and reliability of the Swedish version of the Satisfaction with appearance scale for individuals with systemic sclerosis.","authors":"Malin Mattsson,&nbsp;Roger Hesselstrand,&nbsp;Karin Gunnarsson,&nbsp;Elisabet Dyrsmeds,&nbsp;Monica Holmner,&nbsp;Annica Nordin,&nbsp;Carina Boström","doi":"10.1177/23971983221107858","DOIUrl":"https://doi.org/10.1177/23971983221107858","url":null,"abstract":"<p><strong>Background: </strong>Systemic sclerosis (SSc) can lead to visible changes in appearance which could generate concerns among patients. Thus, valid questionnaires that capture these concerns are valuable to identify and communicate appearance concerns.</p><p><strong>Objective: </strong>To determine aspects of the validity and reliability of the Swedish version of the Satisfaction with Appearance scale for individuals with SSc (SWAP-Swe in SSc).</p><p><strong>Methods: </strong>Content validity was assessed by interviews. In a cross-sectional design, construct validity was evaluated by comparing the self-reported questionnaire SWAP-Swe in SSc to the Scleroderma Health Assessment Questionnaire (SSc HAQ), Patient Health Questionnaire-8 (PHQ-8), RAND-36, modified Rodnan skin score (mRSS), disease duration and age using Spearman's rank correlations (<i>r<sub>s</sub></i> ). Internal consistency was evaluated by Cronbach's alpha coefficient and corrected item-to-total correlations. Test-retest reliability was investigated using the intraclass correlation coefficient (ICC).</p><p><strong>Results: </strong>Eleven patients and 10 health professionals participated in the assessment of content validity. For the other aspects of validity and reliability 134 patients (median age 62 years, women 81%, limited cutaneous SSc 75%) participated. Overall, the content validity was satisfactory. The SWAP-Swe in SSc correlated with SSc HAQ (HAQ-DI <i>r<sub>s</sub></i>  = 0.50, visual analogue scales <i>r<sub>s</sub></i>  = 0.24-0.41), PHQ-8 (<i>r<sub>s</sub></i>  = 0.46), RAND-36 (<i>r<sub>s</sub></i>  = -0.21 to -0.47), mRSS (<i>r<sub>s</sub></i>  = 0.28), disease duration (<i>r<sub>s</sub></i>  = -0.01) and age (<i>r<sub>s</sub></i>  = -0.15). The Cronbach's alpha coefficient was 0.92, corrected item-to-total correlations ⩾ 0.45 and the ICC 0.82.</p><p><strong>Conclusion: </strong>The SWAP-Swe in SSc showed satisfactory content validity, sufficient and good internal consistency and sufficient test-retest reliability. It was more strongly associated with self-reported questionnaires than with physician-assessed skin involvement and age, indicating that appearance concerns in SSc seem to be multidimensional as earlier reported. Our study contributes with a thorough investigation of validity and reliability including aspects that have not been investigated before. However, evaluation of more validity aspects of the SWAP-Swe in SSc is suggested.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 1","pages":"53-63"},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896191/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9648568","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Very Early Diagnosis of Systemic Sclerosis: Deciphering the heterogeneity of systemic sclerosis in the very early stages of the disease. 系统性硬化症的早期诊断:解读系统性硬化症早期阶段的异质性。
IF 1.4
Journal of Scleroderma and Related Disorders Pub Date : 2023-02-01 Epub Date: 2022-10-11 DOI: 10.1177/23971983221129211
Alain Lescoat
{"title":"Very Early Diagnosis of Systemic Sclerosis: Deciphering the heterogeneity of systemic sclerosis in the very early stages of the disease.","authors":"Alain Lescoat","doi":"10.1177/23971983221129211","DOIUrl":"10.1177/23971983221129211","url":null,"abstract":"<p><p>The early diagnosis of systemic sclerosis has been a major challenge for the scleroderma community in the past 50 years. The recent publication of the predictive value of the VEDOSS (Very Early Diagnosis of Systemic Sclerosis) criteria in the <i>Lancet Rheumatology</i> in December 2021 has provided an unprecedented insight in the early stages of the disease. This editorial discusses the main findings from this 2021 VEDOSS publication and highlights key unanswered questions to be proposed on the research agenda in very early systemic sclerosis.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 1","pages":"3-6"},"PeriodicalIF":1.4,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896197/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9648571","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Normotensive scleroderma renal crisis as the presenting symptom of systemic sclerosis sine scleroderma: A case report. 正常血压的硬皮病肾危象是无硬皮病系统性硬化症的首发症状:病例报告。
