Systemic Sclerosis

Ferri, Clodoveo; Valentini, Gabriele; Cozzi, Franco; Sebastiani, Marco; Michelassi, Claudio; Montagna, G.; Bullo, Arianna; Cazzato, Massimiliano; Tirri, Enrico; Storino, Franca; Giuggioli, Dilia; Cuomo, Giovanna; Rosada, Mara; Bombardieri, Stefano; Todesco, S; Tirri, G

doi:10.1097/00005792-200203000-00004

Cited by 659 publications

(147 citation statements)

References 27 publications

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“…In comparison with the literature, the demographic and clinical characteristics of our sample showed a similar distribution of subjects in the subsets of SSc and a similar female:male ratio [3]. However, our SSc group was slightly older and had a longer onset of the pathology compared to the Italian population [3].…”

Section: Limitationssupporting

confidence: 64%

Oropharyngolaryngeal Disorders in Scleroderma: Development and Validation of the SLS Scale

et al. 2009

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Systemic sclerosis (SSc) is a multisystemic disorder characterized by functional and structural abnormalities of small blood vessels and fibrosis of the skin and internal organs. Eighty percent of subjects with SSc have orofacial manifestations. No tests for oral manifestations have been validated for this pathology, and in the literature there are few studies of speech therapy for subjects with SSc. These facts suggested the need for an exhaustive assessment of mouth mobility and muscle strength, and also of swallowing and voice, in order to plan a targeted and effective speech therapy. The Scleroderma Logopedic Scale (SLS) has been developed to assess disorders in five domains: Impairment, Swallow, Voice, Multifield, and Quality of Life. Perception of these disorders was assessed in 84 subjects with SSc and in 40 healthy subjects. After the first draft, a shorter form (39 items) was obtained after statistical analysis. This scale showed good discriminant and concurrent validity. Internal consistency was good: three of five subscales had a Cronbach alpha coefficient higher than 0.8. The test/retest coefficient for the total score was 0.94. Thirty-six percent of examined subjects showed moderate to severe oropharyngolaryngeal disorders. Swallowing disorders and impairment of mouth (e.g., decrease in mobility and strength) were the most commonly reported problems. Conversely, the change of voice due to the pathology was not perceived as a problem. Fifty-five percent of subjects reported a decreased level of quality of life.

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Section: Limitationssupporting

confidence: 64%

Oropharyngolaryngeal Disorders in Scleroderma: Development and Validation of the SLS Scale

et al. 2009

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“…10 Lung involvement can occur in all subsets of the disease including limited cutaneous SSc, diffuse cutaneous SSc, and SSc sine scleroderma, and it can affect all aspects of the respiratory tract including the parenchyma, vasculature, airways, pleura, and musculature. 11 Therefore, when a patient with SSc presents with symptoms of dyspnea, the differential diagnosis can be quite broad (Box 1).…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Management of Systemic-Sclerosis-Associated Interstitial Lung Disease

Silver

2015

Rheumatic Disease Clinics of North America

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Although scleroderma-associated interstitial lung disease (SSc-ILD) is a significant contributor to both morbidity and mortality, its pathogenesis is largely unclear. Pulmonary function tests (PFTs) and high resolution CT (HRCT) scanning continue to be the most effective tools to screen for lung involvement and to monitor for disease progression. More research and better biomarkers are needed to identify patients most at risk for developing SSc-ILD as well as to recognize which of these patients will progress to more severe disease. While immunosuppression remains the mainstay of treatment, anti-fibrotic agents may offer new avenues of treatment for patients with SSc-ILD in the future.

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“…Involvement of critical internal organs leads to death in some patients (3). The skin is affected in Ͼ90% of patients, and cutaneous involvement closely correlates with internal organ pathology and can be used as a surrogate index for prognosis (4,5).…”

mentioning

confidence: 99%

Systemic and cell type-specific gene expression patterns in scleroderma skin

Whitfield

Finlay

Murray

et al. 2003

Proc. Natl. Acad. Sci. U.S.A.

382

311

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We used DNA microarrays representing >12,000 human genes to characterize gene expression patterns in skin biopsies from individuals with a diagnosis of systemic sclerosis with diffuse scleroderma. We found consistent differences in the patterns of gene expression between skin biopsies from individuals with scleroderma and those from normal, unaffected individuals. The biopsies from affected individuals showed nearly indistinguishable patterns of gene expression in clinically affected and clinically unaffected tissue, even though these were clearly distinguishable from the patterns found in similar tissue from unaffected individuals. Genes characteristically expressed in endothelial cells, B lymphocytes, and fibroblasts showed differential expression between scleroderma and normal biopsies. Analysis of lymphocyte populations in scleroderma skin biopsies by immunohistochemistry suggest the B lymphocyte signature observed on our arrays is from CD20 ؉ B cells. These results provide evidence that scleroderma has systemic manifestations that affect multiple cell types and suggests genes that could be used as potential markers for the disease.

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Systemic Sclerosis

Cited by 659 publications

References 27 publications

Oropharyngolaryngeal Disorders in Scleroderma: Development and Validation of the SLS Scale

Oropharyngolaryngeal Disorders in Scleroderma: Development and Validation of the SLS Scale

Management of Systemic-Sclerosis-Associated Interstitial Lung Disease

Systemic and cell type-specific gene expression patterns in scleroderma skin

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