Systemic sclerosis: Advances towards stratified medicine

Publication date: Available online 21 January 2020Source: Best Practice &Research Clinical RheumatologyAuthor(s): A. Quinlivan, L. Ross, S. ProudmanAbstractEpidemiological studies reporting demographic, clinical and serological factors predictive of various outcomes in systemic sclerosis (SSc) range from the prediction of mortality to the development and progression of disease manifestations. However, predicting the disease trajectory in the individual patient is a challenging but important step towards a stratified approach to disease management. Recent technological advances provide the opportunity for new subgroupings of disease based on risk stratification, through the systematic analysis of high-dimensional clinical data combined with genes, their transcription products and their corresponding translated proteins. In addition, these variables offer a rich vein of research to identify non-invasive biomarkers for predicting organ involvement and to assess disease activity and response to therapy. Selection of patients with a clinical phenotype or molecular signature relevant to the therapy under study combined with recent efforts to standardise outcome measures, show promise for improving clinical trial design and the identification of effective targeted therapies.
Source: Best Practice and Research Clinical Rheumatology - Category: Rheumatology Source Type: research

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In this report, we describe two patients with anticentriole antibodies-positive SSc-PAH who were treated with pulmonary vasodilators. Both cases were elderly women with poor physical conditions and clinical findings of SSc. Case 1 was resistant to combination therapy with pulmonary vasodilators; in Case 2, hemodynamic improvement was obtained by upfront combination therapy at an early stage. Because anticentriole antibodies-positive SSc-PAH rapidly deteriorates, careful hemodynamic observation and timely aggressive use of pulmonary vasodilators should be considered. PMID: 32224603 [PubMed - in process]
Source: International Heart Journal - Category: Cardiology Tags: Int Heart J Source Type: research
Researchers here consider a very conservative set of interventions known to modestly slow the progression of aging in laboratory species, largely by altering metabolism to upregulate beneficial cellular stress responses. The researchers look through the lens of lung aging, specifically, reviewing the evidence for these therapies to slow the deterioration in lung function and onset of lung disease in older individuals, or to be the basis for treating established lung disease. To date, the most reliable, best-researched way to extend life span is through the practice of calorie restriction (CR), which involves reduc...
Source: Fight Aging! - Category: Research Authors: Tags: Daily News Source Type: blogs
Although rarely life-threatening, morphea may be accompanied by substantial morbidity. Symptoms of active disease and functional impairment from damage are associated with decreased quality of life. Morphea treatment is ideally tailored to disease subtype and severity, with the goal of controlling active disease and preventing further damage. Evidence-based treatment in morphea has traditionally been limited in light of the rarity of the condition and a lack of standardized outcome measures. However, over the last 10 years, the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) has emerged as a useful outcome measure...
Source: JAMA Dermatology - Category: Dermatology Source Type: research
Collagen vascular disease, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and scleroderma (SSc), all have auto-antibodies in their pathogenesis though the effect after heart transplantation is not known. Our aim was to assess the impact of collagen vascular disease on the risk of post-transplant donor specific antibodies (DSA), rejection, and cardiac allograft vasculopathy (CAV).
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (598) Source Type: research
AbstractTo assess the efficacy and safety of pirfenidone in systemic sclerosis-related interstitial lung disease (SSc-ILD). This was a double-blind, randomised, placebo-controlled, pilot study. Subjects with SSc-ILD and forced vital capacity (FVC) between 50 and 80% of the predicted (%pred) value were randomised in 1:1 ratio to receive either pirfenidone (2400  mg/day) or placebo for 6 months. Primary outcome was the proportion of subjects with either stabilisation or improvement in FVC at 6 months. Secondary outcomes were the absolute change in the %pred FVC, Mahler’s dyspnoea index, 6-min walk distan...
Source: Rheumatology International - Category: Rheumatology Source Type: research
ConclusionIntense dust cloud exposure among responders and PTSD among community members were associated with a statistically significant increased risk of new ‐onset systemic autoimmune disease. Clinicians treating 9/11 survivors should be aware of the potential increased risk of systemic autoimmune disease in this population.
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: Original Article Source Type: research
ConclusionDividing MSC ‐like cells are present in the SSc disease microenvironment where multiple factors, likely acting in concert, promote transdifferentiation, leading to a complex and resistant disease state.
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: FULL LENGTH Source Type: research
CONCLUSIONS: Pulmonary LCDD is characterized by cysts and nodules. The disease is associated with MALT lymphoma, especially in the setting of Sjögren syndrome. PMID: 32217287 [PubMed - as supplied by publisher]
Source: Respiratory Care - Category: Respiratory Medicine Authors: Tags: Respir Med Source Type: research
Systemic sclerosis (SSc) is a connective tissue disease with a significant morbidity and reduced survival of patients. Effective treatment and clinical control of the disease remain challenging. In particular,...
Source: Arthritis Research and Therapy - Category: Rheumatology Authors: Tags: Research article Source Type: research
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Source: Current Opinion in Rheumatology - Category: Rheumatology Tags: CLINICAL THERAPEUTICS AND HEMATOLOGIC COMPLICATIONS: Edited by W. Joseph McCune Source Type: research
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