IF 2
Journal of Scleroderma and Related Disorders Pub Date : 2023-02-01 Epub Date: 2022-06-07 DOI: 10.1177/23971983221101296
Fadi Hassan, Firas Sabbah, Mohammad E Naffaa
{"title":"Normotensive scleroderma renal crisis as the presenting symptom of systemic sclerosis sine scleroderma: A case report.","authors":"Fadi Hassan, Firas Sabbah, Mohammad E Naffaa","doi":"10.1177/23971983221101296","DOIUrl":"10.1177/23971983221101296","url":null,"abstract":"<p><p>Scleroderma renal crisis is a rare but serious complication of systemic sclerosis. It is usually associated with marked hypertension and carries significant risk for morbidity and mortality. Its occurrence prior to the development of skin sclerosis is exceedingly rare. We report a case of a patient who presented with recurrent pericardial effusion and later tested positive for anti-nuclear and anti-topoisomerase antibodies. He later developed normotensive renal crisis as confirmed by kidney biopsy despite complete absence of skin involvement. To our knowledge, this is the first published case of a patient presenting with normotensive renal crisis without any skin involvement.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 1","pages":"NP1-NP5"},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896193/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10718229","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Symptomatic fractures in systemic sclerosis: A case-control study. 系统性硬化症患者的症状性骨折:病例对照研究
IF 2
Journal of Scleroderma and Related Disorders Pub Date : 2023-02-01 Epub Date: 2022-12-08 DOI: 10.1177/23971983221141271
Marília M Sampaio-Barros, Adriana B Bortoluzzo, Henrique Carriço da Silva, Ana Paula Luppino-Assad, Rosa Maria R Pereira, Percival D Sampaio-Barros
{"title":"Symptomatic fractures in systemic sclerosis: A case-control study.","authors":"Marília M Sampaio-Barros, Adriana B Bortoluzzo, Henrique Carriço da Silva, Ana Paula Luppino-Assad, Rosa Maria R Pereira, Percival D Sampaio-Barros","doi":"10.1177/23971983221141271","DOIUrl":"10.1177/23971983221141271","url":null,"abstract":"<p><p>This case-control study analyzed risk factors for symptomatic fractures in a group of 52 patients with systemic sclerosis compared with a group of 104 patients without fractures, matched for sex and age, who were attended at a single systemic sclerosis outpatient clinic from 2010 to 2020. Fractures affected predominantly vertebral (65.4%), rib (13.5%), and hip (7.7%) joints, while the mean age of fracture was 55.3 ± 9.5 years. Age at disease onset, age at diagnosis, disease duration, age at menarche, and age at menopause were similar in both groups, and 58.9% of the patients were menopausal at the time of the fracture. The presence of fractures had a significant association with densitometric osteoporosis (p < 0.001), lower weight (p = 0.032), and bone mineral index (p = 0.044), anti-RNA polymerase III (p = 0.040), use of corticosteroids (p = 0.019), and bisphosphonates (p < 0.001), as well as with densitometric T-scores of lumbar spine (p < 0.001), femoral neck (p = 0.025), and total hip (p = 0.013). Multivariate analysis showed that the variables significantly associated with fractures were high doses of corticosteroids (odds ratio = 4.10; 95% confidence interval = 1.290-13.090; p = 0.017), bisphosphonates (odds ratio = 3.91; 95% confidence interval = 1.699-8.984; p = 0.001), negative anti-Scl70 (OR = 0.34; 95% confidence interval = 0.124-0.943; p = 0.038), and lumbar T-score (odds ratio = 0.39; 95% confidence interval = 0.034-0.460; p = 0.010). In conclusion, symptomatic fractures were associated predominantly with lower bone mineral density of lumbar spine and use of high doses of corticosteroids and bisphosphonates in this cohort.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 1","pages":"79-84"},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896198/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9634278","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Prevalence of and factors independently associated with digital ischemic complications in patients with systemic sclerosis. 系统性硬化症患者中数字缺血性并发症的发生率和独立相关因素。
IF 2
Journal of Scleroderma and Related Disorders Pub Date : 2023-02-01 Epub Date: 2022-08-25 DOI: 10.1177/23971983221118720
Theerajet Guayboon, Chayawee Muangchan
