Therapy of scleroderma renal crisis: State of the art

Autoimmunity Reviews
Elisabetta ZanattaAndrea Doria

Abstract

Scleroderma renal crisis (SRC) is an uncommon but still life-threatening manifestation of systemic sclerosis (SSc). The incidence of SRC has decreased in the last few decades, probably due to a widespread use of vasodilators in SSc patients. It is well-recognized that exposure to different drugs can trigger SRC (corticosteroids, cyclosporine) or might prevent its occurrence (iloprost, calcium channel blockers). The prognosis of this life-threatening manifestation has not substantially improved since 1980s, when ACE-inhibitors were introduced in its treatment. ACE-inhibitors remain the mainstay in the therapy of SRC due to their efficacy in controlling malignant hypertension; indeed, the prognosis largely depends on the rapid improvement of the ongoing renal ischemia. Calcium-channel blockers and in third line diuretics and alpha-blockers should be used as additional therapy if blood pressure control remains suboptimal despite maximum tolerated doses of ACE-inhibitors. Given the growing evidence on the role of complement activation and endothelin-1 in the pathogenesis of SRC, recent case-series and case reports have suggested the use of C5-inhibitors and endothelin receptor antagonists in the therapy of SRC, mainly in the refrac...Continue Reading

Citations

Jun 21, 2020·Arthritis & Rheumatology·Robert W Simms
Jun 7, 2019·Clinical Reviews in Allergy & Immunology·Carlo Selmi
Jun 13, 2020·Journal of Nephrology·Claudio PonticelliGabriella Moroni
Mar 30, 2019·British Journal of Haematology·Elisabetta ZanattaFranco Cozzi
Oct 21, 2020·Expert Review of Clinical Immunology·Francesco BenvenutiAndrea Doria
Feb 9, 2021·European Journal of Internal Medicine·Cosimo BruniMarco Matucci-Cerinic
Mar 20, 2021·Frontiers in Medicine·Francesco PesceLoreto Gesualdo
Apr 21, 2021·Autoimmunity Reviews·Carlos-Hugo Escobar-SotoAdriana Rojas-Villarraga
Jun 26, 2021·Scandinavian Journal of Rheumatology·C-Y LinC-Y Hsu
Jul 1, 2020·Terapevticheskiĭ arkhiv·E L NasonovZ S Alekberova
Jun 11, 2020·Kidney & Blood Pressure Research·Magdalena ChrabaszczLidia Rudnicka

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