Journal of Scleroderma and Related Disorders最新文献

{"title":"Review of systemic sclerosis and antineutrophil cytoplasmic antibody vasculitis overlap: Using autoantibodies for a personalised medicine approach.","authors":"Kristina En Clark","doi":"10.1177/23971983221126850","DOIUrl":"10.1177/23971983221126850","url":null,"abstract":"<p><p>Both antineutrophil cytoplasmic antibody-associated vasculitis and systemic sclerosis are rare autoimmune diseases. Both have the potential for significant multi-organ involvement, and both carry high morbidity and mortality. Disease-specific autoantibodies in these conditions allow for risk stratification for organ-based complications, and for personalised therapeutic strategies. The concomitant presentation of antineutrophil cytoplasmic antibody-associated vasculitis and systemic sclerosis is rare, and only reported in up to 1.3% of systemic sclerosis cases. These patients present more frequently with anti-myeloperoxidase and anti-topoisomerase antibody profiles, with increased incidence of interstitial lung disease and renal involvement than would be expected in either disease independently. Appreciating the role of the autoantibodies in each disease state, and where they overlap, allows for the potential of a more personalised approach to managing these complex patients.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896200/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9769849","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}

{"title":"Evaluation of hypertension in systemic sclerosis and systemic lupus erythematosus overlap.","authors":"Erin Chew, April Barnado, Talat Alp Ikizler, Roy Zent, Tracy Frech","doi":"10.1177/23971983221122673","DOIUrl":"10.1177/23971983221122673","url":null,"abstract":"<p><p>Patients with systemic sclerosis and systemic lupus erythematosus serologies present a unique challenge to the clinician when hypertension is detected in the outpatient setting. Treatment choices for non-renal crisis hypertension are different for systemic sclerosis versus systemic lupus erythematosus. Urgent laboratory studies and, in the presence of certain symptoms, imaging assessment are indicated in systemic sclerosis and systemic lupus erythematosus overlap patients with systemic hypertension. Long-term assessment of systemic hypertension may be enhanced by advances in non-contrast imaging that serve as valuable biomarkers for progressive vasculopathy. In this review, the diagnostic approach to systemic sclerosis and systemic lupus erythematosus overlap patients presenting with hypertension is discussed.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896192/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9648572","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}

{"title":"Effect of pregnancy on scleroderma progression.","authors":"Siobhan Deshauer,&nbsp;Mats Junek,&nbsp;Murray Baron,&nbsp;Karen A Beattie,&nbsp;Margaret J Larché","doi":"10.1177/23971983221101311","DOIUrl":"https://doi.org/10.1177/23971983221101311","url":null,"abstract":"<p><strong>Objective: </strong>To explore the trajectory of scleroderma disease activity in women who experienced a pregnancy after systemic sclerosis diagnosis compared to nulliparous women.</p><p><strong>Methods: </strong>We analyzed data from the Canadian Scleroderma Research Group registry by identifying nulliparous women and women with ⩾1 pregnancy after systemic sclerosis diagnosis. Patient characteristics were compared between groups at registry entry. Controlling for age, smoking, and time since systemic sclerosis diagnosis, generalized estimating equations tested the effect of pregnancy on force vital capacity, diffusing capacity of the lungs for carbon monoxide, right ventricular systolic pressure, glomerular filtration rate, antibody status, active digital ulcers, physician global assessment of activity, and severity over 9 years.</p><p><strong>Results: </strong>At registry entry, numbers of women in the nulliparous and pregnancy after systemic sclerosis diagnosis groups were 153 and 45, respectively. Corresponding numbers at 6 and 9 years were 48 and 21, and 18 and 9, respectively. The prevalence of anti-topoisomerase positivity was 18.3% in nulliparous and 12.5% in pregnancy after systemic sclerosis diagnosis. Baseline differences included mean (Standard deviation) age of diagnosis (nulliparous: 38.8 (14.0), pregnancy after systemic sclerosis diagnosis: 22.6 (6.8) years, <i>p</i> < 0.001), disease duration (nulliparous: 9.6 (8.9), pregnancy after systemic sclerosis diagnosis: 21.9 (9.6) years; <i>p</i> < 0.001), and inflammatory arthritis (nulliparous: 41 (28%), pregnancy after systemic sclerosis diagnosis: 22 (49%), <i>p</i> = 0.009). There were no significant differences between groups in the change of any outcomes over time.</p><p><strong>Conclusion: </strong>Results demonstrated that having ⩾1 pregnancy after systemic sclerosis diagnosis did not appear to significantly impact long-term renal, respiratory, or global function outcomes. While this offers a hopeful message to systemic sclerosis patients planning a pregnancy, physicians and patients should remain vigilant for potential post-partum complications.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896202/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9648574","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}

