Kidney Involvement in Systemic Sclerosis

Abstract

Background: Systemic sclerosis is a chronic multisystem autoimmune disease, characterized by diffuse fibrosis and abnormalities of microcirculation and small arterioles in the skin, joints and visceral organs. Material and Methods: We searched for the relevant articles on systemic sclerosis and kidney involvement in systemic sclerosis in the NIH library of medicine, transplant, rheumatologic and nephrological journals. Results: Half of patients with systemic sclerosis have clinical evidence of kidney involvement. Scleroderma renal crisis represents the most specific and serious renal event associated with this condition. It is characterized by an abrupt onset of moderate to marked hypertension and kidney failure. Early and aggressive treatment is mandatory to prevent irreversible organ damage and death. The advent of ACE-inhibitors revolutionized the management of scleroderma renal crisis. However, the outcomes of this serious complication are still poor, and between 20 to 50% of patients progress to end stage renal disease. Conclusions: Scleroderma renal crisis still represents a serious and life-threatening event. Thus, further studies on its prevention and on new therapeutic strategies should be encouraged.

National Trends in Mortality and Urgent Dialysis after Acute Hypertension in Japan From 2010 Through 2019

“…Early detection and intervention are important because the outcomes remain poor, with 20% to 50% of patients developing end-stage renal disease. 37 Secondary hypertension is reported to be more common in patients with hypertensive emergencies compared with the other hypertensive population. 38 In our analysis, secondary hypertension was relatively common, especially in MHT, with SLE being a mortality risk and scleroderma associated with an urgent dialysis risk.…”

Biomarkers in Systemic Sclerosis: An Overview

“…Scleroderma renal crisis (CRS) is the most frequent renal complication in SSc representing a medical emergency [19,[141][142][143][144][145][146][147][148][149][150]. The use of ACE inhibitors has reduced the occurrence of CRS.…”

“…CRS is characterized by malignant hypertension, microangiopathic haemolysis, microthrombosis, thrombocytopenia, vasospasm, and progressive renal failure which can be caused by a variety of causes, such as various drugs (e.g., corticosteroids, cyclosporine, and tacrolimus) [19,[141][142][143][144][145][146][147][148][149][150]. Pathologically, CRS is characterized by rather bland or subtle findings but may show the typical "onion bulb" findings, hyperplasia of the juxtaglomerular apparatus, membranous proliferation, renovascular endothelial damage, intimal proliferation, thrombotic angiopathy, microthrombi of fibrin, hemolysis, vasospasm, vascular occlusion, ischemia, necrosis, vascular remodeling and possibly fibrosis associated with hyperreninemia and accelerated hypertension [19,[141][142][143][144][145][146][147][148][149][150]. Anti-fibrillarin antibodies, anti-RNA polymerase III antibodies, and speckled pattern ANA have been closely associated with the development of SRD; however, anti-topoisomerase antibodies have also been associated with a high incidence of CRS in some populations [19,[141][142][143][144][145][146][147][148][149][150].…”

Autoimmunity, autoinflammation and kidney

“…У 10% больных системной склеродермией развивается почечный криз, характеризующийся внезапным появлением артериальной гипертонии, в том числе злокачественной, и почечной недостаточности при отсутствии признаков гломерулонефрита [17]. Причиной почечного криза считают ухудшение перфузии почек вследствие вазоспазма и сужения почечных артериол, которые нередко осложняются тромбозом (ТМА).…”

“…Признаки ТМА нередко наблюдаются у пациентов со склеродермическим почечным кризом, в основе патогенеза которого лежат эндотелиальная дисфункция и повреждение почечных сосудов, вызывающие актива-АКТУАЛЬНАЯ ПРОБЛЕМА КЛИНИЧЕСКАЯ ФАРМАКОЛОГИЯ И ТЕРАПИЯ, 2022, 31 (4) цию коагуляции и образование тромбов в микрососудах [17]. ТМА встречается и при других аутоиммунных заболеваниях, в том числе АНЦА-ассоциированном васкулите.…”