Journal of Scleroderma and Related Disorders最新文献

{"title":"Reasons for hospitalization and in-hospital mortality in adults with systemic sclerosis: Analysis of the National Inpatient Sample.","authors":"Joanna Potera,&nbsp;Augustine M Manadan","doi":"10.1177/23971983221083225","DOIUrl":"10.1177/23971983221083225","url":null,"abstract":"<p><strong>Objective: </strong>Systemic sclerosis is an autoimmune condition with significant morbidity and mortality despite modern medical therapies. The goal of this investigation was to comprehensively analyze all reasons for hospitalization and in-hospital death of systemic sclerosis patients.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of the adult systemic sclerosis hospitalizations from the 2016-2018 National Inpatient Sample. We included patients with a primary or secondary diagnosis of systemic sclerosis and compared them to the group without the disease. The incidence of inpatient death and total hospitalization charges were recorded along with the most frequent principal diagnoses for systemic sclerosis hospitalizations and mortality categorized into subgroups.</p><p><strong>Results: </strong>There were 94,515 adult systemic sclerosis hospitalizations recorded in the 2016-2018 National Inpatient Sample database. Systemic sclerosis patients had higher inpatient mortality compared to the non-systemic sclerosis group (4.5% vs 2.2%, respectively, p < 0.0001), were more likely to be female (84% vs 58%, p < 0.0001), had a longer mean length of stay (6.1 vs 4.7 days, p < 0.0001), and greater mean total hospital charges ($70,018 vs $53,556, p < 0.0001). Sepsis, unspecified organism (A41.9) was the most common principal diagnosis for both hospitalized and deceased systemic sclerosis patients. Cardiovascular diagnoses (21.9%) were the most common reasons for hospitalization and infectious (28%)-for in-hospital death.</p><p><strong>Conclusion: </strong>Our analysis of the National Inpatient Sample database from 2016 to 2018 showed that infections and cardiovascular diseases were a significant cause of morbidity and mortality among hospitalized systemic sclerosis patients. Sepsis was the most frequent specific diagnosis for both hospitalization and inpatient deaths. These results stress the importance of early recognition of life-threatening infections in this patient population.</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/PMC9537709/pdf/10.1177_23971983221083225.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33515661","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 and clinical associations with primary hypogonadism in male systemic sclerosis.","authors":"Sapol Thepwiwatjit,&nbsp;Suranut Charoensri,&nbsp;Wichien Sirithanaphol,&nbsp;Ajanee Mahakkanukrauh,&nbsp;Siraphop Suwannaroj,&nbsp;Chingching Foocharoen","doi":"10.1177/23971983221112021","DOIUrl":"10.1177/23971983221112021","url":null,"abstract":"<p><strong>Background: </strong>Systemic sclerosis may affect male and female fertility. Premature ovarian failure has been reported in female systemic sclerosis patients, but the effects on male fertility in systemic sclerosis have not been studied.</p><p><strong>Objectives: </strong>We aimed to determine the prevalence and clinical associations with primary hypogonadism among male systemic sclerosis patients.</p><p><strong>Methods: </strong>This was a cross-sectional pilot study, including 30 adult male systemic sclerosis patients attending the Scleroderma Clinic, Khon Kaen University. Testosterone deficiency symptoms were assessed using the Aging Males' Symptoms Rating Scale, urological examination, and blood testing (for total testosterone, free testosterone, follicle-stimulating hormone, and luteinizing hormone). We excluded patients with congenital hypogonadism and any acquired disorders of the testes and genitalia. The definition of primary hypogonadism was based on the International Society for the Study of the Aging Male 2015 diagnostic criteria for hypogonadism.</p><p><strong>Results: </strong>Seven patients met the definition of primary hypogonadism-a prevalence of 23.3% (95% confidence interval: 9.9-42.3). The respective mean age and mean systemic sclerosis duration was 59.4 ± 11.9 and 5.5 ± 4.7 years. Older age at onset, high triglyceride level, and older age starting corticosteroid treatment were significantly associated with primary hypogonadism (<i>p</i> = 0.02, 0.02, and 0.03, respectively). Systemic sclerosis subset, disease severity, and immunosuppressant use were not associated with primary hypogonadism among Thai male systemic sclerosis patients.