Cargando…
The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement
BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening form of antiphospholipid syndrome (APS) involving many organs and leading to their insufficiency. The pathogenesis of CAPS is associated with the presence of antiphospholipid antibodies (aPL). Typical therapy inclu...
Autores principales: | , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2018
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5994130/ https://www.ncbi.nlm.nih.gov/pubmed/29884138 http://dx.doi.org/10.1186/s12882-018-0928-z |
_version_ | 1783330365793894400 |
---|---|
author | Rymarz, Aleksandra Niemczyk, Stanisław |
author_facet | Rymarz, Aleksandra Niemczyk, Stanisław |
author_sort | Rymarz, Aleksandra |
collection | PubMed |
description | BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening form of antiphospholipid syndrome (APS) involving many organs and leading to their insufficiency. The pathogenesis of CAPS is associated with the presence of antiphospholipid antibodies (aPL). Typical therapy includes anticoagulation, glucocorticoids, therapeutic plasma exchanges and/or intravenous immunoglobulin. Despite this aggressive treatment, the mortality rate of 37% is still high. Novel therapeutic agents are required. Rituximab (RTX) is the most studied drug in APS also used in CAPS. Because of the rarity of CAPS occurrence it is impossible to plan a controlled, randomized study exploring its efficacy in CAPS. Therefore, case reports of its usage can be a source of our knowledge in this matter. CASE PRESENTATION: A 35-year-old woman who displayed dyspnoea and peripheral edema was admitted to the Nephrology Clinic because of rapidly progressive renal insufficiency. Her history included autoimmune hemolytic anemia anemia, two miscarriages and the diagnosis of APS with the treatment of heparin and acetylosalicylic acid during her next pregnancy. In spite of this treatment, she gave birth to a dead fetus in 22 Hbd. She then developed CAPS with involvement of the kidneys, brain, skin, peripheral veins and central retinal artery. Lupus anticoagulant and β(2−)glicoprotein-I antibodies were positive. Immediately upon admission to the nephrology clinic, she received anticoagulation and corticosteroids along with therapeutic plasma exchanges. As a supportive treatment hemodialysis sessions were necessary. Under this treatment the amelioration of the general state was observed but renal failure persisted, therefore intravenous immunoglobulin was added. Afterwards, the kidney function recovered and the renal replacement therapy could be stopped. After this therapy, aPL became negative. Four weeks later lupus anticoagulant began to increase. Taking into account the risk factors of the relapse and the life-threatening course of the disease, rituximab was introduced. After administration of 2 g of RTX in three separate doses, we observed no new thrombotic events, the further amelioration of renal function and the negative profile of aPL. CONCLUSIONS: CAPS is a life-threatening condition and a prompt, complex treatment is necessary. Rituximab together with conventional therapy can be an additional option in case of the risk of relapse. |
format | Online Article Text |
id | pubmed-5994130 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-59941302018-06-21 The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement Rymarz, Aleksandra Niemczyk, Stanisław BMC Nephrol Case Report BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening form of antiphospholipid syndrome (APS) involving many organs and leading to their insufficiency. The pathogenesis of CAPS is associated with the presence of antiphospholipid antibodies (aPL). Typical therapy includes anticoagulation, glucocorticoids, therapeutic plasma exchanges and/or intravenous immunoglobulin. Despite this aggressive treatment, the mortality rate of 37% is still high. Novel therapeutic agents are required. Rituximab (RTX) is the most studied drug in APS also used in CAPS. Because of the rarity of CAPS occurrence it is impossible to plan a controlled, randomized study exploring its efficacy in CAPS. Therefore, case reports of its usage can be a source of our knowledge in this matter. CASE PRESENTATION: A 35-year-old woman who displayed dyspnoea and peripheral edema was admitted to the Nephrology Clinic because of rapidly progressive renal insufficiency. Her history included autoimmune hemolytic anemia anemia, two miscarriages and the diagnosis of APS with the treatment of heparin and acetylosalicylic acid during her next pregnancy. In spite of this treatment, she gave birth to a dead fetus in 22 Hbd. She then developed CAPS with involvement of the kidneys, brain, skin, peripheral veins and central retinal artery. Lupus anticoagulant and β(2−)glicoprotein-I antibodies were positive. Immediately upon admission to the nephrology clinic, she received anticoagulation and corticosteroids along with therapeutic plasma exchanges. As a supportive treatment hemodialysis sessions were necessary. Under this treatment the amelioration of the general state was observed but renal failure persisted, therefore intravenous immunoglobulin was added. Afterwards, the kidney function recovered and the renal replacement therapy could be stopped. After this therapy, aPL became negative. Four weeks later lupus anticoagulant began to increase. Taking into account the risk factors of the relapse and the life-threatening course of the disease, rituximab was introduced. After administration of 2 g of RTX in three separate doses, we observed no new thrombotic events, the further amelioration of renal function and the negative profile of aPL. CONCLUSIONS: CAPS is a life-threatening condition and a prompt, complex treatment is necessary. Rituximab together with conventional therapy can be an additional option in case of the risk of relapse. BioMed Central 2018-06-08 /pmc/articles/PMC5994130/ /pubmed/29884138 http://dx.doi.org/10.1186/s12882-018-0928-z Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Case Report Rymarz, Aleksandra Niemczyk, Stanisław The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement |
title | The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement |
title_full | The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement |
title_fullStr | The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement |
title_full_unstemmed | The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement |
title_short | The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement |
title_sort | complex treatment including rituximab in the management of catastrophic antiphospholid syndrome with renal involvement |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5994130/ https://www.ncbi.nlm.nih.gov/pubmed/29884138 http://dx.doi.org/10.1186/s12882-018-0928-z |
work_keys_str_mv | AT rymarzaleksandra thecomplextreatmentincludingrituximabinthemanagementofcatastrophicantiphospholidsyndromewithrenalinvolvement AT niemczykstanisław thecomplextreatmentincludingrituximabinthemanagementofcatastrophicantiphospholidsyndromewithrenalinvolvement AT rymarzaleksandra complextreatmentincludingrituximabinthemanagementofcatastrophicantiphospholidsyndromewithrenalinvolvement AT niemczykstanisław complextreatmentincludingrituximabinthemanagementofcatastrophicantiphospholidsyndromewithrenalinvolvement |