Management of systemic sclerosis: the first five years

Purpose of review This review provides a risk-stratified and evidence-based management for subsets of systemic sclerosis (SSc) patients in the first five years from disease onset. Recent findings Cardiopulmonary disease remains the primary cause of mortality in SSc patients. Morbidity and mortality in SSc-associated pulmonary arterial hypertension have improved with combination treatment, in either an upfront or sequential treatment pattern. Traditional therapies for interstitial lung disease (SSc-ILD) have targeted those with clinically significant and progressive ILD with immunosuppression. New data suggest a possible paradigm shift, introducing immunosuppressive therapy to patients before they develop clinically significant or progressive ILD. The year 2019 saw the approval of the first FDA-approved therapy for SSc-associated interstitial lung disease, using an antifibrotic agent previously approved for idiopathic pulmonary fibrosis. To date, only autologous hematopoietic stem cell transplant has demonstrated a mortality benefit for SSc-ILD, albeit in a narrow spectrum of SSc-ILD patients. Summary SSc is a highly heterogeneous autoimmune disease typified by varying clinical trajectories. Its management may be stratified within the first five years by subclassifying patients based on factors that have important prognostic significance: skin distribution and autoantibody status.
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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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
Semin Respir Crit Care Med 2019; 40: 227-234 DOI: 10.1055/s-0039-1685537Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease characterized by the production of pathogenic autoantibodies and immune complexes and is responsible for significant morbidity and mortality through a wide range of clinical manifestations which can affect almost any organ. Pulmonary involvement is prevalent and seen in 50 to 70% of SLE patients and may even be the presenting feature in 4 to 5% of patients. By 10 years postdiagnosis, 12% will have accumulated an element of permanent lung damage. Pulmonary complications are broad and ...
Source: Seminars in Respiratory and Critical Care Medicine - Category: Respiratory Medicine Authors: Tags: Review Article Source Type: research
ConclusionsThe lung should be carefully studied during CTDs. PFTs can represent an important prognostic tool for diagnosis and follow up of RA-ILD, but, on their own, lack sufficient specificity or sensitivity to describe lung involvement in SSDs and IIMs. Several composite indexes potentially able to describe the evolution of lung damage and response to treatment in SSDs are under investigation. Considering the potential severity of these conditions, an HRCT jointly with PFTs should be performed in all new diagnoses of SSDs and IIMs. Moreover, follow up PFTs should be interpreted in the light of the risk factor for respir...
Source: Multidisciplinary Respiratory Medicine - Category: Respiratory Medicine Source Type: research
Therapeutic Targeting of Fibrotic Epithelial-Mesenchymal Transition–An Outstanding Challenge Attila Fintha1, Ákos Gasparics2, László Rosivall3 and Attila Sebe3,4* 12nd Department of Pathology, Semmelweis University, Budapest, Hungary 21st Department of Obstetrics and Gynecology, Semmelweis University, Budapest, Hungary 3Department of Pathophysiology, International Nephrology Research and Training Center, Semmelweis University, Budapest, Hungary 4Division of Medical Biotechnology, Paul Ehrlich Institute, Langen, Germany Back in 1995, a landmark paper was published, which shaped the fi...
Source: Frontiers in Pharmacology - Category: Drugs & Pharmacology Source Type: research
Authors: Rizzi M, Radovanovic D, Airoldi A, Cristiano A, Frassanito F, Gaboardi P, Saad M, Atzeni F, Sarzi-Puttini P, Santus P Abstract Systemic sclerosis (SSc) is an autoimmune disease characterised by tissue fibrosis leading to vascular injury. Nitric oxide (NO) has been implicated in the pathogenesis of autoimmune diseases. A deficiency in basal NO production by the constitutive endothelial isoform of nitric oxide synthase may promote vasoconstriction and vascular wall thickening. In January 2017, we searched the PubMed/Medline, Cochrane Library and Enbase/Medline databases for studies analysing physio-pathologi...
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
In the past decade, the attention has been focused on the relationship between interstitial lung disease (ILD) and connective tissue diseases (CTDs). The clinical impact, as well as the therapeutic approach, classification and diagnosis have been addressed. These two latter items represent an area of great interest because the underlying conditions leading to ILD and the most appropriate treatment still needs to be defined. Recently, the European Respiratory Society/American Thoracic Society proposed the interstitial pneumonia with autoimmune features (IPAF) criteria which represents the effort of pulmonologists to classif...
Source: European Respiratory Review - Category: Respiratory Medicine Authors: Tags: Interstitial and orphan lung disease Editorials Source Type: research
Authors: Eldoma M, Pope J Abstract INTRODUCTION: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease (CTD) characterized by vascular dysfunction, fibrosis, inflammation and autoantibodies. The pathophysiology of SSc is not completely understood, and many patients acquire organ or tissue damage despite advances in treatment. Current treatments target affected organs with modest improvements. Areas covered: This review evaluates several treatment strategies for SSc based on involved organs including skin, pulmonary, cardiac, renal, musculoskeletal and gastrointestinal. Currently, pulmonary hyperte...
Source: Expert Review of Clinical Immunology - Category: Allergy & Immunology Tags: Expert Rev Clin Immunol Source Type: research
ConclusionpPTT was found to be shorter in SSc patients. pPTT might serve as a surrogate marker of pulmonary hemodynamics in patients with SSc, even prior to the development of pulmonary hypertension.
Source: Lung - Category: Respiratory Medicine Source Type: research
AbstractPurpose of ReviewThe study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sj ögren’s syndrome.Recent FindingsAlthough collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient populat...
Source: Current Rheumatology Reports - Category: Rheumatology Source Type: research
AbstractBackgroundSystemic sclerosis is a chronic debilitating autoimmune disease characterized by endothelial dysfunction and multi-organ fibrosis. Interstitial lung disease, a common manifestation of SSc, is termed scleroderma-related interstitial lung disease (SSc-ILD) and along with pulmonary hypertension contributes to a majority of deaths in SSc. SSc-ILD patients frequently develop pulmonary hypertension, which prognosticates a poorer outcome. We investigated pulmonary artery dimensions as an outcome predictor in patients with SSc-ILD.MethodsA retrospective chart review abstracting data from SSc-ILD patients evaluate...
Source: Lung - Category: Respiratory Medicine Source Type: research
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