Journal of Scleroderma and Related Disorders最新文献

{"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":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/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}

{"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":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/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}

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

{"title":"Fecal microbiome in systemic sclerosis, in search for the best candidate for microbiota-targeted therapy for small intestinal bacterial overgrowth control.","authors":"Elisa Fiorentini,&nbsp;Edda Russo,&nbsp;Amedeo Amedei,&nbsp;Silvia Bellando Randone","doi":"10.1177/23971983221118871","DOIUrl":"10.1177/23971983221118871","url":null,"abstract":"<p><p>Gastrointestinal involvement is a common complication in systemic sclerosis patients and must be suspected and investigated already in the early stages of the disease. Gastrointestinal symptoms and complications-such as gastroesophageal reflux disease, intestinal pseudo-obstruction, malnutrition, diarrhea, constipation, and small intestinal bacterial overgrowth-severely impair systemic sclerosis patients' quality of life and affect their prognosis. Although some pathogenetic aspects of the gastrointestinal involvement in systemic sclerosis remain unclear, defining the characteristics of the microbiota and its role could help in risk stratification, selection of candidates for microbiota-targeted therapies, prediction of standard treatment efficacy, and prognosis of systemic sclerosis patients. Finally, understanding how to modify the microbiota composition may represent an important therapeutic approach to target gastrointestinal involvement in systemic sclerosis.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9537701/pdf/10.1177_23971983221118871.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33515662","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 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 mm² 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":null,"pages":null},"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}

