Progress in Understanding, Diagnosing, and Managing Cardiac Complications of Systemic Sclerosis

AbstractPurpose of the ReviewSystemic sclerosis (scleroderma) is a complex autoimmune disease that commonly involves the cardiovascular system. Even if often subclinical, cardiac involvement is considered a poor prognostic factor as it is a leading cause of death in scleroderma patients. We review the cardiac manifestations of scleroderma, the diagnostic methods useful in detection, and current advances in therapeutic management.Recent FindingsBeside the routine exams for the assessment of cardiac status (including EKG, standard echocardiography, provocative tests) novel techniques such as myocardial strain imaging on echocardiography, cardiac magnetic resonance imaging, invasive hemodynamic assessment, and endomyocardial biopsy have been demonstrated to be useful in understanding the cardiac alterations that typically affect scleroderma patients.SummaryRecent application of novel cardiac detection strategies is providing increased insight into the breadth and pathogenesis of cardiac complications of scleroderma. Further studies coupling exercise provocation, invasive and imaging assessment, and mechanistic studies in scleroderma cardiac tissue are needed to develop the optimal approach to early detection of cardiac disease in scleroderma and targeted therapies.
Source: Current Rheumatology Reports - Category: Rheumatology Source Type: research

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CONCLUSIONS: In our cohort, the prevalence of serodiscordant SSc patients was low. They differed from their counterparts in some clinical manifestations. The management of patients with SSc should be guided by both serology and cutaneous subtype. PMID: 31969223 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
Resetting the immune system through autologous hematopoietic stem cell transplant (autoHSCT) is a highly effective treatment in patients with autoimmune diseases (AID). AutoHSCT achieved long-term remission in patients with relapsed refractory and secondary progressive multiple sclerosis (Muraro 2017), superior to their previous standard of care (Burt 2019). AutoHSCT in scleroderma patients achieved superior outcomes in two randomized studies (Tyndall 2014, Sullivan 2018). These impressive results are achieved by both eradication of autoreactive immune effector cells and re-establishment of a self-tolerant immune system, i...
Source: Biology of Blood and Marrow Transplantation - Category: Hematology Authors: Tags: 469 Source Type: research
Understanding the functions of disease-associated noncoding variants is essential for understanding the molecular mechanisms driving diseases with a genetic cause and for identifying therapeutic targets. Combined computational and experimental analyses have demonstrated that IRF5 is hyperactivated by a pathogenic allele of TNPO3 through long-distance chromatin looping. This finding identifies a molecular mechanism contributing to the polygenic autoimmune diseases of systemic lupus erythematosus and systemic sclerosis.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Commentary Source Type: research
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
a Rocha Pitta MG Abstract BACKGROUND: Rheumatoid arthritis (RA) is a systemic autoimmune disease characterized by chronic and progressive inflammation that can cause a high degree of disability in affected individuals. Proinflammatory cytokines play central roles in the development of degradative and inflammatory responses in RA. IL-29 has been identified in RA and reported as a biomarker of the disease. OBJECTIVE: To analyze serum levels and accuracy of IL-29 in RA patients compared to healthy subjects and patients with other rheumatic diseases. METHODS: IL-29 serum levels were measured in 121 patients ...
Source: Immunology Letters - Category: Allergy & Immunology Authors: Tags: Immunol Lett Source Type: research
This study confirms the efficacy of autologous stem cell transplantation in real-life practice for severe systemic sclerosis using non myeloablative conditioning. Careful cardio-pulmonary assessment to identify organ involvement at patient referral, reduced cyclophosphamide doses and CD34+ selection may improve outcomes. The study was registered at NCT02516124. PMID: 31949011 [PubMed - as supplied by publisher]
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica 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
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
AbstractThe last 2  decades have witnessed the discovery and characterization of a new family of cytokines with immunological characteristics similar to those described for type I interferons, type III or lambda interferons. Unraveling the molecular mechanisms underlying each type of interferon has allowed us to unde rstand how some autoimmune diseases can be considered as interferonopathies. Under normal conditions, type III interferons play a key role in the defense against viruses by modulating the functioning of several types of innate and adaptive immune cells. These effects include upregulation of major hi stoco...
Source: Archivum Immunologiae et Therapiae Experimentalis - Category: Allergy & Immunology Source Type: research
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