{"title":"Prevalence of and factors independently associated with digital ischemic complications in patients with systemic sclerosis.","authors":"Theerajet Guayboon, Chayawee Muangchan","doi":"10.1177/23971983221118720","DOIUrl":"10.1177/23971983221118720","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the prevalence of and independent predictors for digital ischemic complications in patients with systemic sclerosis.</p><p><strong>Method: </strong>Patients enrolled in the Siriraj Systemic Sclerosis Cohort registry during 2013-2019 were classified as having or not having digital ischemic complications at the baseline and 1-year timepoints.</p><p><strong>Results: </strong>A total of 171 patients with systemic sclerosis were included. The prevalence of digital pulp loss, digital pitting scar, digital ulcer, and digital amputation at baseline and 1 year was 41.5%, 39.8%, 3.5%, 7.6% and 37.4%, 43.9%, 14.1%, 6.4%, respectively. Over half (58.5%) of overall systemic sclerosis had developed new digital ischemic complications during the 1-year follow-up. Those with digital ischemic complications at baseline were at high risk for developing new digital ischemic complications (odds ratio: 15.9). Diffuse cutaneous systemic sclerosis is associated with digital ischemic complications (odds ratio: 6.0), digital pitting scar (odds ratio: 4.9), and digital pulp loss (odds ratio: 6.4). Tendon friction rub is associated with digital pitting scar (odds ratio: 5.0). Salt-and-pepper skin appearance is associated with digital pulp loss (odds ratio: 3.0) and digital ulcer (odds ratio: 6.9). Disease duration <b>></b> 3 years is associated with digital ulcer (odds ratio: 4.4). Male gender is associated with digital ulcer (odds ratio: 5.4).</p><p><strong>Conclusion: </strong>Digital pulp loss, digital pitting scar, digital ulcer, and digital amputation were common manifestations of digital ischemic complications, and diffuse cutaneous systemic sclerosis was the strongest of the six independent predictors.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 1","pages":"43-52"},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896203/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9634279","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Ultrasound and magnetic resonance imaging of hands in systemic sclerosis: A cross-sectional analytical study of prevalence of inflammatory changes in patients with subclinical arthropathy. 系统性硬化症患者手部的超声波和磁共振成像:亚临床关节病患者炎症性病变发生率的横断面分析研究。
IF 2
Journal of Scleroderma and Related Disorders Pub Date : 2023-02-01 Epub Date: 2023-01-04 DOI: 10.1177/23971983221140673
Akash Babulal Vadher, Anindita Sinha, Shayeri Roy Choudhury, Mahesh Prakash, Muniraju Maralakunte, Tanveer Rehman, Shefali Sharma, Yashwant Kumar
{"title":"Ultrasound and magnetic resonance imaging of hands in systemic sclerosis: A cross-sectional analytical study of prevalence of inflammatory changes in patients with subclinical arthropathy.","authors":"Akash Babulal Vadher, Anindita Sinha, Shayeri Roy Choudhury, Mahesh Prakash, Muniraju Maralakunte, Tanveer Rehman, Shefali Sharma, Yashwant Kumar","doi":"10.1177/23971983221140673","DOIUrl":"10.1177/23971983221140673","url":null,"abstract":"<p><strong>Objectives: </strong>Prevalence of synovitis, tenosynovitis, erosions, acro-osteolysis and bone marrow edema in systemic sclerosis is not extensively reported. We aimed to estimate the prevalence of changes in individual joints of hands in systemic sclerosis patients.</p><p><strong>Method: </strong>A cross-sectional analytical study consisting of 34 adults (females, <i>n</i> = 32) with systemic sclerosis. Patients with clinical synovitis were excluded. All patients underwent ultrasound (US) and magnetic resonance imaging of bilateral hands.</p><p><strong>Results: </strong>On US, synovitis, tenosynovitis, erosions, and acro-osteolysis were detected in 97%, 94%, 97%, and 29% patients. Grade I synovitis observed in 67% joints-first carpometacarpal joint (55%), first metacarpophalangeal joint (54%), distal radioulnar joint (50%), and intercarpal joints (47%) were commonly affected. Erosions were common in distal phalanges (first DP72% to fifth DP39%). On magnetic resonance imaging, synovitis, tenosynovitis, erosions, and bone edema were observed in 91%, 85%, 97%, and 85% patients. Grade I synovitis was seen in 70% joints, affecting intercarpal joint (70.6%) and third metacarpophalangeal joint (52.9%) commonly. Grade I erosions were seen in 61%, affecting distal phalanges (55.8%), capitate (60.3%), and lunate (55.8%). Grade I edema was commonly affecting lunate (39%) and capitate (26%). On magnetic resonance imaging, acro-osteolysis was present in 28% (97/340) distal phalanges. Fair agreement (0.21-0.40) was noted between US and magnetic resonance imaging for synovitis and erosions.</p><p><strong>Conclusion: </strong>High prevalence of low-grade inflammation is found in systemic sclerosis patients on US and magnetic resonance imaging. Distal joint assessment in addition to proximal joints improves accurate estimation of prevalence of early arthropathy.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 1","pages":"72-78"},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896195/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9634281","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Development and validation of Hebrew version of the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument 2.0. 开发并验证希伯来语版加州大学洛杉矶分校硬皮病临床试验联盟胃肠道工具 2.0。