</p><p><strong>Conclusion: </strong>Around one-quarter of male systemic sclerosis patients had primary hypogonadism. Elderly onset of systemic sclerosis, hypertriglyceridemia, and late corticosteroid treatment were risk factors for developing primary hypogonadism.</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/PMC9537708/pdf/10.1177_23971983221112021.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33495410","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":"Diagnosis and monitoring of systemic sclerosis-associated interstitial lung disease using high-resolution computed tomography.","authors":"Dinesh Khanna, Oliver Distler, Vincent Cottin, Kevin K Brown, Lorinda Chung, Jonathan G Goldin, Eric L Matteson, Ella A Kazerooni, Simon Lf Walsh, Michael McNitt-Gray, Toby M Maher","doi":"10.1177/23971983211064463","DOIUrl":"10.1177/23971983211064463","url":null,"abstract":"<p><p>Patients with systemic sclerosis are at high risk of developing systemic sclerosis-associated interstitial lung disease. Symptoms and outcomes of systemic sclerosis-associated interstitial lung disease range from subclinical lung involvement to respiratory failure and death. Early and accurate diagnosis of systemic sclerosis-associated interstitial lung disease is therefore important to enable appropriate intervention. The most sensitive and specific way to diagnose systemic sclerosis-associated interstitial lung disease is by high-resolution computed tomography, and experts recommend that high-resolution computed tomography should be performed in all patients with systemic sclerosis at the time of initial diagnosis. In addition to being an important screening and diagnostic tool, high-resolution computed tomography can be used to evaluate disease extent in systemic sclerosis-associated interstitial lung disease and may be helpful in assessing prognosis in some patients. Currently, there is no consensus with regards to frequency and scanning intervals in patients at risk of interstitial lung disease development and/or progression. However, expert guidance does suggest that frequency of screening using high-resolution computed tomography should be guided by risk of developing interstitial lung disease. Most experienced clinicians would not repeat high-resolution computed tomography more than once a year or every other year for the first few years unless symptoms arose. Several computed tomography techniques have been developed in recent years that are suitable for regular monitoring, including low-radiation protocols, which, together with other technologies, such as lung ultrasound and magnetic resonance imaging, may further assist in the evaluation and monitoring of patients with systemic sclerosis-associated interstitial lung disease. A video abstract to accompany this article is available at: https://www.globalmedcomms.com/respiratory/Khanna/HRCTinSScILD.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/33/da/10.1177_23971983211064463.PMC9537704.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33495411","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":"Transition from epoprostenol to selexipag in a patient with systemic sclerosis and pulmonary hypertension during the postoperative period of colon cancer surgery: A case report.","authors":"Takuma Tsuzuki Wada,&nbsp;Kazuhiro Yokota,&nbsp;Shinichiro Iida,&nbsp;Yuki Kanno,&nbsp;Nozomi Shinozuka,&nbsp;Kojiro Sato,&nbsp;Yu Funakubo Asanuma,&nbsp;Keiji Yamamoto,&nbsp;Toshihide Mimura","doi":"10.1177/23971983211063711","DOIUrl":"https://doi.org/10.1177/23971983211063711","url":null,"abstract":"<p><strong>Introduction: </strong>Most pulmonary vasodilators are administered orally; however, in patients with pulmonary hypertension undergoing gastrointestinal surgery, a switch to parenteral drugs is needed. Parenteral pulmonary vasodilators carry a risk of infection and reduced quality of life owing to long-term central venous catheterization; therefore, it is preferable to switch them to oral vasodilators after surgery. Here, we present the case of a patient with systemic sclerosis complicated by pulmonary hypertension and colon cancer, for which treatment was successfully switched from epoprostenol to selexipag postoperatively.