Complement activation and effect of eculizumab in scleroderma renal crisis

Devresse, Arnaud; Aydın, Selda; Quintrec, Moglie Le; Demoulin, Nathalie; Stordeur, Patrick; Lambert, Catherine; Gastoldi, Sara; Pirson, Yves; Jadoul, Michel; Morelle, Johann

doi:10.1097/md.0000000000004459

Cited by 61 publications

(48 citation statements)

References 22 publications

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“…We do not know with certainty the physiopathology, but recent findings suggest that an activation of the complement system through the classical pathway may be are involved. It is believed that certain proteins are involved in either promoting or maintaining an inflammatory state, such as a variation on factor H as associated with endothelial cells damage [11, 12]. …”

Section: Discussion–conclusionmentioning

confidence: 99%

Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab

Uriarte

Larrarte

Rey

2018

Case Reports in Nephrology

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We had the challenged to treat a 40-year-old female with Systemic Scleroderma who was showing unspecific symptoms. During her time at the hospital she rapidly develops renal dysfunction, associated with hypertension. She required renal replacement therapy initiation and we observed a decline in hemoglobin and platelets numbers. We confirm a microangiopathic hemolytic anemia and rule out other immune diseases or thrombotic thrombocytopenic purpura. Systemic Sclerosis is a chronic immune disorder of unknown origin that it is not completely understood. It is believed that environmental factors may contribute and also altered genes may be implicated in the immune system's function. Microangiopathic hemolytic anemia occurs in 43% of patients who develop scleroderma renal crisis and an activation of the complement system through the classical pathway may be involved. Given that context we decided to treat the patients with C5 blocker Eculizumab and obtain an extraordinary positive response.

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Section: Discussion–conclusionmentioning

confidence: 99%

Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab

Uriarte

Larrarte

Rey

2018

Case Reports in Nephrology

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“…The drug inhibits the generation of C5a and C5b-9, and thus inhibits lysis and endothelial damage [59]. The rationale for the involvement of the complement system in the pathogenesis of SRC, as well as for the usage of eculizumab, is as follows: an association of hypocomplementemia with SSc and vascular involvement, occurrence of microangiopathic hemolytic anemia in SRC, C5b-9 deposits in capillaries of SSc patients’ skin biopsies and C4d deposits in renal peritubular capillaries of SSc patients with a poor renal outcome [60].…”

Section: Scleroderma Renal Crisismentioning

confidence: 99%

“…Chemerin, one of the adipokines, appears to be a promising marker of increased risk of impaired renal function in the early stage of SSc [71, 72]. Adipsin was suggested to take part in the pathogenesis of SRC due to an alternative pathway of complement activation [60]. Apelin, the secretion of which is decreased in SSc, was reported to inhibit the fibrotic process in numerous organs including the kidneys [73].…”

Section: Scleroderma Renal Crisismentioning

confidence: 99%

Renal Involvement in Systemic Sclerosis: An Update

Chrabąszcz

Małyszko

Sikora

et al. 2020

Kidney Blood Press Res

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Background: Systemic sclerosis is an immune-mediated rheumatic disease characterized by vascular abnormalities, tissue fibrosis and autoimmune phenomena. Summary: Renal disease occurring in patients with systemic sclerosis may have a variable clinicopathological picture. The most specific renal condition associated with systemic sclerosis is scleroderma renal crisis, characterized by acute onset of renal failure and severe hypertension. Although the management of scleroderma renal crisis was revolutionized by the introduction of angiotensin-converting enzyme inhibitors, there is still a significant proportion of patients with poor outcomes. Therefore, research on establishing disease markers (clinical, ultrasonographical and serological) and clear diagnostic criteria, which could limit the risk of developing scleroderma renal crisis and facilitate diagnosis of this complication, is ongoing. Other forms of renal involvement in systemic sclerosis include vasculitis, an isolated reduced glomerular filtration rate in systemic sclerosis, antiphospholipid-associated nephropathy, high intrarenal arterial stiffness and proteinuria. Key Messages: Scleroderma renal crisis is the most specific and lifethreatening renal presentation of systemic sclerosis, albeit with declining prevalence. In patients with scleroderma renal crisis, it is mandatory to control blood pressure early with increasing doses of angiotensin-converting enzyme inhibitors, along with other antihypertensive drugs if necessary. There is a strong association between renal involvement and patients' outcomes in systemic sclerosis; consequently, it becomes mandatory to find markers that may be used to identify patients with an especially high risk of scleroderma renal crisis.

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“…Различные биологические агенты, включая ингибиторы TGF-a, анти-CD20 (ритуксимаб), моноклональные антитела к интерлейкину-6, эцулизумаб и ингибиторы тирозинкиназы, проходят исследования для оценки эффективности терапии СПК [81].…”

Section: индукционная терапияunclassified

Особенности Поражения Почек При Системных Заболеваниях Соединительной Ткани

Golovach¹,

Yehudіna²

2021

KIDNEYS

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Погляд на проблему Looking at the Problem Введение Почки являются одним из органов, наиболее часто вовлекаемых в патологический процесс у пациентов с системными ревматологическими заболеваниями. В той или иной степени нарушение функции почек присутствует при многих системных заболеваниях соединительной ткани (СЗСТ): у 50 % больных системной красной волчанкой (СКВ), у 5 %-системной склеродермией (ССД), режепри воспалительных аутоиммунных миопатиях, ревматоидном артрите (РА) и синдроме Шегрена [1]. Почечные проявления могут варьировать от бессимптомных поражений до развития терминальной почечной недостаточности, требующей почеч

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Complement activation and effect of eculizumab in scleroderma renal crisis

Abstract: Supplemental Digital Content is available in the text

Cited by 61 publications

References 22 publications

Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab

Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab

Renal Involvement in Systemic Sclerosis: An Update

Особенности Поражения Почек При Системных Заболеваниях Соединительной Ткани

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