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The association between adult-onset Still’s disease and collapsing glomerulopathy: a case report
BACKGROUND: Collapsing glomerulopathy, characterized by marked hypertrophy and hyperplasia of the podocytes with eventual collapse of the glomerular tuft, is an important cause of end-stage renal disease. Among the many causes of collapsing glomerulopathy, autoimmune diseases, such as systemic lupus...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9560741/ https://www.ncbi.nlm.nih.gov/pubmed/36229843 http://dx.doi.org/10.1186/s13256-022-03606-1 |
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author | Orentas, Matas Patel, Nilam Rodby, Roger Hassan, Sobia |
author_facet | Orentas, Matas Patel, Nilam Rodby, Roger Hassan, Sobia |
author_sort | Orentas, Matas |
collection | PubMed |
description | BACKGROUND: Collapsing glomerulopathy, characterized by marked hypertrophy and hyperplasia of the podocytes with eventual collapse of the glomerular tuft, is an important cause of end-stage renal disease. Among the many causes of collapsing glomerulopathy, autoimmune diseases, such as systemic lupus erythematosus, have been implicated. There are also rare reports of adult-onset Still’s disease, an autoinflammatory condition characterized by fever, rash, and inflammatory arthritis being associated with collapsing glomerulopathy. CASE PRESENTATION: Herein, we present a review of three published cases, and present a new case of a 15-year-old African American female patient with collapsing glomerulopathy who was diagnosed with adult-onset Still’s disease 12 years later when she presented with fevers, arthralgias, sore throat, lymphadenopathy, hepatocellular injury, and elevated serum ferritin. Her collapsing glomerulopathy was initially well controlled following induction therapy with cyclosporine and prednisone and maintenance therapy with losartan. However, after developing adult-onset Still’s disease, she had multiple flare-ups despite various immunosuppressive therapies and developed worsening renal function, eventually progressing to end-stage renal disease. CONCLUSIONS: Our case-based review highlights a rare but important association between adult-onset Still’s disease and collapsing glomerulopathy, and postulates a possible pathophysiological link. |
format | Online Article Text |
id | pubmed-9560741 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-95607412022-10-14 The association between adult-onset Still’s disease and collapsing glomerulopathy: a case report Orentas, Matas Patel, Nilam Rodby, Roger Hassan, Sobia J Med Case Rep Case Report BACKGROUND: Collapsing glomerulopathy, characterized by marked hypertrophy and hyperplasia of the podocytes with eventual collapse of the glomerular tuft, is an important cause of end-stage renal disease. Among the many causes of collapsing glomerulopathy, autoimmune diseases, such as systemic lupus erythematosus, have been implicated. There are also rare reports of adult-onset Still’s disease, an autoinflammatory condition characterized by fever, rash, and inflammatory arthritis being associated with collapsing glomerulopathy. CASE PRESENTATION: Herein, we present a review of three published cases, and present a new case of a 15-year-old African American female patient with collapsing glomerulopathy who was diagnosed with adult-onset Still’s disease 12 years later when she presented with fevers, arthralgias, sore throat, lymphadenopathy, hepatocellular injury, and elevated serum ferritin. Her collapsing glomerulopathy was initially well controlled following induction therapy with cyclosporine and prednisone and maintenance therapy with losartan. However, after developing adult-onset Still’s disease, she had multiple flare-ups despite various immunosuppressive therapies and developed worsening renal function, eventually progressing to end-stage renal disease. CONCLUSIONS: Our case-based review highlights a rare but important association between adult-onset Still’s disease and collapsing glomerulopathy, and postulates a possible pathophysiological link. BioMed Central 2022-10-14 /pmc/articles/PMC9560741/ /pubmed/36229843 http://dx.doi.org/10.1186/s13256-022-03606-1 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report Orentas, Matas Patel, Nilam Rodby, Roger Hassan, Sobia The association between adult-onset Still’s disease and collapsing glomerulopathy: a case report |
title | The association between adult-onset Still’s disease and collapsing glomerulopathy: a case report |
title_full | The association between adult-onset Still’s disease and collapsing glomerulopathy: a case report |
title_fullStr | The association between adult-onset Still’s disease and collapsing glomerulopathy: a case report |
title_full_unstemmed | The association between adult-onset Still’s disease and collapsing glomerulopathy: a case report |
title_short | The association between adult-onset Still’s disease and collapsing glomerulopathy: a case report |
title_sort | association between adult-onset still’s disease and collapsing glomerulopathy: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9560741/ https://www.ncbi.nlm.nih.gov/pubmed/36229843 http://dx.doi.org/10.1186/s13256-022-03606-1 |
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