</p><p><strong>Case description: </strong>A 59-year-old woman, who was diagnosed with mixed group I and III pulmonary hypertension and systemic sclerosis, was on oral triple pulmonary vasodilators for pulmonary hypertension and Raynaud's phenomenon. She was diagnosed as having colon cancer 3 months before admission. Despite the severe pulmonary condition and treatment with oral triple pulmonary vasodilators, colon cancer resection surgery was performed with the management for pulmonary hypertension through multidisciplinary treatments in collaboration with cardiology specialists. Medications for patients with pulmonary hypertension undergoing gastrointestinal surgery need to be switched from oral vasodilators to epoprostenol perioperatively. On postoperative day 19, 0.4 mg/day of selexipag was administered with epoprostenol. Subsequently, the epoprostenol dosage was gradually decreased, and selexipag was increased. On postoperative day 30, the dose of selexipag was increased to 1.2 mg/day and epoprostenol was discontinued. The patient was discharged on postoperative day 40.</p><p><strong>Conclusion: </strong>In our case, transition from epoprostenol to selexipag contributed to a more useful management strategy for systemic sclerosis and pulmonary hypertension in the postoperative period.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9109508/pdf/10.1177_23971983211063711.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9556145","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":"Reliability, validity, and responsiveness to change of the Patient-Reported Outcomes Measurement Information System self-efficacy for managing chronic conditions measure in systemic sclerosis.","authors":"Susan L Murphy, Veronica J Berrocal, Janet L Poole, Dinesh Khanna","doi":"10.1177/23971983211049846","DOIUrl":"10.1177/23971983211049846","url":null,"abstract":"<p><strong>Objective: </strong>The aim of this study is to examine validity, reliability, and responsiveness to change of Patient-Reported Outcomes Measurement Information System Self-Efficacy for Managing Chronic Conditions in persons with systemic sclerosis.</p><p><strong>Methods: </strong>We conducted a post hoc analysis of the Patient-Reported Outcomes Measurement Information System Self-Efficacy measure and other quality-of-life measures from systemic sclerosis participants from a 16-week randomized control trial. The trial compared an Internet-based self-management program to a control condition where participants were provided an educational book. All participants completed outcome measures at baseline and following the 16-week trial period.</p><p><strong>Results: </strong>The mean age of participants was 53.7 years, 91% were female and systemic sclerosis subtype included 44.9% limited/sine and 43.1% diffuse; mean disease duration was 9.0 years. All self-efficacy subscales (Managing Emotions, Symptoms, Daily Activities, Social Interactions, and Medications/Treatment) demonstrated good internal consistency (.92-.96). All subscales showed statistically significant correlations with other validated measures of depressive symptoms and quality of life (.20-.86) but were not associated with satisfaction nor with appearance. The subscales appropriately discriminated between those with and without depressive symptoms and demonstrated responsiveness to change over the 16-week period for those who had a corresponding increase in reported quality of life.</p><p><strong>Conclusion: </strong>The Patient-Reported Outcomes Measurement Information System Self-Efficacy measure is valid, reliable, and responsive to change for persons with systemic sclerosis.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9109504/pdf/10.1177_23971983211049846.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10617530","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":"Systemic sclerosis manifestations and clinical outcomes in Hispanics/Latinos of the American Southwest.","authors":"Sharon E Nunez,&nbsp;Angie Ariza-Hutchinson,&nbsp;Roderick A Fields,&nbsp;Jaime A Vondenberg,&nbsp;Rosemina A Patel,&nbsp;N Suzanne Emil,&nbsp;Maheswari Muruganandam,&nbsp;James I Gibb,&nbsp;Janet L Poole,&nbsp;Wilmer L Sibbitt","doi":"10.1177/23971983221086214","DOIUrl":"https://doi.org/10.1177/23971983221086214","url":null,"abstract":"<p><strong>Objective: </strong>Certain Hispanic/Latino (Hispanic) populations have been reported to have higher rates and severity of systemic sclerosis; however, little is known of systemic sclerosis in the American Southwest. This study compared manifestations of systemic sclerosis in Hispanics with non-Hispanics of New Mexico.