Pulmonary involvement in systemic sclerosis: exploring cellular, genetic and epigenetic mechanisms

Doskaliuk, Bohdana; Zaiats, L. M.; Yatsyshyn, Roman; Gerych, Petro; Cherniuk, Natalia; Zimba, Olena

doi:10.1007/s00296-020-04658-6

Cited by 14 publications

(6 citation statements)

References 167 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…After 6 weeks of treatments, this animal revealed significant lung abnormalities. The perivascular inflammatory cell infiltration and microvessel wall thickening were presented in this animal model [27 ▪ ]. Taken together, HOCl induced animal model decapsulates specific oxidation in the autoantibody production and is a useful tool for studying pathogenesis of oxidant stress ROS pathways and autoantibodies in SSc.…”

Section: Hypochlorous Acid-induced Animal Model Of Systemic Sclerosismentioning

confidence: 78%

“…Cytokines including IL-1β, IL-6, IL-17, IL-33, and tumor necrosis factor (TNF)-α were also increased in HOCl-induced mice [25,26 ▪ ]. Another interesting study utilized adult Wistar rats to administer subcutaneous injection of 5% sodium hypochlorite (NaClO) three times a week for 6 consecutive weeks [27 ▪ ]. NaOCl breaks down to form HOCL [28].…”

Section: Hypochlorous Acid-induced Animal Model Of Systemic Sclerosismentioning

confidence: 99%

See 1 more Smart Citation

Animal models in systemic sclerosis: an update

Bi,

Mills,

2023

Current Opinion in Rheumatology

View full text Add to dashboard Cite

Purpose of review Systemic sclerosis (SSc) is a multisystem autoimmune connective tissue disease characterized by early inflammation followed by excessive fibrosis in the skin and internal organs. Enhancing our comprehension of SSc pathogenesis is essential to develop effective therapeutic strategies. Animal models that mimic one or more aspects of SSc have been proven to be a valuable resource for investigating disease mechanisms. This review aims to provide an updated overview of the existing SSc animal models and the potentially relevant pathways to SSc pathogenesis. Recent findings This review focuses on the most recently generated and investigated animal models, which delve into novel pathways beyond existing models or employ genetic technologies to gain a deeper understanding of SSc pathogenesis including activation of early type I interferon (IFN) signaling pathway, immune cell function and pulmonary artery hypertension (PAH). Summary While no single animal model can fully replicate SSc, a combination of different models can offer valuable insights into the pathways involved in the onset and advancement of the SSc. These insights can prove animal models as a crutial preclinical tool for developing effective treatments for SSc.

show abstract

Section: Hypochlorous Acid-induced Animal Model Of Systemic Sclerosismentioning

confidence: 78%

Section: Hypochlorous Acid-induced Animal Model Of Systemic Sclerosismentioning

confidence: 99%

Animal models in systemic sclerosis: an update

Bi,

Mills,

2023

Current Opinion in Rheumatology

View full text Add to dashboard Cite

show abstract

“…Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by immune inflammation, vasculopathy, and enhanced fibrosis [ 1 ]. Pulmonary involvement is a common finding in SSc, occurring in up to 70–90% of cases and can be due to parenchymal and vascular disorders [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Spontaneous Pneumo-Mediastinum in a Post-COVID-19 Patient with Systemic Sclerosis

Mormile

Rea

et al. 2022

Healthcare

View full text Add to dashboard Cite

Pulmonary involvement is the most common cause of death among patients with systemic sclerosis (SSc). The current coronavirus disease 2019 (COVID-19) is particularly problematic to manage in SSc patients since they may experience a more severe evolution of COVID-19 due to the pre-existent interstitial lung disease (ILD) and the administration of immunosuppressive treatments. In addition, the remarkable radiological similarities between SSc-ILD and COVID-19 complicate the differential diagnosis between these two entities. Herein, we present the first case of spontaneous pneumo-mediastinum in a post-COVID-19 patient with SSc. In our patient, both smoking and pulmonary fibrosis could lead to cyst formation, which possibly spontaneously broke and caused pneumo-mediastinum. Moreover, megaesophagus perforation due to the smooth muscle atrophy, replacement with fibrosis, and achalasia may extend into the mediastinum or pleural space and has also been described as a rare case of spontaneous pneumo-pericardium. Finally, spontaneous pneumo-mediastinum and pneumothorax have been recently reported as an established complication of severe COVID-19 pneumonia and among COVID-19 long-term complication. This case report underlines that the worsening of respiratory symptoms in SSc patients, especially when recovered from COVID-19, requires further investigations for ruling out other tentative diagnoses besides the evolution of the SSc-ILD.

show abstract

“…Systemic sclerosis involves multiple organs through the deposition of brotic tissue and microvascular obliteration [1]. Pulmonary involvement affects all organ systems [2], and myocardial brosis deposits before signs or symptoms of heart failure are observed in those with systemic sclerosis [3], which leads to left ventricular diastolic dysfunction detected by echocardiography. Longitudinal assessment over 3.4 years of follow-up demonstrated the pathological progress in the parameters such as the early lling to early diastolic mitral annular velocity ratio (E/E' ratio) and tricuspid regurgitant maximum velocity [4].…”

Section: Introductionmentioning

confidence: 99%

Lung Diffusion Capacity Affects Stroke Volume in Systemic Sclerosis

Someya

Mugii

2020

Preprint

View full text Add to dashboard Cite

Objective: In our previous cross-sectional study, we reported impaired stroke volume in patients with systemic sclerosis; however, no cardiac parameter using echocardiography was found to be related to stroke volume reduction. As few studies have focused on the hemodynamic clinical course, the patients were re-evaluated in a median interval of 18 months, and correlations between stroke volume and cardiopulmonary parameters were analyzed.Results: Seventeen patients who were able to complete the 6-minute walk test wearing a non-invasive impedance cardiograph device were assigned for re-evaluation, and individual changes in the stroke volume were noted. The stroke volume was not related to cardiac parameters, but it was significantly related to the diffusion capacity of the lung for carbon monoxide after walking in the first evaluation (r = 0.63, p = 0.006), and at rest in the re-evaluation (r = 0.72, p = 0.001). Significant relation was also found between the distance walked and the stroke volume after walking in the first evaluation and re-evaluation, and at rest in the re-evaluation. Thus, a limited lung diffusion capacity may affect stroke volume due to the exercise intolerance in systemic sclerosis without pathological heart involvement.

show abstract

Pulmonary involvement in systemic sclerosis: exploring cellular, genetic and epigenetic mechanisms

Cited by 14 publications

References 167 publications

Animal models in systemic sclerosis: an update

Animal models in systemic sclerosis: an update

Spontaneous Pneumo-Mediastinum in a Post-COVID-19 Patient with Systemic Sclerosis

Lung Diffusion Capacity Affects Stroke Volume in Systemic Sclerosis

Contact Info

Product

Resources

About