{"title":"Nephrogenic systemic fibrosis-related pulmonary restriction: An under-appreciated manifestation potentially reversible with imatinib therapy.","authors":"Jennifer Mansour,&nbsp;Cheralyn Coleman,&nbsp;Fabian Mendoza,&nbsp;Matthew Lammi,&nbsp;Lesley Ann Saketkoo","doi":"10.1177/23971983221088213","DOIUrl":"10.1177/23971983221088213","url":null,"abstract":"<p><p>Nephrogenic systemic fibrosis typically occurs in patients with renal failure and is strongly associated with gadolinium exposure through stimulation of macrophage-activated fibrosis. Patients present with prominent fibrosis of the skin and internal organs. Quality of life is significantly diminished due to impairment from restrictive mobility of large and small joint contractures, pain, and ensuing psychological stress. Nephrogenic systemic fibrosis can be severe and life-threatening. Nephrogenic systemic fibrosis patients reliant on hemodialysis with cutaneous symptoms, defined as hyperpigmentation, hardening, and tethering of skin on the extremities, experience rates of mortality as high as 48%. Physician awareness and preventive strategies coincided with a reduction in the incidence of nephrogenic systemic fibrosis. Several treatments, of which physical therapy may be a key adjuvant, have been used to treat nephrogenic systemic fibrosis, with variable and inconsistent results, lacking wide consensus. Improvement of renal function may improve nephrogenic systemic fibrosis, with some patients demonstrating stabilization or improvement after renal transplantation or resolution of acute renal failure. Imatinib, a tyrosine kinase inhibitor, demonstrates antifibrotic effects in the skin and recently was used to successfully treat nephrogenic systemic fibrosis. We report a case of severe nephrogenic systemic fibrosis with extensive skin fibrosis causing extrapulmonary restriction who demonstrated improved lung function following treatment with imatinib.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9537706/pdf/10.1177_23971983221088213.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33495412","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 need to accurately measure energy intake and expenditure in patients with systemic sclerosis.","authors":"Michael Hughes,&nbsp;Elizabeth Harrison,&nbsp;Ariane L Herrick,&nbsp;John T McLaughlin,&nbsp;Simon Lal","doi":"10.1177/23971983221095763","DOIUrl":"10.1177/23971983221095763","url":null,"abstract":"<p><strong>Background: </strong>Malnutrition is common in systemic sclerosis and patients are frequently underweight. However, the balance between assessed dietary energy intake versus expenditure has been neglected to date. This study aimed to assess energy (dietary) intakes and expenditures and to compare discrepancies in systemic sclerosis.</p><p><strong>Methods: </strong>Thirty-six outpatients with systemic sclerosis completed the study. Demographics and clinical data were recorded. Functional questionnaires were completed. Predicted energy requirements were calculated. Over a consecutive 3-day period, patients completed an estimated food diary and wore a specialist energy expenditure monitor (SenseWear^® Armband). Assessments of intake and expenditure were compared for individual patients, and the impact according to patient demographics, clinical manifestations and disease severity evaluated.</p><p><strong>Results: </strong>Energy intake did not correlate with predicted (s = 0.117; p = 0.511) or measured (s = -0.039; p = 0.825) expenditures. Predicted and measured energy expenditures correlated, but actual values differed for individuals (intraclass correlation = 0.62; 95% limits of agreement = -459 to 751 kcal). Respiratory involvement was negatively correlated with number of steps (s = -0.350; p = 0.04) and time spent lying (s = 0.333; p = 0.05). There was a significant correlation between body mass index and predicted versus measured energy discrepancy (s = 0.41; p = 0.02), and this discrepancy was greater with higher body mass indices.</p><p><strong>Conclusion: </strong>There was no correlation between intake and either predicted or measured energy expenditure. Predicted and measured energy expenditures were strongly correlated yet differed for the individual patient. In patients with systemic sclerosis, where energy expenditure must be accurately assessed, it should be directly measured.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9537710/pdf/10.1177_23971983221095763.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33495408","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":"Primary heart involvement in systemic sclerosis, from conventional to innovative targeted therapeutic strategies.","authors":"Arianna Ferlito,&nbsp;Corrado Campochiaro,&nbsp;Alessandro Tomelleri,&nbsp;Lorenzo Dagna,&nbsp;Giacomo De Luca","doi":"10.1177/23971983221083772","DOIUrl":"10.1177/23971983221083772","url":null,"abstract":"<p><p>Primary heart involvement is frequent in systemic sclerosis, even though often sub-clinical, and includes cardiac abnormalities that are predominantly attributable to systemic sclerosis rather than other causes and/or complications. A timely diagnosis is crucial to promptly start the appropriate therapy and to prevent the potential life-threatening early and late complications. There is little evidence on how to best manage systemic sclerosis-primary heart involvement as no specific treatment recommendations for heart disease are available, and a shared treatment approach is still lacking. The objective of this review is to summarize the state of the art of current literature and the overall management strategies and therapeutic approaches for systemic sclerosis-primary heart involvement. Novel insights into pathogenic mechanisms of systemic sclerosis-primary heart involvement are presented to facilitate the comprehension of therapeutic targets and novel treatment strategies.