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

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 hemo...

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Detalles Bibliográficos
Autores principales: Uriarte, Maite Hurtado, Larrarte, Carolina, Rey, Laura Bravo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6218791/
https://www.ncbi.nlm.nih.gov/pubmed/30425869
http://dx.doi.org/10.1155/2018/6051083
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author Uriarte, Maite Hurtado
Larrarte, Carolina
Rey, Laura Bravo
author_facet Uriarte, Maite Hurtado
Larrarte, Carolina
Rey, Laura Bravo
author_sort Uriarte, Maite Hurtado
collection PubMed
description 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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spelling pubmed-62187912018-11-13 Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab Uriarte, Maite Hurtado Larrarte, Carolina Rey, Laura Bravo Case Rep Nephrol Case Report 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. Hindawi 2018-10-23 /pmc/articles/PMC6218791/ /pubmed/30425869 http://dx.doi.org/10.1155/2018/6051083 Text en Copyright © 2018 Maite Hurtado Uriarte et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Uriarte, Maite Hurtado
Larrarte, Carolina
Rey, Laura Bravo
Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab
title Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab
title_full Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab
title_fullStr Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab
title_full_unstemmed Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab
title_short Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab
title_sort scleroderma renal crisis debute with thrombotic microangiopathy: a successful case treated with eculizumab
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6218791/
https://www.ncbi.nlm.nih.gov/pubmed/30425869
http://dx.doi.org/10.1155/2018/6051083
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