Glomerular lipidosis as a feature of renal-limited macrophage activation syndrome in a transplanted kidney: a case report
Abstract Background Glomerular lipidosis is a rare histological feature presenting the extensive glomerular accumulation of lipids with or without histiocytic infiltration, which develops under various conditions. Among its various etiologies, macrophage activation syndrome (MAS) is a condition repo...
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| Language: | English |
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BMC
2023-11-01
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| Series: | BMC Nephrology |
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| Online Access: | https://doi.org/10.1186/s12882-023-03380-2 |
| _version_ | 1827764616425373696 |
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| author | Kentaro Sugisaki Takahiro Uchida Sachiko Iwama Masaaki Okihara Isao Akashi Yu Kihara Osamu Konno Masayuki Kuroda Junki Koike Hitoshi Iwamoto Takashi Oda |
| author_facet | Kentaro Sugisaki Takahiro Uchida Sachiko Iwama Masaaki Okihara Isao Akashi Yu Kihara Osamu Konno Masayuki Kuroda Junki Koike Hitoshi Iwamoto Takashi Oda |
| author_sort | Kentaro Sugisaki |
| collection | DOAJ |
| description | Abstract Background Glomerular lipidosis is a rare histological feature presenting the extensive glomerular accumulation of lipids with or without histiocytic infiltration, which develops under various conditions. Among its various etiologies, macrophage activation syndrome (MAS) is a condition reported to be associated with histiocytic glomerular lipidosis. Here we describe the first case of glomerular lipidosis observed in a renal allograft that histologically mimicked histiocytic glomerulopathy owing to MAS. Case presentation A 42-year-old man underwent successful living-donor kidney transplantation. However, middle-grade proteinuria and increased serum triglyceride levels indicative of type V hyperlipidemia developed rapidly thereafter. An allograft biopsy performed 6 months after the transplantation showed extensive glomerular infiltration of CD68+ foam cells (histiocytes) intermingled with many CD3+ T-cells (predominantly CD8+ cells). Furthermore, frequent contact between glomerular T-cells and histiocytes, and the existence of activated CD8+ cells (CD8+, HLA-DR+ cells) were observed by double immunostaining. There was no clinicopathological data suggesting lipoprotein glomerulopathy or lecithin cholesterol acyltransferase deficiency, both of which are well-known causes of glomerular lipidosis. The histological findings were relatively similar to those of histiocytic glomerulopathy caused by MAS. As systemic manifestations of MAS, such as fever, pancytopenia, coagulation abnormalities, hyperferritinemia, increased liver enzyme levels, hepatosplenomegaly, and lymphadenopathy were minimal, this patient was clinicopathologically diagnosed as having renal-limited MAS. Although optimal treatment strategies for MAS in kidney transplant patients remains unclear, we strengthened lipid-lowering therapy using pemafibrate, without modifying the amount of immunosuppressants. Serum triglyceride levels were normalized with this treatment; however, the patient’s extensive proteinuria and renal dysfunction did not improve. Biopsy analysis at 1 year after the transplantation demonstrated the disappearance of glomerular foamy changes, but the number of glomerular infiltrating cells remained similar. Conclusion To our knowledge, this is the first reported case of glomerular lipidosis in a transplanted kidney. Increased interaction-activation of histiocytes (macrophages) and CD8+ T-cells, the key pathogenic feature of MAS, was observed in the glomeruli of this patient, who did not demonstrate overt systemic manifestations, suggesting a pathological condition of renal-limited MAS. The clinical effects of triglyceride-lowering therapy were limited, suggesting that hypertriglyceridemia was not the cause of but rather may be a consequence of renal-limited MAS. |
| first_indexed | 2024-03-11T11:07:58Z |
| format | Article |
| id | doaj.art-ec446790834049389f9c94fb8c0b15a9 |
| institution | Directory Open Access Journal |
| issn | 1471-2369 |
| language | English |
| last_indexed | 2024-03-11T11:07:58Z |
| publishDate | 2023-11-01 |
| publisher | BMC |
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| series | BMC Nephrology |
