scholarly journals Therapeutic molecular targets of SSc-ILD

2020 ◽  
Vol 5 (2_suppl) ◽  
pp. 17-30 ◽  
Author(s):  
Yun Zhang ◽  
Jörg HW Distler

Systemic sclerosis is a fibrosing chronic connective tissue disease of unknown etiology. A major hallmark of systemic sclerosis is the uncontrolled and persistent activation of fibroblasts, which release excessive amounts of extracellular matrix, lead to organ dysfunction, and cause high mobility and motility of patients. Systemic sclerosis–associated interstitial lung disease is one of the most common fibrotic organ manifestations in systemic sclerosis and a major cause of death. Treatment options for systemic sclerosis–associated interstitial lung disease and other fibrotic manifestations, however, remain very limited. Thus, there is a huge medical need for effective therapies that target tissue fibrosis, vascular alterations, inflammation, and autoimmune disease in systemic sclerosis–associated interstitial lung disease. In this review, we discuss data suggesting therapeutic ways to target different genes in distinct tissues/organs that contribute to the development of SSc.

Author(s):  
Tariq J Cheema ◽  
Meilin Young ◽  
Erica Rabold ◽  
Ashley N Barbieri ◽  
Nancy Baldwin ◽  
...  

Systemic sclerosis–associated interstitial lung disease is challenging to diagnose and treat. Patients and physicians can perceive the disease differently and have different views on its management. Communication issues between them can lead to suboptimal disease management. Despite a clear need for improvement in the speed and accuracy of the diagnostic workup, the heterogeneity of clinical symptoms renders the process long and challenging. When considering treatment options, physicians may be more focused on the evidence supporting a particular treatment or on a patient’s pulmonary function test results, as opposed to the realities of the patient’s difficulties with symptoms or the psychosocial effects of systemic sclerosis–associated interstitial lung disease. Disease management plans should be determined by the patient’s own preferences and goals as well as the objective clinical situation. Health care providers must consider their patients as partners on a journey in which treatment decisions are reached jointly. This review will focus on the perspectives of physicians and patients in relation to the diagnosis and management of systemic sclerosis–associated interstitial lung disease. Similarities and differences in these perspectives will be identified, and strategies for achieving optimal disease management will be proposed.


Author(s):  
Hidekata Yasuoka

Systemic sclerosis (SSc) is a disorder characterized by immune dysfunction, microvascular injury, and fibrosis. Organ involvement in patients with SSc is variable; however, pulmonary involvement occurs in up to 90% of patients with SSc. Interstitial lung disease (ILD) is a major cause of mortality and, thus, a major determinant in the prognosis of patients with SSc. This review summarizes current findings about the characteristics of ILD in patients with SSc, selection of patients with SSc-ILD who are candidates for the treatment, and current treatment options.


Dermato ◽  
2021 ◽  
Vol 1 (2) ◽  
pp. 59-70
Author(s):  
Karolina Krawczyk ◽  
Ewelina Mazur ◽  
Jaromir Kargol ◽  
Robert Kijowski ◽  
Adam Reich

About 20% of patients with systemic sclerosis have symptoms of another connective tissue disease (CTD). Interstitial lung disease (ILD) is one of the most common organ manifestations in systemic sclerosis (SSc) as well as viral illnesses, such as COVID-19, and can lead not only to diffuse alveolar damage, but also trigger an exacerbation of fibrosis among patients with preexisting ILD. It is also associated with substantial morbidity and mortality. According to the World Scleroderma Foundation, SSc-ILD can mask or mimic early COVID-19 lesions and there are no available computed tomography guidelines on how to discern those two conditions. We present a case of systemic sclerosis exacerbation after COVID-19 in a patient with SSc-Lupus Overlap Syndrome.


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