Pulmonary involvement in systemic sclerosis: exploring cellular, genetic and epigenetic mechanisms
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Rheumatology International https://doi.org/10.1007/s00296-020-04658-6
INTERNATIONAL
REVIEW
Pulmonary involvement in systemic sclerosis: exploring cellular, genetic and epigenetic mechanisms Bohdana Doskaliuk1,2 · Liubomyr Zaiats2 · Roman Yatsyshyn1 · Petro Gerych1 · Nataliia Cherniuk1 · Olena Zimba3 Received: 29 June 2020 / Accepted: 20 July 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Systemic sclerosis (SSc) is a chronic progressive autoimmune disease characterized by immune inflammation, vasculopathy, and fibrosis. There are still numerous uncertainties in the understanding of disease initiation and progression. Pulmonary involvement in SSc, and particularly pulmonary fibrosis, is critical for all organ systems affections in this disease. This review is aimed to describe and analyze new findings in the pathophysiology of SSc-associated pulmonary involvement and to explore perspective diagnostic and therapeutic strategies. A myriad of cellular interactions is explored in the dynamics of progressive interstitial lung disease (ILD) and pulmonary hypertension (PH) in SSc. The role of exosomes, microvesicles, and apoptotic bodies is examined and the impact of micro and long non-coding RNAs, DNA methylation, and histone modification in SSc is discussed. Keywords Systemic sclerosis · Interstitial lung disease · Pulmonary hypertension · Genetic epigenesis · Extracellular vesicles
Introduction
* Bohdana Doskaliuk [email protected] Liubomyr Zaiats [email protected] Roman Yatsyshyn [email protected] Petro Gerych [email protected] Nataliia Cherniuk [email protected] Olena Zimba [email protected] 1
Academician Ye. M. Neiko Department of Internal Medicine #1, Clinical Immunology and Allergology, Ivano-Frankivsk National Medical University, Ivano‑Frankivsk, Ukraine
2
Department of Pathophysiology, Ivano-Frankivsk National Medical University, Halytska Str. 2, Ivano‑Frankivsk 76000, Ukraine
3
Department of Internal Medicine #2, Danylo Halytsky Lviv National Medical University, Lviv, Ukraine
Systemic sclerosis (SSc) is an autoimmune disease that belongs to the systemic connective tissue diseases (SCTD) family characterized by immune dysregulation, vasculopathy, and enhanced fibrosis. This rheumatic disease is unevenly distributed all over the world, with the largest number of cases registered in North America. Latest epidemiological studies have recorded the prevalence of SSc ranging from 3.8 to 44.3 per 100,000 inhabitants across continents [1–3]. Some epidemiologic features of the disease point to the ethnic predisposition. With the progressive course of SSc affecting functions of multiple organs, premature death is often the main outcome of the disease [4]. When compared to other SCTD, SSc has the most dreadful impact on the patients’ lifespan expectancy. A steady decrease in survival rates over the disease course is widely known. The survival rate of SSc patients has increased to 89.3% for a 3-year period due to the introduction of t
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