Outcomes of limited cutaneous systemic sclerosis patients: Results on more than 12,000 patients from the EUSTAR database

ConclusionsLcSSc patients have a low mRSS at baseline with marginal changes with time. Conversely, SSc-ILD can be as progressive as in DcSSc supporting the inclusion of LcSSc patients in SSc-ILD trials and suggesting potential benefit of any anti-ILD drugs. Similarly, although slightly less common, DU should receive the same attention in the two subsets.
Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research

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To the Editor, Morphea is an autoimmune disease that causes fibrosis of the skin that may result in significant morbidity. Eosinophilic fasciitis (EF) may be considered a deeper form of morphea, and features of both morphea and EF can be present in individual patients. Morphea and EF have been postulated to have pathogenic features that overlap with systemic sclerosis (Laxer and Zulian, 2006). In these diseases, dysregulated immune responses are thought to induce fibrosis (Torok et al., 2019). Treatment of these diseases with non-specific immunosuppressants such as corticosteroids is often ineffective and associated with toxicity.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Letters to the Editor Source Type: research
Systemic sclerosis (SSc) is a rare and severe connective tissue disease combining auto-immune and vasculopathy features, ultimately leading to organ fibrosis. Impaired angiogenesis is an often silent and life-threatening complication of the disease. We hypothesize that CCN3 (NOV), a member of the CCN family of extra-cellular matrix proteins, which is an antagonist of the pro-fibrotic protein CCN2 (CTGF) as well as a pro-angiogenic factor, is implicated in SSc pathophysiology.We performed skin biopsies on 26 SSc patients, both in fibrotic and non-fibrotic areas for 17 patients, and collected 18 healthy control skin specimen...
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Original Article Source Type: research
Authors: Eyigor S, Sezgin B, Kuntman BD, Karabulut G, Zihni Yargucu F, Ozturk K, Kirazli T PMID: 31928591 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
AbstractInterstitial lung disease (ILD) remains a major cause of morbidity and mortality in systemic sclerosis (SSc). Study aim is to characterize and quantify SSc-ILD by using Computer-Aided Lung Informatics for Pathology Evaluation and Rating (CALIPER). Secondly, our objective is to evaluate which radiological pattern is predictive of lung function decline at 12  months follow-up. In the prospective study (IRB 5435), 66 SSc patients underwent high-resolution computerized tomography (HRCT) at baseline. HRCT was performed according to standard protocol using a CT 64GE light speed VCT power scanner. CALIPER classified ...
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
This study highlights the possible role of NFC as biomarker of AICTD, particularly in SSc and IIM.
Source: Rheumatology International - Category: Rheumatology Source Type: research
Systemic sclerosis (SSc) is a multisystem autoimmune and vascular disease resulting in multiple organ fibrosis [1,2], in which skin fibrosis is caused by the combination of dermal fibroblast activation [3 –6], Th2/Th17-skewed immune polarization [7,8], M2 macrophage differentiation [9], aberrant B cell activation [10,11], increased infiltration of plasmacytoid dendritic cells [12], vascular functional and structural abnormalities [13–16] and epithelial cell activation [17–20]. In particular, im mune cells seem to play a central role because immunosuppressants and/or corticosteroids are mostly effective for skin fibrosis.
Source: Journal of Dermatological Science - Category: Dermatology Authors: Source Type: research
ConclusionsOur results suggest that a combined exercise protocol was feasible for people with lcSSc, with no adverse events,  resulting in high adherence and low attrition rates, high enjoyment levels and intentions for future engagement to this exercise. Thus, the specific protocol is a safe adjunct therapy for people with lcSSc.Trial registration:ClinicalTrials.gov (NCT number): NCT03058887, February 23, 2017,https://clinicaltrials.gov/ct2/show/NCT03058887?term=NCT03058887&rank=1Key Points• High-intensity interval training in combination with resistance training constitutes a feasible exercise protocol for peop...
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
Abstract Chronic inflammation has profound tumor-promoting effects. Inflammatory cells are the key players in immunosurveillance against tumors, and immunosuppression is known to increase the risk of tumors. Autoimmune diseases, which manifest as loss of self-tolerance and chronic immune dysregulation, provide a perfect environment for tumor development. Aside from managing the direct inflammatory consequences of autoimmune pathogenesis, cancer risk profiles should be considered as a part of a patient's treatment. In this review, we describe the various associations of malignancies with autoimmune diseases, specif...
Source: Lupus - Category: Rheumatology Authors: Tags: Lupus Source Type: research
Abstract Objective: Despite being a cardinal clinical sign of systemic sclerosis (SSc), digital pitting has been little studied. Our objective was to test, in a pilot study, the hypothesis that pitting is painful and associated with digital vascular disease severity.Method: Fifty patients with SSc were recruited: 25 with and 25 without digital pitting. Fingertip pain was assessed on a 0-10 scale. Thermography of both hands assessed surface temperature, allowing calculation of the distal-dorsal difference (temperature gradient) for each finger. Nailfold capillaroscopy was performed in each finger using a dermatosco...
Source: Scandinavian Journal of Rheumatology - Category: Rheumatology Authors: Tags: Scand J Rheumatol Source Type: research
CONCLUSIONS AND CLINICAL IMPORTANCE: To the best of the authors' knowledge, this is the first report of multicentric B-cell lymphoma in a dog with concurrent diffuse cutaneous sclerosis, similar to a human paraneoplastic reaction. PMID: 31930629 [PubMed - as supplied by publisher]
Source: Veterinary Dermatology - Category: Veterinary Research Authors: Tags: Vet Dermatol Source Type: research
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