</p><p><strong>Methods: </strong>This cross-sectional longitudinal study included 109 systemic sclerosis patients followed over a mean of 12.6 ± 8.9 years. Subjects were repetitively evaluated including physical examination, echocardiography, chest imaging, and serologic testing and observed for complications. Disease characteristics and long-term outcomes were statistically compared between self-identified Hispanic and non-Hispanic subjects.</p><p><strong>Results: </strong>A total of 73 (67%) systemic sclerosis subjects were Hispanic and 36 (33%) were non-Hispanic. The cohorts were similar in mean age, age of systemic sclerosis onset, limited versus diffuse cutaneous systemic sclerosis, telangiectases, gastroesophageal reflux disease, Raynaud's phenomenon, autoantibody profile, interstitial lung disease, pulmonary hypertension, scleroderma renal crisis, mortality, and comorbid malignancy (all <i>p</i> > 0.05). However, the standardized mortality ratio was increased in both cohorts relative to age-adjusted mortality: Hispanic: 2.08, confidence interval (1.94-2.24); non-Hispanic: 1.56, confidence interval (1.46-1.68). Furthermore, the standardized incidence ratio for malignancy was increased in both cohorts: Hispanic: 1.45, confidence interval (1.35-1.56); non-Hispanic: 1.24, confidence interval (1.16-1.34). The mean age of cancer diagnosis occurred at a significantly younger age in Hispanics (Hispanics: 53.1 ± 9.7 years; non-Hispanics 63.7 ± 7.9 years; 95% confidence interval: -19 ⩽ 10.6 ⩽ 2.2; <i>p</i> = 0.016).</p><p><strong>Conclusion: </strong>Systemic sclerosis phenotype, autoantibodies, complications, outcomes, malignancy rates, and mortality are generally similar between Hispanics and non-Hispanics with systemic sclerosis in the American Southwest. However, age-adjusted comorbid malignancy and mortality rates are significantly increased in both groups.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9109509/pdf/10.1177_23971983221086214.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9556141","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":"Nailfold capillaroscopy for the early diagnosis of the scleroderma spectrum of diseases in patients without Raynaud's phenomenon.","authors":"Cassandra Hong,&nbsp;Ling Xiang,&nbsp;Seyed Ehsan Saffari,&nbsp;Andrea Hl Low","doi":"10.1177/23971983221088460","DOIUrl":"https://doi.org/10.1177/23971983221088460","url":null,"abstract":"<p><strong>Background: </strong>The utility of nailfold capillaroscopy in the evaluation of patients without Raynaud's phenomenon is unclear.</p><p><strong>Objective: </strong>This study aims to compare the utility of nailfold capillaroscopy for the early diagnosis of the scleroderma-spectrum of diseases in patients who present with Raynaud's phenomenon, undifferentiated non-Raynaud's phenomenon features and positive systemic sclerosis-associated antibodies without scleroderma-spectrum of disease features.</p><p><strong>Methods: </strong>Eligible patients were divided into three referral criteria groups: (I) Raynaud's phenomenon; (II) Undifferentiated non-Raynaud's phenomenon features and (III) Positive systemic sclerosis-associated autoantibodies without features to suggest scleroderma-spectrum of diseases. This includes systemic sclerosis, mixed connective tissue disease and dermatomyositis. The association between baseline scleroderma pattern on nailfold capillaroscopy (systemic sclerosis-nailfold capillaroscopy) and final diagnosis at follow-up was determined using logistic regression analysis. Test characteristics of nailfold capillaroscopy were compared and stratified by referral groups.</p><p><strong>Results: </strong>Of 95 patients followed-up for a mean of 1.6 years, 28 (29.5%) patients developed scleroderma-spectrum of diseases, 36 (37.9%) patients had suspected/other connective tissue disease and 27 (28.4%) patients had no connective tissue disease. Baseline systemic sclerosis-nailfold capillaroscopy was significantly associated with the development of scleroderma-spectrum of diseases in patients from Group I (odds ratio, 7.1, <i>p</i> = 0.01) and Group II (odds ratio 7.3, <i>p</i> = 0.005). In Group II patients, nailfold capillaroscopy had a sensitivity, specificity, positive and negative predictive values of 71.4%, 76.5%, 55.6% and 86.7%, respectively. Specificity (81.8%) and PPV (69.2%) were the highest in Group I patients. Nailfold capillaroscopy had the highest negative predictive value in Group III (100%), followed by Group II (86.7%) and Group I (78.3%) patients.