IF 2
Journal of Scleroderma and Related Disorders Pub Date : 2023-02-01 Epub Date: 2022-12-08 DOI: 10.1177/23971983221138712
David Ozeri, Shani Peretz, Amit Oppenheim, Abdallah Watad, Merav Lidar, Yolanda Braun-Moscovici
{"title":"Development and validation of Hebrew version of the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument 2.0.","authors":"David Ozeri, Shani Peretz, Amit Oppenheim, Abdallah Watad, Merav Lidar, Yolanda Braun-Moscovici","doi":"10.1177/23971983221138712","DOIUrl":"10.1177/23971983221138712","url":null,"abstract":"<p><strong>Aim: </strong>The aim of this study was to test the reliability of the University of California, Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract (UCLA SCTC GIT) 2.0 questionnaire in Hebrew.</p><p><strong>Methods: </strong>UCLA SCTC GIT 2.0 was translated into Hebrew using the translation-retranslation method. The Hebrew version of the UCLA SCTC GIT 2.0 and the Hebrew version of the Short Form 36 (SF-36) were administered to 19 Hebrew-speaking patients with systemic sclerosis. Internal reliability was assessed using Cronbach's alpha. The Hebrew questionnaire was then tested for external validity using Spearman's correlation coefficient. Correlations (<i>rho</i>) ⩽ 0.29 were considered small, 0.30 to 0.49 were moderate, and those ⩾0.50 were considered large. Differences were considered statistically significant at <i>p</i> < 0.05.</p><p><strong>Results: </strong>A group of 19 patients treated at Sheba Medical Center meeting the ACR/EULAR classification system for systemic sclerosis were included in the study. The mean age of the participants was 60.4 ± 12 years with a female predominance (84%). Diffuse cutaneous scleroderma accounted for 10 of the participants (54%), 7 had limited cutaneous scleroderma (36%) with 2 having an overlap syndrome (10%). The Cronbach's alpha value for the UCLA SCTC GIT 2.0 scale was 0.908 showing reliability. In addition, the UCLA SCTC GIT 2.0 showed correlation to the SF-36.</p><p><strong>Conclusion: </strong>The translation of the Hebrew UCLA SCTC GIT 2.0 scale was reliable and valid with a total Cronbach's alpha score among the participants of 0.908. Cronbach's alpha was particularly reliable in reflux, bloating, social function, and emotional well-being. Our results suggest that our Hebrew version of the UCLA SCTC GIT 2.0 scale can be used as a tool in future studies with Hebrew-speaking patients. In the abstract conclusion, it states that \"Cronbach's alpha was particularly reliable in reflux, bloating, social function, and emotional well-being.\" The related data should be listed in the results section and then an interpretation of the results should be listed in the conclusions section. Please revise.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 1","pages":"31-35"},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896201/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9648570","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Extranodal localization of non-Hodgkin's lymphoma in systemic sclerosis: A diagnostic challenge and review of the literature. 系统性硬化症中非霍奇金淋巴瘤的淋巴结外定位:诊断挑战和文献综述。
IF 2
Journal of Scleroderma and Related Disorders Pub Date : 2022-10-01 Epub Date: 2022-04-10 DOI: 10.1177/23971983221088459
Giorgio Galoppini, Beatrice Maranini, Giovanni Ciancio, Melissa Padovan, Gian Luca Casoni, Francesco Cavazzini, Roberta Gafà, Giovanni Lanza, Marcello Govoni
