| Autor: |
Keiki Shimada, Takaya Sasaki, Masahiro Okabe, Kentaro Koike, Daisuke Takahashi, Risa Terashima, Yu Honda, Naoto Matsumoto, Akira Fukui, Masahiro Ishikawa, Nobuo Tsuboi, Takashi Yokoo |
| Jazyk: |
angličtina |
| Rok vydání: |
2021 |
| Předmět: |
|
| Zdroj: |
Kidney Medicine, Vol 3, Iss 2, Pp 286-293 (2021) |
| Druh dokumentu: |
article |
| ISSN: |
2590-0595 |
| DOI: |
10.1016/j.xkme.2020.10.011 |
| Popis: |
TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal insufficiency, and organomegaly) syndrome is a systemic inflammatory disease sharing some features with Castleman disease and POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome in relation to abnormal secretions of interleukin 6 and vascular endothelial growth factor. The kidney is a main target organ of TAFRO syndrome but the kidney histopathology associated with TAFRO syndrome is yet to be completely defined. We report 3 TAFRO syndrome cases with different clinical courses in which kidney biopsies were performed. In all 3 cases, kidney biopsies showed similar glomerular lesions of diffuse global swelling of the endothelium and expansion of subendothelial spaces, consistent with severe glomerular endothelial injury. Case 3 showed an additional finding of focal tubulointerstitial injury characterized by marked plasma cell infiltration, which was absent in the other 2 cases. Clinical symptoms in cases 1 and 2, which had lower disease severity scores of TAFRO syndrome, were effectively treated with the administration of corticosteroids or a combination of corticosteroids and cyclosporine A. Case 3, with a higher disease severity score, had an aggressive clinical course that was refractory to corticosteroids and tocilizumab; the patient ultimately died of multiple organ failure. In all 3 cases, kidney biopsy provided indications for the diagnosis process and clinical management of TAFRO syndrome. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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