The association between adult-onset Still’s disease and collapsing glomerulopathy: a case report
Abstract 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 system...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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BMC
2022-10-01
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| Series: | Journal of Medical Case Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13256-022-03606-1 |
| _version_ | 1811182245339201536 |
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| author | Matas Orentas Nilam Patel Roger Rodby Sobia Hassan |
| author_facet | Matas Orentas Nilam Patel Roger Rodby Sobia Hassan |
| author_sort | Matas Orentas |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-04-11T09:29:21Z |
| format | Article |
| id | doaj.art-d0eacce44fb14c37824952a368b1f026 |
| institution | Directory Open Access Journal |
| issn | 1752-1947 |
| language | English |
| last_indexed | 2024-04-11T09:29:21Z |
| publishDate | 2022-10-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Medical Case Reports |
| spelling | doaj.art-d0eacce44fb14c37824952a368b1f0262022-12-22T04:31:56ZengBMCJournal of Medical Case Reports1752-19472022-10-011611810.1186/s13256-022-03606-1The association between adult-onset Still’s disease and collapsing glomerulopathy: a case reportMatas Orentas0Nilam Patel1Roger Rodby2Sobia Hassan3Internal Medicine Department, Rush University Medical CenterInternal Medicine Department, Rush University Medical CenterNephrology Department, Rush University Medical CenterRheumatology Department, Rush University Medical CenterAbstract 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.https://doi.org/10.1186/s13256-022-03606-1Adult-onset Still’s diseaseCollapsing glomerulopathyInterferons |
| spellingShingle | Matas Orentas Nilam Patel Roger Rodby Sobia Hassan The association between adult-onset Still’s disease and collapsing glomerulopathy: a case report Journal of Medical Case Reports Adult-onset Still’s disease Collapsing glomerulopathy Interferons |
| 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 | Adult-onset Still’s disease Collapsing glomerulopathy Interferons |
| url | https://doi.org/10.1186/s13256-022-03606-1 |
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