scholarly journals Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions

2007 ◽  
Vol 9 (Suppl 2) ◽  
pp. S4 ◽  
Author(s):  
Thomas Krieg ◽  
David Abraham ◽  
Robert Lafyatis
2019 ◽  
Vol 40 (02) ◽  
pp. 173-183 ◽  
Author(s):  
Hossam Fayed ◽  
J. Gerry Coghlan

Pulmonary hypertension (PH) is common in most forms of connective tissue disease (CTD); the prevalent type of PH depends on the particular CTD. Thus, pulmonary arterial hypertension (PAH) is dominantly associated with scleroderma, while postcapillary PH is most common in rheumatoid arthritis and lung disease-associated PH is typically found in myositis and sarcoidosis.Considerable expertise is required to identify, diagnose, and manage CTD-PH, as the primary physicians providing the majority of care for this population, rheumatologists, need a good working knowledge of CTD-PH, its rather subtle presentation, and how to access the necessary investigations to screen for and identify patients with PH. The role of the rheumatologist does not stop at diagnosis; in some conditions such as lupus, optimizing immunosuppression is key to the management of PH, and unlike simple idiopathic PAH, the natural history of CTD-PH is often punctuated by complications of the CTD rather than just events due to progression of PH or therapy-related adverse events.The aim of this article is to provide an overview of all forms of CTD-PH, and to provide an easy reference source on current best practice.


2019 ◽  
Vol 51 (2) ◽  
pp. 101-108 ◽  
Author(s):  
Tian Zhu ◽  
Yiman Wang ◽  
Hongzhong Jin ◽  
Li Li

2013 ◽  
Vol 39 (6) ◽  
pp. 728-741 ◽  
Author(s):  
Daniel Antunes Silva Pereira ◽  
Alexandre de Melo Kawassaki ◽  
Bruno Guedes Baldi

The initial evaluation of patients with interstitial lung disease (ILD) primarily involves a comprehensive, active search for the cause. Autoantibody assays, which can suggest the presence of a rheumatic disease, are routinely performed at various referral centers. When interstitial lung involvement is the condition that allows the definitive diagnosis of connective tissue disease and the classical criteria are met, there is little debate. However, there is still debate regarding the significance, relevance, specificity, and pathophysiological role of autoimmunity in patients with predominant pulmonary involvement and only mild symptoms or formes frustes of connective tissue disease. The purpose of this article was to review the current knowledge of autoantibody positivity and to discuss its possible interpretations in patients with ILD and without clear etiologic associations, as well as to enhance the understanding of the natural history of an allegedly new disease and to describe the possible prognostic implications. We also discuss the proposition of a new term to be used in the classification of ILDs: lung-dominant connective tissue disease.


2017 ◽  
Vol 38 (05) ◽  
pp. 619-635 ◽  
Author(s):  
Rajan Saggar ◽  
John Belperio ◽  
Elizabeth Volkmann ◽  
Augustine Chung ◽  
Joseph Lynch

Pulmonary hypertension (PH) may complicate connective tissue disease (CTD; particularly systemic sclerosis [scleroderma]), and is associated with increased mortality. More than 70% of cases of PH complicating CTD occur in patients with systemic sclerosis (SSc), which is the major focus of this article. Pulmonary complications (i.e., interstitial lung disease [ILD] and PH) are the leading causes of SSc-related deaths. “Isolated” PH (i.e., without ILD) complicates SSc in 7.5 to 20% of cases; secondary PH may also occur in patients with SSc-associated ILD. Several clinical markers and specific autoantibody profiles have been associated with PH in SSc. The role of PH-specific therapy in improving CTD-PH outcomes is under investigation, as prognosis and responsiveness to therapy appear to be worse in SSc-associated PH compared with idiopathic pulmonary arterial hypertension. We discuss medical therapies for CTD-associated PH and the role of lung transplantation for patients who fail medical therapy.


PLoS ONE ◽  
2012 ◽  
Vol 7 (3) ◽  
pp. e33362 ◽  
Author(s):  
Celia Murciano ◽  
David L. Moyes ◽  
Manohursingh Runglall ◽  
Priscila Tobouti ◽  
Ayesha Islam ◽  
...  

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