CURRENT VIEWS ON THE HETEROGENEITY OF RENAL INVOLVEMENTS IN PATIENTS WITH SYSTEMIC SCLEROSIS

Systemic sclerosis (SS), is an autoimmune connective tissue disease, the main clinical signs of which are due to disseminated microcirculatory disorders and fibrosis of the skin and viscera. Morphological examinations showed that 80% of patients with SS had renal changes, including those unassociate...

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Main Authors: A. V. Gordeev, A. Yu. Zakharova, Z. Yu. Mutovina, L. P. Ananyeva
Format: Article
Language:Russian
Published: IMA PRESS LLC 2015-09-01
Series:Научно-практическая ревматология
Subjects:
Online Access:https://rsp.mediar-press.net/rsp/article/view/2116
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author A. V. Gordeev
A. Yu. Zakharova
Z. Yu. Mutovina
L. P. Ananyeva
author_facet A. V. Gordeev
A. Yu. Zakharova
Z. Yu. Mutovina
L. P. Ananyeva
author_sort A. V. Gordeev
collection DOAJ
description Systemic sclerosis (SS), is an autoimmune connective tissue disease, the main clinical signs of which are due to disseminated microcirculatory disorders and fibrosis of the skin and viscera. Morphological examinations showed that 80% of patients with SS had renal changes, including those unassociated with rheumatic diseases. Whereas the prevalence of scleroderma renal crisis is now estimated to be 2–5%, there is considerably often an asymptomatic reduction in renal function (silent uremia), which is caused by multimorbidity and comorbidity. Its incidence in patients with SS may be as high as 55%. The presence of autoimmune connective tissue disease may be itself regarded as a risk factor of renal involvement. Fifteen-year survival is 72% in SS patients with no renal involvement and not more than 13% in those having renal involvement. In patients with SS, proteinuria is one of the most important independent risk factors for fatal outcomes (relative risk, 3.34), leaving far behind canonical risk factors, such as pulmonary hypertension, restrictive lung disease (a forced expiratory volume in one second to forced vital capacity ratio of <80%), respiratory failure (NYHA Class III and IV), as well as decreased lung diffuse capacity and high skin scores. The authors first propose a concept of the existence and pathogenesis of chronic scleroderma nephropathy, the basis for which is the vascular endothelial dysfunction phenomenon developing in different structural components of the nephron and kidney as a whole.
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spelling doaj.art-b4c8ad4b7cfb4d3894e01e07824da98f2023-03-22T13:45:50ZrusIMA PRESS LLCНаучно-практическая ревматология1995-44841995-44922015-09-0153434344510.14412/1995-4484-2015-343-4452001CURRENT VIEWS ON THE HETEROGENEITY OF RENAL INVOLVEMENTS IN PATIENTS WITH SYSTEMIC SCLEROSISA. V. Gordeev0A. Yu. Zakharova1Z. Yu. Mutovina2L. P. Ananyeva3V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia; 2Training and Research Medical Center, Department for Presidential Affairs of the Russian Federation, Moscow, Russia 34A, Kashirskoe Shosse, Moscow 115522V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia; 2Training and Research Medical Center, Department for Presidential Affairs of the Russian Federation, Moscow, Russia 34A, Kashirskoe Shosse, Moscow 115522Training and Research Medical Center, Department for Presidential Affairs of the Russian Federation, Moscow, Russia 19, Marshal Timoshenko St., Build 1A, Moscow 121359V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia; 2Training and Research Medical Center, Department for Presidential Affairs of the Russian Federation, Moscow, Russia 34A, Kashirskoe Shosse, Moscow 115522Systemic sclerosis (SS), is an autoimmune connective tissue disease, the main clinical signs of which are due to disseminated microcirculatory disorders and fibrosis of the skin and viscera. Morphological examinations showed that 80% of patients with SS had renal changes, including those unassociated with rheumatic diseases. Whereas the prevalence of scleroderma renal crisis is now estimated to be 2–5%, there is considerably often an asymptomatic reduction in renal function (silent uremia), which is caused by multimorbidity and comorbidity. Its incidence in patients with SS may be as high as 55%. The presence of autoimmune connective tissue disease may be itself regarded as a risk factor of renal involvement. Fifteen-year survival is 72% in SS patients with no renal involvement and not more than 13% in those having renal involvement. In patients with SS, proteinuria is one of the most important independent risk factors for fatal outcomes (relative risk, 3.34), leaving far behind canonical risk factors, such as pulmonary hypertension, restrictive lung disease (a forced expiratory volume in one second to forced vital capacity ratio of <80%), respiratory failure (NYHA Class III and IV), as well as decreased lung diffuse capacity and high skin scores. The authors first propose a concept of the existence and pathogenesis of chronic scleroderma nephropathy, the basis for which is the vascular endothelial dysfunction phenomenon developing in different structural components of the nephron and kidney as a whole.https://rsp.mediar-press.net/rsp/article/view/2116systemic sclerosisscleroderma renal crisisvascular endothelial dysfunctionchronic scleroderma nephropathynonimmune factors of progressive nephropathieschronic kidney disease
spellingShingle A. V. Gordeev
A. Yu. Zakharova
Z. Yu. Mutovina
L. P. Ananyeva
CURRENT VIEWS ON THE HETEROGENEITY OF RENAL INVOLVEMENTS IN PATIENTS WITH SYSTEMIC SCLEROSIS
Научно-практическая ревматология
systemic sclerosis
scleroderma renal crisis
vascular endothelial dysfunction
chronic scleroderma nephropathy
nonimmune factors of progressive nephropathies
chronic kidney disease
title CURRENT VIEWS ON THE HETEROGENEITY OF RENAL INVOLVEMENTS IN PATIENTS WITH SYSTEMIC SCLEROSIS
title_full CURRENT VIEWS ON THE HETEROGENEITY OF RENAL INVOLVEMENTS IN PATIENTS WITH SYSTEMIC SCLEROSIS
title_fullStr CURRENT VIEWS ON THE HETEROGENEITY OF RENAL INVOLVEMENTS IN PATIENTS WITH SYSTEMIC SCLEROSIS
title_full_unstemmed CURRENT VIEWS ON THE HETEROGENEITY OF RENAL INVOLVEMENTS IN PATIENTS WITH SYSTEMIC SCLEROSIS
title_short CURRENT VIEWS ON THE HETEROGENEITY OF RENAL INVOLVEMENTS IN PATIENTS WITH SYSTEMIC SCLEROSIS
title_sort current views on the heterogeneity of renal involvements in patients with systemic sclerosis
topic systemic sclerosis
scleroderma renal crisis
vascular endothelial dysfunction
chronic scleroderma nephropathy
nonimmune factors of progressive nephropathies
chronic kidney disease
url https://rsp.mediar-press.net/rsp/article/view/2116
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