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9537702/pdf/10.1177_23971983221083772.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33515660","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":"Relationship between ectopic calcifications and bone fragility depicted on computed tomography scan in 70 patients with systemic sclerosis.","authors":"Marine Fauny,&nbsp;Elodie Bauer,&nbsp;Edem Allado,&nbsp;Eliane Albuisson,&nbsp;Joëlle Deibener,&nbsp;François Chabot,&nbsp;Damien Mandry,&nbsp;Olivier Huttin,&nbsp;Isabelle Chary-Valckenaere,&nbsp;Damien Loeuille","doi":"10.1177/23971983221104415","DOIUrl":"10.1177/23971983221104415","url":null,"abstract":"<p><strong>Background: </strong>A higher risk of osteoporotic fracture was described in systemic sclerosis patients than in healthy patients.</p><p><strong>Objective: </strong>To evaluate the relation between osteoporotic fracture risk measured by the scanographic bone attenuation coefficient of the first lumbar vertebra (SBAC-L1) on computed tomography (CT) scan and the presence of ectopic calcifications: vascular, valvular and spinal.</p><p><strong>Methods: </strong>This monocentric retrospective study was performed on patients followed between 2000 and 2014 at Nancy University Hospital. Systemic sclerosis patients, according to ACR/EULAR 2013 criteria, followed from 2000 to 2014 and who underwent, during their follow-up, a CT including the first lumbar vertebra were included. The SBAC-L1 was measured with a threshold set at 145 Hounsfield units (HU). Vascular and spinal calcifications were studied on CT. For vascular calcifications, the Agatston score was used. Valvular calcifications were studied on echocardiography.</p><p><strong>Results: </strong>A total of 70 patients were included (mean age: 62.3 (±15.6) years, women 88.5%). The mean SBAC-L1 was 157.26 (±52.1) HU, and 35 patients (50%) presented an SBAC-L1 ⩽ 145 HU. The reproducibility of the calcification evaluation was good, with kappa coefficients varying between 0.63 and 1. In univariate analysis, spinal and vascular calcifications were associated with an SBAC-L1 ⩽ 145 HU, with ORs of 13.6 (1.6-113.3) and 8 (95%CI: 2.5-25.5), respectively. In multivariate analysis, the SBAC-L1 was not associated with the presence of any ectopic calcifications. The SBAC-L1 decreased with age (p = 0.0001).</p><p><strong>Conclusion: </strong>Patients with systemic sclerosis with an SBAC-L1 ⩽ 145 HU were older, but they did not have more ectopic calcification.</p><p><strong>Trial registration: </strong>The ethics committee of Nancy Hospital agreed with this study (referral file number 166). This study was designed in accordance with the general ethical principles outlined in the Declaration of Helsinki.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9537705/pdf/10.1177_23971983221104415.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33495407","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":"Severe digital ischemia as an unrecognized manifestation in patients with antisynthetase autoantibodies: Case series and systematic literature review.","authors":"Akira Yoshida,&nbsp;Takahisa Gono,&nbsp;Yuka Okazaki,&nbsp;Yuichiro Shirai,&nbsp;Mitsuhiro Takeno,&nbsp;Masataka Kuwana","doi":"10.1177/23971983221090857","DOIUrl":"10.1177/23971983221090857","url":null,"abstract":"<p><strong>Objective: </strong>Severe digital ischemia, including digital ulcers and gangrene, is considered rare in patients with antisynthetase antibodies. This study aimed to elucidate the clinical features of antisynthetase-positive patients complicated with digital ulcers and/or gangrene using a systematic literature review and case series in a single-center cohort.</p><p><strong>Methods: </strong>A systematic literature review was conducted to identify reports describing antisynthetase-positive cases with digital ulcers and/or gangrene. Our cohort of consecutive patients with antisynthetase antibodies was stratified by the history of severe digital ischemia. Demographic and clinical features and outcomes in patients with severe digital ischemia identified in the systematic literature review and our cohort were compared with those in patients without severe digital ischemia in our cohort.</p><p><strong>Results: </strong>The systematic literature review revealed 12 antisynthetase-positive patients with severe digital ischemia from one case series and eight case reports. Seven (7%) of 100 patients with antisynthetase antibodies in our cohort had a record of severe digital ischemia. Severe digital ischemia was often found at presentation and was associated with the classification of systemic sclerosis with or without myositis overlap. Clinical features associated with severe digital ischemia in antisynthetase-positive patients included Raynaud's phenomenon (<i>p</i> < 0.001), digital pitting scars (<i>p</i> = 0.001), and nailfold capillary abnormality (<i>p</i> = 0.02). Outcomes of severe digital ischemia were generally favorable with vasodilators.</p><p><strong>Conclusion: </strong>Severe digital ischemia is an overlooked complication in antisynthetase-positive patients. Antisynthetase antibodies should be measured in patients presenting with digital ulcers or gangrene, especially in those with systemic sclerosis phenotype and features associated with antisynthetase antibodies in the absence of systemic sclerosis-specific autoantibodies.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9537703/pdf/10.1177_23971983221090857.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33495413","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}