</p><p><strong>Conclusion: </strong>In addition to evaluating patients with Raynaud's phenomenon, nailfold capillaroscopy was useful in the evaluation and exclusion of scleroderma-spectrum of diseases in patients with undifferentiated non-Raynaud phenomenon features and those with systemic sclerosis-associated antibodies without features to suggest scleroderma-spectrum of diseases.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9109506/pdf/10.1177_23971983221088460.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9927959","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":"Management of scleroderma gastrointestinal disease: Lights and shadows.","authors":"Jenice X Cheah,&nbsp;Dinesh Khanna,&nbsp;Zsuzsanna H McMahan","doi":"10.1177/23971983221086343","DOIUrl":"https://doi.org/10.1177/23971983221086343","url":null,"abstract":"<p><p>Gastrointestinal symptoms affect the great majority of patients with systemic sclerosis. Management of these complications is often challenging as any region of the gastrointestinal tract may be involved, and significant heterogeneity exists in clinical presentation, kinetics, and outcomes. Here, we highlight new findings relevant to the management of systemic sclerosis-related gastrointestinal disease (lights) and consider areas that we have yet to elucidate (shadows).</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9109510/pdf/10.1177_23971983221086343.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9556146","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":"Systemic sclerosis in Asians: Are there racial differences?","authors":"Sue-Ann Ng,&nbsp;Andrea Hsiu Ling Low","doi":"10.1177/23971983221074749","DOIUrl":"https://doi.org/10.1177/23971983221074749","url":null,"abstract":"<p><p>Systemic sclerosis is a multisystemic autoimmune disease characterized by vasculopathy and fibrosis. Racial factors exert a significant influence on the epidemiology, clinical manifestations, antibody profile, mortality and genetic factors in systemic sclerosis. In this review, we examined Asian systemic sclerosis cohorts reported in Asia and multi-racial cohort studies to evaluate the disease characteristics and outcomes of systemic sclerosis in Asians. Asian patients have distinct genetic susceptibility to systemic sclerosis, younger age of systemic sclerosis onset, higher frequency of diffuse skin involvement, different autoantibody profiles such as higher frequency of anti-Scl70 and anti-U1-RNP antibodies, and more severe clinical phenotype. There was a suggestion of poorer survival among Asians that may be contributed by more severe disease, socioeconomic factors and differences in healthcare systems. Recognizing the influence of racial differences in systemic sclerosis disease course is important as it has implications for appropriate treatment, monitoring and prognostication.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9109507/pdf/10.1177_23971983221074749.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9556143","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":"A patient with scleroderma associated with severe acro-osteolysis: A case report.","authors":"Mahsa Zargaran,&nbsp;Atiyeh Rostamian,&nbsp;Mahrooz Malek,&nbsp;Abdolrahman Rostamian","doi":"10.1177/23971983211070117","DOIUrl":"https://doi.org/10.1177/23971983211070117","url":null,"abstract":"<p><p>Systemic sclerosis is an autoimmune chronic sclerotic disease that can damage organs and cause serious complications for the patient such as musculoskeletal manifestations, Gastrointestinal involvement, pulmonary involvement, and renal disease. Acro-osteolysis is one of the musculoskeletal manifestations that causes corrosion of the bones in the fingertips of the hand and feet. In this paper, we have reported the rarely current evidence of severe Acro-osteolysis of the distal phalanges of the hands by radiological x-ray.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9109502/pdf/10.1177_23971983211070117.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9556144","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}