{"title":"Mouth opening in systemic sclerosis: Its course over time, determinants and impact on mouth handicap.","authors":"Sarah J H Khidir, Maaike Boonstra, Sytske Anne Bergstra, Gerry W M Boerrigter, Elles M Voogt-van der Harst, Maarten K Ninaber, Nina Ajmone Marsan, Tom W J Huizinga, Annette H M van der Helm-van Mil, Jeska K de Vries-Bouwstra","doi":"10.1177/23971983221138177","DOIUrl":"10.1177/23971983221138177","url":null,"abstract":"<p><strong>Objective: </strong>Decreased maximal mouth opening is a common and disabling manifestation in systemic sclerosis patients. We aimed to study the course of maximal mouth opening, determinants of smaller maximal mouth opening over time and the burden of smaller maximal mouth opening on mouth handicap.</p><p><strong>Methods: </strong>Consecutive systemic sclerosis patients participating in the prospective Leiden Combined Care in systemic sclerosis cohort were included. Annual clinical assessment included maximal mouth opening measurement and mouth handicap evaluation (Mouth Handicap in Systemic Sclerosis scale). Presence of microstomia (maximal mouth opening < 30 mm) was studied. Maximal mouth opening over time was assessed on group level and for all patients individually. Baseline characteristics were analysed for their association with smaller maximal mouth opening over time (linear mixed-effects models). Furthermore, cross-sectional association between maximal mouth opening with Mouth Handicap in Systemic Sclerosis scale was assessed (linear regression analysis).</p><p><strong>Results: </strong>A total of 382 systemic sclerosis patients were studied with median follow-up time of 2.0 years (interquartile range = 0.0-3.0). At baseline, mean maximal mouth opening was 42.2 ± 8.0 mm and 7% suffered from microstomia. Annual decrease of > 5.0 mm in maximal mouth opening during follow-up occurred in 63 patients and was accompanied by increase in disease severity. Disease characteristics at baseline independently predictive for smaller maximal mouth opening over time were: more extended skin subtype; peripheral vasculopathy; pulmonary, renal and gastrointestinal involvement. Smaller maximal mouth opening was significantly associated with more reported mouth handicap.</p><p><strong>Conclusion: </strong>The course of maximal mouth opening is stable in a majority of systemic sclerosis patients. Still, maximal mouth opening over time was smaller in patients with more severe organ involvement. Although microstomia was infrequent, a smaller maximal mouth opening was significantly associated with more mouth handicap, indicating the importance to address maximal mouth opening in routine care of systemic sclerosis patients.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896194/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9715820","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}

{"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":null,"pages":null},"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}

{"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_s</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_s</i>  = 0.50, visual analogue scales <i>r_s</i>  = 0.24-0.41), PHQ-8 (<i>r_s</i>  = 0.46), RAND-36 (<i>r_s</i>  = -0.21 to -0.47), mRSS (<i>r_s</i>  = 0.28), disease duration (<i>r_s</i>  = -0.01) and age (<i>r_s</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":null,"pages":null},"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}

{"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":null,"pages":null},"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}

{"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":null,"pages":null},"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}

{"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":null,"pages":null},"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}

{"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":null,"pages":null},"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}