Renal involvement in autoinflammatory diseases
Background: Autoinflammatory diseases (AIDs) were first proposed 20 years ago and caused by dysregulation of the innate immune system leading to episodes of systemic inflammation. Advances in next-generation sequencing and biological technology have resulted in the identification of new monogenic di...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Karger Publishers
2023-02-01
|
| Series: | Kidney Diseases |
| Online Access: | https://www.karger.com/Article/FullText/529917 |
| _version_ | 1797862067383828480 |
|---|---|
| author | Changming Zhang Jiahui Peng Zhihong Liu Qing Zhou |
| author_facet | Changming Zhang Jiahui Peng Zhihong Liu Qing Zhou |
| author_sort | Changming Zhang |
| collection | DOAJ |
| description | Background: Autoinflammatory diseases (AIDs) were first proposed 20 years ago and caused by dysregulation of the innate immune system leading to episodes of systemic inflammation. Advances in next-generation sequencing and biological technology have resulted in the identification of new monogenic diseases and the corresponding signaling pathways that may guide us in targeted therapy. The kidney is a major target organ of various inflammatory process.
Summary: During systemic inflammation, increased proinflammatory cytokines, such as IL-6, IL-1β and TNF, lead to over transcription and release of acute phase reactant serum amyloid A (SAA). Sustained high SAA levels promote a cascade of pathophysiological events, including protein misfolding, protein fragmentation and aggregation into highly ordered amyloid fibrils. Amyloid fibrils deposition in kidney cause progressive glomerular and vascular damage. Renal AA amyloidosis is a common and severe complication of AIDs, including FMF, CAPS, TRAPS, MKD/HIDS, and DADA2. Amyloidosis may even be the first clinical manifestation in some patients, presenting with asymtomatic proteinuria, nephritic syndrome, progressive renal insufficiency or end-stage kidney disease. In addition, major dysregulated pathways in different AIDs lead to endogenous inflammation, which is due to direct endothelial cytotoxicity caused by IL-1β, type I interferon, or possibly immune complexes. The kidney is frequently affected by various vasculitis and renal involvement is a major determinant of treatment options and outcomes. The renal vasculitis involved in AIDs includes renal artery Takayasu vasculitis, polyarteritis nodosa, and IgA vasculitis. Moreover, other kidney diseases, such as glomerulonephritis, lupus nephritis, and renal tubular dysfunction were also reported in AIDs.
Key Messages: Kidney manifestations can be a coexisting disease seen with AIDs. They may also be one of the characteristics of AIDs. Clinicians should be aware of the possibility that amyloidosis, vasculitis or other renal diseases may be associated with AIDs in order to make appropriate diagnosis and treatment. Renal biopsy may be of great significance. Biologics, which switch off the underlying cytokine mediated inflammatory process, have the potential to restore organ damage and improve the outcome in the very early stage of the disease. |
| first_indexed | 2024-04-09T22:13:15Z |
| format | Article |
| id | doaj.art-cc54b7e1d2494af895499cc9cafd1b20 |
| institution | Directory Open Access Journal |
| issn | 2296-9381 2296-9357 |
| language | English |
| last_indexed | 2024-04-09T22:13:15Z |
| publishDate | 2023-02-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Kidney Diseases |
| spelling | doaj.art-cc54b7e1d2494af895499cc9cafd1b202023-03-23T06:54:58ZengKarger PublishersKidney Diseases2296-93812296-93572023-02-011110.1159/000529917529917Renal involvement in autoinflammatory diseasesChangming ZhangJiahui PengZhihong LiuQing ZhouBackground: Autoinflammatory diseases (AIDs) were first proposed 20 years ago and caused by dysregulation of the innate immune system leading to episodes of systemic inflammation. Advances in next-generation sequencing and biological technology have resulted in the identification of new monogenic diseases and the corresponding signaling pathways that may guide us in targeted therapy. The kidney is a major target organ of various inflammatory process. Summary: During systemic inflammation, increased proinflammatory cytokines, such as IL-6, IL-1β and TNF, lead to over transcription and release of acute phase reactant serum amyloid A (SAA). Sustained high SAA levels promote a cascade of pathophysiological events, including protein misfolding, protein fragmentation and aggregation into highly ordered amyloid fibrils. Amyloid fibrils deposition in kidney cause progressive glomerular and vascular damage. Renal AA amyloidosis is a common and severe complication of AIDs, including FMF, CAPS, TRAPS, MKD/HIDS, and DADA2. Amyloidosis may even be the first clinical manifestation in some patients, presenting with asymtomatic proteinuria, nephritic syndrome, progressive renal insufficiency or end-stage kidney disease. In addition, major dysregulated pathways in different AIDs lead to endogenous inflammation, which is due to direct endothelial cytotoxicity caused by IL-1β, type I interferon, or possibly immune complexes. The kidney is frequently affected by various vasculitis and renal involvement is a major determinant of treatment options and outcomes. The renal vasculitis involved in AIDs includes renal artery Takayasu vasculitis, polyarteritis nodosa, and IgA vasculitis. Moreover, other kidney diseases, such as glomerulonephritis, lupus nephritis, and renal tubular dysfunction were also reported in AIDs. Key Messages: Kidney manifestations can be a coexisting disease seen with AIDs. They may also be one of the characteristics of AIDs. Clinicians should be aware of the possibility that amyloidosis, vasculitis or other renal diseases may be associated with AIDs in order to make appropriate diagnosis and treatment. Renal biopsy may be of great significance. Biologics, which switch off the underlying cytokine mediated inflammatory process, have the potential to restore organ damage and improve the outcome in the very early stage of the disease.https://www.karger.com/Article/FullText/529917 |
| spellingShingle | Changming Zhang Jiahui Peng Zhihong Liu Qing Zhou Renal involvement in autoinflammatory diseases Kidney Diseases |
| title | Renal involvement in autoinflammatory diseases |
| title_full | Renal involvement in autoinflammatory diseases |
| title_fullStr | Renal involvement in autoinflammatory diseases |
| title_full_unstemmed | Renal involvement in autoinflammatory diseases |
| title_short | Renal involvement in autoinflammatory diseases |
| title_sort | renal involvement in autoinflammatory diseases |
| url | https://www.karger.com/Article/FullText/529917 |
| work_keys_str_mv | AT changmingzhang renalinvolvementinautoinflammatorydiseases AT jiahuipeng renalinvolvementinautoinflammatorydiseases AT zhihongliu renalinvolvementinautoinflammatorydiseases AT qingzhou renalinvolvementinautoinflammatorydiseases |