Abstract

MIRANDA BUCHELI, Alfredo; MORA ALFONSO, Sergio  and  SALDARRIAGA RIVERA, Lina María. Eculizumab as a therapeutic option in patients with scleroderma renal crisis associated with haemolytic uraemic syndrome. Rev.Colomb.Reumatol. [online]. 2020, vol.27, n.3, pp.205-209.  Epub Apr 25, 2021. ISSN 0121-8123.  https://doi.org/10.1016/j.rcreu.2019.08.002.

Scleroderma renal crisis (SRC) is a rare manifestation of systemic sclerosis (SSc), presented as hypertension of new onset, worsening and / or acceleration of chronic hypertension, or rapid deterioration of renal function, often accompanied by signs of microangiopathic haemolysis. It is rarely associated with haemolytic uraemic syndrome, and there is only one similar case reported in the literature. The case is presented here of a 36-year-old woman on treatment for SSc with global, pulsatile and intense headache, clonic tonic convulsions, high blood pressure levels, acute renal failure, and persistent non-autoimmune haemoly sis. The evaluation of ADAMTS13 showed 60.6% of activity. The genetic study to search for mutations predisposing to atypical haemolytic uraemic syndrome (aHUS) revealed a homozygous variant in ADAMTS13 gene, c.3287G>A (p.Arg1096His). Eculizumab was star ted, with an improvement being observed in a short time in the haemolysis, renal function, and clinical status, with some notable and unexpected beneficial effects on SSc.

Keywords : Scleroderma renal crisis; Uraemic haemolytic syndrome; Eculizumab; Progressive scleroderma.

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