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Relationship between changes observed in nailfold capillaroscopy and serological profile, lung fibrosis, and elevated risk of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease

INTRODUCTION: Microvascular changes play a significant role in systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The most serious complications of SSc and MCTD are lung fibrosis (LF) and pulmonary hypertension (PH). AIM: To determine the relationship of the changes observed in cap...

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Autores principales: Niklas, Karolina, Niklas, Arkadiusz, Mularek-Kubzdela, Tatiana, Puszczewicz, Mariusz, Samborski, Włodzimierz
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Termedia Publishing House 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9704465/
https://www.ncbi.nlm.nih.gov/pubmed/36457689
http://dx.doi.org/10.5114/ada.2022.120882
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author Niklas, Karolina
Niklas, Arkadiusz
Mularek-Kubzdela, Tatiana
Puszczewicz, Mariusz
Samborski, Włodzimierz
author_facet Niklas, Karolina
Niklas, Arkadiusz
Mularek-Kubzdela, Tatiana
Puszczewicz, Mariusz
Samborski, Włodzimierz
author_sort Niklas, Karolina
collection PubMed
description INTRODUCTION: Microvascular changes play a significant role in systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The most serious complications of SSc and MCTD are lung fibrosis (LF) and pulmonary hypertension (PH). AIM: To determine the relationship of the changes observed in capillaries with the serological profile, LF, PH, and finger ulcerations in patients with SSc and MCTD. MATERIAL AND METHODS: The tested group comprised 80 persons (61 SSc, 19 MCTD); mean age 53.6 ±13.6 years. Patients were qualified to the LF group based on HRCT. Likelihood of PH was determined using echocardiography. The presence of antinuclear antibodies (ANA) was assessed using indirect immunofluorescence, while ANA profile, and sclerosis profile were assessed using EUROIMMUN kits, and antiphospholipid antibodies (aPL) using the ELISA method. Capillaroscopy was performed using the Nikon CPS 160 optical microscope. RESULTS: The following were found: a relationship between occurrence of anti-SS-A (p = 0.006) and anti-centromere B antibodies (p = 0.012) and ramified vessels, between anti-SS-B and capillary haemorrhages (p = 0.019), a positive correlation between NOR90 antibodies and winding loops (p = 0.021), PM-Scl 100 antibodies and enlarged vessels (p = 0.033), a negative correlation between Scl-70 antibodies and winding loops (p = 0.033), and a relationship between aCL and winding loops (p = 0.002). No relationship between the capillaroscopy image and PH risk was found. A positive correlation was found between avascularisation areas and LF and between giant capillaries and finger ulcerations. A negative correlation was found between U1-RNP antibodies and finger ulcerations (p = 0.009), and a positive correlation between antibodies to fibrillarin and ulcerations (p = 0.028). CONCLUSIONS: SS-A, SS-B and anti-centromere antibodies are associated with the late phase of sclerodermic microangiopathy. Avascularisation areas significantly correlate with a higher prevalence of LF. U1-RNP antibodies have a protective role, while anti-fibrillarin antibodies are the risk factor for finger ulcerations.
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spelling pubmed-97044652022-11-30 Relationship between changes observed in nailfold capillaroscopy and serological profile, lung fibrosis, and elevated risk of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease Niklas, Karolina Niklas, Arkadiusz Mularek-Kubzdela, Tatiana Puszczewicz, Mariusz Samborski, Włodzimierz Postepy Dermatol Alergol Original Paper INTRODUCTION: Microvascular changes play a significant role in systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The most serious complications of SSc and MCTD are lung fibrosis (LF) and pulmonary hypertension (PH). AIM: To determine the relationship of the changes observed in capillaries with the serological profile, LF, PH, and finger ulcerations in patients with SSc and MCTD. MATERIAL AND METHODS: The tested group comprised 80 persons (61 SSc, 19 MCTD); mean age 53.6 ±13.6 years. Patients were qualified to the LF group based on HRCT. Likelihood of PH was determined using echocardiography. The presence of antinuclear antibodies (ANA) was assessed using indirect immunofluorescence, while ANA profile, and sclerosis profile were assessed using EUROIMMUN kits, and antiphospholipid antibodies (aPL) using the ELISA method. Capillaroscopy was performed using the Nikon CPS 160 optical microscope. RESULTS: The following were found: a relationship between occurrence of anti-SS-A (p = 0.006) and anti-centromere B antibodies (p = 0.012) and ramified vessels, between anti-SS-B and capillary haemorrhages (p = 0.019), a positive correlation between NOR90 antibodies and winding loops (p = 0.021), PM-Scl 100 antibodies and enlarged vessels (p = 0.033), a negative correlation between Scl-70 antibodies and winding loops (p = 0.033), and a relationship between aCL and winding loops (p = 0.002). No relationship between the capillaroscopy image and PH risk was found. A positive correlation was found between avascularisation areas and LF and between giant capillaries and finger ulcerations. A negative correlation was found between U1-RNP antibodies and finger ulcerations (p = 0.009), and a positive correlation between antibodies to fibrillarin and ulcerations (p = 0.028). CONCLUSIONS: SS-A, SS-B and anti-centromere antibodies are associated with the late phase of sclerodermic microangiopathy. Avascularisation areas significantly correlate with a higher prevalence of LF. U1-RNP antibodies have a protective role, while anti-fibrillarin antibodies are the risk factor for finger ulcerations. Termedia Publishing House 2022-11-09 2022-10 /pmc/articles/PMC9704465/ /pubmed/36457689 http://dx.doi.org/10.5114/ada.2022.120882 Text en Copyright: © 2022 Termedia Sp. z o. o. https://creativecommons.org/licenses/by-nc-sa/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License, allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
spellingShingle Original Paper
Niklas, Karolina
Niklas, Arkadiusz
Mularek-Kubzdela, Tatiana
Puszczewicz, Mariusz
Samborski, Włodzimierz
Relationship between changes observed in nailfold capillaroscopy and serological profile, lung fibrosis, and elevated risk of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease
title Relationship between changes observed in nailfold capillaroscopy and serological profile, lung fibrosis, and elevated risk of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease
title_full Relationship between changes observed in nailfold capillaroscopy and serological profile, lung fibrosis, and elevated risk of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease
title_fullStr Relationship between changes observed in nailfold capillaroscopy and serological profile, lung fibrosis, and elevated risk of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease
title_full_unstemmed Relationship between changes observed in nailfold capillaroscopy and serological profile, lung fibrosis, and elevated risk of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease
title_short Relationship between changes observed in nailfold capillaroscopy and serological profile, lung fibrosis, and elevated risk of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease
title_sort relationship between changes observed in nailfold capillaroscopy and serological profile, lung fibrosis, and elevated risk of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease
topic Original Paper
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9704465/
https://www.ncbi.nlm.nih.gov/pubmed/36457689
http://dx.doi.org/10.5114/ada.2022.120882
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