Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumab
Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypic...
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| Format: | Article |
| Language: | English |
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MDPI AG
2016-09-01
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| Series: | Hematology Reports |
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| Online Access: | http://www.pagepress.org/journals/index.php/hr/article/view/6625 |
| _version_ | 1797936210006507520 |
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| author | Alexander G. Raufi Shruti Scott Omar Darwish Kevin Harley Kanwarpal Kahlon Sheetal Desai Yuxin Lu Minh-Ha Tran |
| author_facet | Alexander G. Raufi Shruti Scott Omar Darwish Kevin Harley Kanwarpal Kahlon Sheetal Desai Yuxin Lu Minh-Ha Tran |
| author_sort | Alexander G. Raufi |
| collection | DOAJ |
| description | Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) – a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage. Although plasma exchange is considered first-line therapy for thrombotic thrombocytopenic purpura – a TMA classically associated with autoimmune depletion of ADAMTS-13 – aHUS demonstrates less reliable responsiveness to this modality. Instead, use of the late complement inhibitor Eculizumab has emerged as an effective modality for the management of such patients. Diagnosis of aHUS, however, is largely clinically based, relying heavily upon a multidisciplinary approach. Herein we present the case of a patient with atypical HUS successfully treated with Eculizumab in the setting of Class IV-G (A) lupus nephritis and hypocomplementemia. |
| first_indexed | 2024-04-10T18:26:07Z |
| format | Article |
| id | doaj.art-ee85553235b841069caab39fab409dc9 |
| institution | Directory Open Access Journal |
| issn | 2038-8322 2038-8330 |
| language | English |
| last_indexed | 2024-04-10T18:26:07Z |
| publishDate | 2016-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Hematology Reports |
| spelling | doaj.art-ee85553235b841069caab39fab409dc92023-02-02T05:48:42ZengMDPI AGHematology Reports2038-83222038-83302016-09-018310.4081/hr.2016.66253524Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumabAlexander G. Raufi0Shruti Scott1Omar Darwish2Kevin Harley3Kanwarpal Kahlon4Sheetal Desai5Yuxin Lu6Minh-Ha Tran7Department of Internal Medicine, University of California, Irvine, CADepartment of Internal Medicine, University of California, Irvine, CADepartment of Internal Medicine, University of California, Irvine, CADepartment of Internal Medicine, University of California, Irvine, CADepartment of Internal Medicine, University of California, Irvine, CADepartment of Internal Medicine, University of California, Irvine, CADepartment of Pathology and Laboratory Medicine, University of California, Irvine, CADepartment of Pathology and Laboratory Medicine, University of California, Irvine, CAAmong the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) – a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage. Although plasma exchange is considered first-line therapy for thrombotic thrombocytopenic purpura – a TMA classically associated with autoimmune depletion of ADAMTS-13 – aHUS demonstrates less reliable responsiveness to this modality. Instead, use of the late complement inhibitor Eculizumab has emerged as an effective modality for the management of such patients. Diagnosis of aHUS, however, is largely clinically based, relying heavily upon a multidisciplinary approach. Herein we present the case of a patient with atypical HUS successfully treated with Eculizumab in the setting of Class IV-G (A) lupus nephritis and hypocomplementemia.http://www.pagepress.org/journals/index.php/hr/article/view/6625Atypical hemolytic uremic syndromeMicroangiopathic hemolytic anemiaThrombotic microangiopathyLupus nephritis |
| spellingShingle | Alexander G. Raufi Shruti Scott Omar Darwish Kevin Harley Kanwarpal Kahlon Sheetal Desai Yuxin Lu Minh-Ha Tran Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumab Hematology Reports Atypical hemolytic uremic syndrome Microangiopathic hemolytic anemia Thrombotic microangiopathy Lupus nephritis |
| title | Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumab |
| title_full | Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumab |
| title_fullStr | Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumab |
| title_full_unstemmed | Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumab |
| title_short | Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumab |
| title_sort | atypical hemolytic uremic syndrome secondary to lupus nephritis responsive to eculizumab |
| topic | Atypical hemolytic uremic syndrome Microangiopathic hemolytic anemia Thrombotic microangiopathy Lupus nephritis |
| url | http://www.pagepress.org/journals/index.php/hr/article/view/6625 |
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