| spelling | doaj.art-ec446790834049389f9c94fb8c0b15a92023-11-12T12:09:43ZengBMCBMC Nephrology1471-23692023-11-012411910.1186/s12882-023-03380-2Glomerular lipidosis as a feature of renal-limited macrophage activation syndrome in a transplanted kidney: a case reportKentaro Sugisaki0Takahiro Uchida1Sachiko Iwama2Masaaki Okihara3Isao Akashi4Yu Kihara5Osamu Konno6Masayuki Kuroda7Junki Koike8Hitoshi Iwamoto9Takashi Oda10Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, 1163 TatemachiDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, 1163 TatemachiDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, 1163 TatemachiDepartment of Kidney Transplantation Surgery, Kidney Disease Center, Tokyo Medical University Hachioji Medical CenterDepartment of Kidney Transplantation Surgery, Kidney Disease Center, Tokyo Medical University Hachioji Medical CenterDepartment of Kidney Transplantation Surgery, Kidney Disease Center, Tokyo Medical University Hachioji Medical CenterDepartment of Kidney Transplantation Surgery, Kidney Disease Center, Tokyo Medical University Hachioji Medical CenterCenter for Advanced Medicine, Chiba UniversityDepartment of Pathology, St. Marianna University School of MedicineDepartment of Kidney Transplantation Surgery, Kidney Disease Center, Tokyo Medical University Hachioji Medical CenterDepartment of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, 1163 TatemachiAbstract Background Glomerular lipidosis is a rare histological feature presenting the extensive glomerular accumulation of lipids with or without histiocytic infiltration, which develops under various conditions. Among its various etiologies, macrophage activation syndrome (MAS) is a condition reported to be associated with histiocytic glomerular lipidosis. Here we describe the first case of glomerular lipidosis observed in a renal allograft that histologically mimicked histiocytic glomerulopathy owing to MAS. Case presentation A 42-year-old man underwent successful living-donor kidney transplantation. However, middle-grade proteinuria and increased serum triglyceride levels indicative of type V hyperlipidemia developed rapidly thereafter. An allograft biopsy performed 6 months after the transplantation showed extensive glomerular infiltration of CD68+ foam cells (histiocytes) intermingled with many CD3+ T-cells (predominantly CD8+ cells). Furthermore, frequent contact between glomerular T-cells and histiocytes, and the existence of activated CD8+ cells (CD8+, HLA-DR+ cells) were observed by double immunostaining. There was no clinicopathological data suggesting lipoprotein glomerulopathy or lecithin cholesterol acyltransferase deficiency, both of which are well-known causes of glomerular lipidosis. The histological findings were relatively similar to those of histiocytic glomerulopathy caused by MAS. As systemic manifestations of MAS, such as fever, pancytopenia, coagulation abnormalities, hyperferritinemia, increased liver enzyme levels, hepatosplenomegaly, and lymphadenopathy were minimal, this patient was clinicopathologically diagnosed as having renal-limited MAS. Although optimal treatment strategies for MAS in kidney transplant patients remains unclear, we strengthened lipid-lowering therapy using pemafibrate, without modifying the amount of immunosuppressants. Serum triglyceride levels were normalized with this treatment; however, the patient’s extensive proteinuria and renal dysfunction did not improve. Biopsy analysis at 1 year after the transplantation demonstrated the disappearance of glomerular foamy changes, but the number of glomerular infiltrating cells remained similar. Conclusion To our knowledge, this is the first reported case of glomerular lipidosis in a transplanted kidney. Increased interaction-activation of histiocytes (macrophages) and CD8+ T-cells, the key pathogenic feature of MAS, was observed in the glomeruli of this patient, who did not demonstrate overt systemic manifestations, suggesting a pathological condition of renal-limited MAS. The clinical effects of triglyceride-lowering therapy were limited, suggesting that hypertriglyceridemia was not the cause of but rather may be a consequence of renal-limited MAS.https://doi.org/10.1186/s12882-023-03380-2CD68CD8Glomerular lipidosisMacrophage activation syndrome (MAS)HistiocyteKidney transplant |
| spellingShingle | Kentaro Sugisaki Takahiro Uchida Sachiko Iwama Masaaki Okihara Isao Akashi Yu Kihara Osamu Konno Masayuki Kuroda Junki Koike Hitoshi Iwamoto Takashi Oda Glomerular lipidosis as a feature of renal-limited macrophage activation syndrome in a transplanted kidney: a case report BMC Nephrology CD68 CD8 Glomerular lipidosis Macrophage activation syndrome (MAS) Histiocyte Kidney transplant |
| title | Glomerular lipidosis as a feature of renal-limited macrophage activation syndrome in a transplanted kidney: a case report |
| title_full | Glomerular lipidosis as a feature of renal-limited macrophage activation syndrome in a transplanted kidney: a case report |
| title_fullStr | Glomerular lipidosis as a feature of renal-limited macrophage activation syndrome in a transplanted kidney: a case report |
| title_full_unstemmed | Glomerular lipidosis as a feature of renal-limited macrophage activation syndrome in a transplanted kidney: a case report |
| title_short | Glomerular lipidosis as a feature of renal-limited macrophage activation syndrome in a transplanted kidney: a case report |
| title_sort | glomerular lipidosis as a feature of renal limited macrophage activation syndrome in a transplanted kidney a case report |
| topic | CD68 CD8 Glomerular lipidosis Macrophage activation syndrome (MAS) Histiocyte Kidney transplant |
| url | https://doi.org/10.1186/s12882-023-03380-2 |
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