Association of karyomegalic interstitial nephritis with focal segmental glomerulosclerosis

Karyomegalic interstitial nephritis (KIN), first described in 1974, is a rare form of chronic tubulointerstitial nephritis. It is defined by the presence of markedly enlarged, hyperchromatic nuclei with prominent nucleoli, mainly involving tubular epithelial cells of the kidney, accompanied by mark...

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Autores principales:	Momal Tara Chand, Awais Zaka, Hong Qu
Formato:	article
Lenguaje:	EN
Publicado:	University of São Paulo 2021
Materias:	Glomerulosclerosis Focal Segmental Kidney Nephritis Interstitial Medicine R Internal medicine RC31-1245
Acceso en línea:	https://doaj.org/article/85f4091531ad40989ad4421f87687677
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spelling	oai:doaj.org-article:85f4091531ad40989ad4421f876876772021-11-24T04:10:40ZAssociation of karyomegalic interstitial nephritis with focal segmental glomerulosclerosis2236-1960https://doaj.org/article/85f4091531ad40989ad4421f876876772021-11-01T00:00:00Zhttps://www.revistas.usp.br/autopsy/article/view/192807https://doaj.org/toc/2236-1960 Karyomegalic interstitial nephritis (KIN), first described in 1974, is a rare form of chronic tubulointerstitial nephritis. It is defined by the presence of markedly enlarged, hyperchromatic nuclei with prominent nucleoli, mainly involving tubular epithelial cells of the kidney, accompanied by marked interstitial fibrosis. The disease presents as asymptomatic proteinuria, gradually progresses to chronic kidney disease and eventually leads to end-stage renal disease by 30-40 years. The etiology of the disease remains unclear; however, genetic risk factors and possible association with HLA (B27/35) is proposed by some. It has also been linked to FAN1 (FANCD2/FANC1- associated nuclease 1) mutation. Case Report: We present two cases of KIN with associated focal segmental glomerulosclerosis. Both patients presented with nephrotic range proteinuria. The biopsies demonstrated marked enlargement of tubular nuclei (3-5x larger than the uninvolved tubular nuclei, a metric used by some authors in previous studies) in some tubules, meeting the diagnostic criteria of KIN.. Interestingly, case one had a prior biopsy that showed minimal change disease. In the biopsies done at our institution, H&E sections showed patchy tubular attenuation with readily recognizable tubular cell mitotic figures, indicating concurrent acute tubular injury. Electron microscopy showed diffuse podocyte foot process effacement, along with microvillous transformation, podocyte hypertrophy, and cytoplasmic vacuoles, suggesting podocyte injury. This cytoplasmic vacuolization was also observed in the tubular epithelial cells. In both cases, the injury factor appeared to target both podocytes and tubular cells. Momal Tara ChandAwais Zaka Hong QuUniversity of São PauloarticleGlomerulosclerosisFocal SegmentalKidneyNephritisInterstitialMedicineRInternal medicineRC31-1245ENAutopsy and Case Reports, Vol 11 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Glomerulosclerosis Focal Segmental Kidney Nephritis Interstitial Medicine R Internal medicine RC31-1245
spellingShingle	Glomerulosclerosis Focal Segmental Kidney Nephritis Interstitial Medicine R Internal medicine RC31-1245 Momal Tara Chand Awais Zaka Hong Qu Association of karyomegalic interstitial nephritis with focal segmental glomerulosclerosis
description	Karyomegalic interstitial nephritis (KIN), first described in 1974, is a rare form of chronic tubulointerstitial nephritis. It is defined by the presence of markedly enlarged, hyperchromatic nuclei with prominent nucleoli, mainly involving tubular epithelial cells of the kidney, accompanied by marked interstitial fibrosis. The disease presents as asymptomatic proteinuria, gradually progresses to chronic kidney disease and eventually leads to end-stage renal disease by 30-40 years. The etiology of the disease remains unclear; however, genetic risk factors and possible association with HLA (B27/35) is proposed by some. It has also been linked to FAN1 (FANCD2/FANC1- associated nuclease 1) mutation. Case Report: We present two cases of KIN with associated focal segmental glomerulosclerosis. Both patients presented with nephrotic range proteinuria. The biopsies demonstrated marked enlargement of tubular nuclei (3-5x larger than the uninvolved tubular nuclei, a metric used by some authors in previous studies) in some tubules, meeting the diagnostic criteria of KIN.. Interestingly, case one had a prior biopsy that showed minimal change disease. In the biopsies done at our institution, H&E sections showed patchy tubular attenuation with readily recognizable tubular cell mitotic figures, indicating concurrent acute tubular injury. Electron microscopy showed diffuse podocyte foot process effacement, along with microvillous transformation, podocyte hypertrophy, and cytoplasmic vacuoles, suggesting podocyte injury. This cytoplasmic vacuolization was also observed in the tubular epithelial cells. In both cases, the injury factor appeared to target both podocytes and tubular cells.
format	article
author	Momal Tara Chand Awais Zaka Hong Qu
author_facet	Momal Tara Chand Awais Zaka Hong Qu
author_sort	Momal Tara Chand
title	Association of karyomegalic interstitial nephritis with focal segmental glomerulosclerosis
title_short	Association of karyomegalic interstitial nephritis with focal segmental glomerulosclerosis
title_full	Association of karyomegalic interstitial nephritis with focal segmental glomerulosclerosis
title_fullStr	Association of karyomegalic interstitial nephritis with focal segmental glomerulosclerosis
title_full_unstemmed	Association of karyomegalic interstitial nephritis with focal segmental glomerulosclerosis
title_sort	association of karyomegalic interstitial nephritis with focal segmental glomerulosclerosis
publisher	University of São Paulo
publishDate	2021
url	https://doaj.org/article/85f4091531ad40989ad4421f87687677
work_keys_str_mv	AT momaltarachand associationofkaryomegalicinterstitialnephritiswithfocalsegmentalglomerulosclerosis AT awaiszaka associationofkaryomegalicinterstitialnephritiswithfocalsegmentalglomerulosclerosis AT hongqu associationofkaryomegalicinterstitialnephritiswithfocalsegmentalglomerulosclerosis
_version_	1718416044895240192

Association of karyomegalic interstitial nephritis with focal segmental glomerulosclerosis

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