{"title":"Extranodal localization of non-Hodgkin's lymphoma in systemic sclerosis: A diagnostic challenge and review of the literature.","authors":"Giorgio Galoppini,&nbsp;Beatrice Maranini,&nbsp;Giovanni Ciancio,&nbsp;Melissa Padovan,&nbsp;Gian Luca Casoni,&nbsp;Francesco Cavazzini,&nbsp;Roberta Gafà,&nbsp;Giovanni Lanza,&nbsp;Marcello Govoni","doi":"10.1177/23971983221088459","DOIUrl":"10.1177/23971983221088459","url":null,"abstract":"<p><strong>Background: </strong>Systemic sclerosis is associated with an increased incidence of malignancies, in particular solid neoplasms. Hematological cancers have been also observed in autoimmune diseases, though rarely present with lung involvement. The latter may be misdiagnosed in systemic sclerosis patients, due to the frequent concomitant interstitial lung disease.</p><p><strong>Case description: </strong>Here, we present the case of a 63-year-old man affected by systemic sclerosis presenting with an atypical lung imaging and splenomegaly, who was diagnosed with splenic marginal zone lymphoma, thus raising the suspicion of lung secondarism. We discuss the diagnostic challenge of differential diagnosis in interstitial lung presentation and briefly review the available literature on this topic.</p><p><strong>Conclusion: </strong>Several reports have demonstrated an increased risk of malignancy in patients with systemic sclerosis. Still, the lack of concretely defined guidelines for systemic sclerosis, along with systemic sclerosis multifaceted organ involvement at presentation, may challenge diagnosis and management. Here, we remark the importance of clinical work-up and a multidisciplinary approach in systemic sclerosis, to early detect and treat concomitant hematological malignancies, especially during the first years of the disease.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"7 3","pages":"NP1-NP6"},"PeriodicalIF":2.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9537711/pdf/10.1177_23971983221088459.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33494583","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Effect of skin phototype on quantitative nailfold capillaroscopy. 皮肤光型对定量甲襞乳头镜检查的影响。
IF 2
Journal of Scleroderma and Related Disorders Pub Date : 2022-10-01 Epub Date: 2022-06-05 DOI: 10.1177/23971983221102688
Devender Bairwa, Chengappa G Kavadichanda, Saikumar Dunga, Anoop Mathew, Aishwarya G, Gayathri M S, Gorijavolu Mamatha, Molly Mary Thabah, Vir Singh Negi
{"title":"Effect of skin phototype on quantitative nailfold capillaroscopy.","authors":"Devender Bairwa,&nbsp;Chengappa G Kavadichanda,&nbsp;Saikumar Dunga,&nbsp;Anoop Mathew,&nbsp;Aishwarya G,&nbsp;Gayathri M S,&nbsp;Gorijavolu Mamatha,&nbsp;Molly Mary Thabah,&nbsp;Vir Singh Negi","doi":"10.1177/23971983221102688","DOIUrl":"10.1177/23971983221102688","url":null,"abstract":"<p><strong>Objectives: </strong>To determine the impact of Fitzpatrick scale-based skin phototype on visualization of capillary density using nailfold capillaroscopy in healthy Indian adults.</p><p><strong>Methods: </strong>In this cross-sectional study, healthy adults were examined for nailfold capillaroscopy findings utilizing a portable capillary microscope at 800× magnification. Photographs of two contiguous areas measuring 1 mm<sup>2</sup> each of the distal row of capillaries were captured. Images were captured from the central area of all fingers except thumb in both hands. Capillary density and morphology of nailfold capillaroscopies were assessed by two blinded assessors. The nailfold capillaroscopy parameters were compared between the Standard Fitzpatrick scale-based skin phototypes.</p><p><strong>Results: </strong>A total of 118 healthy adults were enrolled in the study. Type III, IV, V, and VI skin phototypes were seen in 27 (22.90%), 32 (27.19%), 29 (24.58%), and 30 (25.42%) participants, respectively. All participants (100%) had normal nailfold capillaroscopy morphology and architecture. Zero capillaries were visible in 11 fingers among 5 patients (4.24%) and all of them had Type VI phototype. The median capillary density per mm was 5.19 (interquartile range = 4.37-6.75) with 90 (76.27%) participants having less than seven capillaries. The median average capillary density was significantly different (<i>p</i>-value < 0.0001) across Type III (8.13, interquartile range = 6.44-8.88), Type IV (5.67, interquartile range = 4.41-6.98), Type V (4.94, interquartile range = 4.19-5.38), and Type VI (4.53, interquartile range = 3.72-4.91) phototypes (<i>p</i> < 0.05).</p><p><strong>Conclusion: </strong>The number of capillaries visualized during nailfold capillaroscopy decreases as the skin pigmentation increases. There is a need to redefine the nailfold capillaroscopy density and avascularity by taking skin phototype as one of the determinants before labeling a nailfold capillaroscopy finding with less visualized capillaries as abnormal.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"7 3","pages":"197-203"},"PeriodicalIF":2.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9537707/pdf/10.1177_23971983221102688.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33495409","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
相关产品
×
本文献相关产品
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信