Karyomegalic Interstitial Nephritis-A Rare Cause Of Chronic Tubulointerstitial Nephritis

Karyomegalic Interstitial Nephritis (KIN) is a rare disease, which usually presents with slowly progressive chronic kidney disease, eventually leading to end stage renal disease in early adulthood. Histological findings consist of enlarged and hyperchromatic nuclei in scattered tubular epithelial cells throughout the nephron accompanied by interstitial fibrosis around atrophic tubules.

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MO282EARLY RENAL RECOVERY AFTER A FIRST FLARE OF PAUCI IMMUNE GLOMERULONEPHRITIS

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfab104.0040 ◽

2021 ◽

Vol 36 (Supplement_1) ◽

Author(s):

Jeremy Zaworski ◽

Cyrille Vandenbussche ◽

Pierre Bataille ◽

Eric Hachulla ◽

Francois Glowacki ◽

...

Keyword(s):

Renal Disease ◽

End Stage Renal Disease ◽

Interstitial Fibrosis ◽

Univariate Analysis ◽

Renal Recovery ◽

Neurological Involvement ◽

Factors Associated ◽

Stage Renal Disease ◽

End Stage

Abstract Background and Aims Renal involvement is a severe manifestation of ANCA-associated vasculitis. Patients often progress to end-stage renal disease. The potential for renal recovery after a first flare has seldom been studied. Our objectives were to describe the evolution of the estimated glomerular filtration rate (eGFR) and identify factors associated with the change in eGFR between diagnosis and follow-up at 3 months (ΔeGFRM0–M3) in a cohort of patients with a first flare of pauci-immune glomerulonephritis. Methods This was a retrospective study over the period 2003–2018 of incident patients in the Nord-Pas-de-Calais (France). Patients were recruited if they had a first histologically-proven flare of pauci immune glomerulonephritis with at least 1 year of follow up. Kidney function was estimated with MDRD-equation and analysed at diagnosis, 3rd, 6th and 12th months. The primary outcome was ΔeGFRM0–M3. Factors evaluated were histological (Berden classification, interstitial fibrosis, percentage of crescents), clinical (extra-renal manifestations, sex, age) or biological (severity of acute kidney injury, dialysis, ANCA subtype). Results One hundred and seventy-seven patients were included. The eGFR at 3 months was significantly higher than at diagnosis (mean ± standard deviation, 40 ± 24 vs 28 ± 26 ml/min/1.73 m2, p < 0.001), with a ΔeGFRM0–M3 of 12 ± 19 ml/min/1.73 m2. The eGFR at 12 months was higher than at 3 months (44 ± 13 vs 40 ± 24 ml/min/1.73m2, p = 0.003). The factors significantly associated with ΔeGFRM0–M3 in univariate analysis were: sclerotic class according to Berden classification, percentage of interstitial fibrosis, percentage of cellular crescents, acute tubular necrosis, neurological involvement. The factors associated with ΔeGFRM0–M3 in multivariate analysis were the percentage of cellular crescents and neurological involvement. The mean increase in eGFR was 2.90 ± 0.06 ml/min/1.73m2 for every 10-point gain in the percentage of cellular crescents. ΔeGFRM0–M3 was not associated with the risks of end-stage renal disease or death in long-term follow-up. Conclusions Early renal recovery after a first flare of pauci-immune glomerulonephritis occurred mainly in the first three months of treatment. The percentage of cellular crescents was the main independent predictor of early renal recovery.

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Renal function and structure in a rat model of arterial calcification and increased pulse pressure

AJP Renal Physiology ◽

10.1152/ajprenal.00081.2008 ◽

2008 ◽

Vol 295 (4) ◽

pp. F1222-F1229 ◽

Cited By ~ 4

Author(s):

Virginie Gaillard ◽

Bernard Jover ◽

Daniel Casellas ◽

Magali Cordaillat ◽

Jeffrey Atkinson ◽

...

Keyword(s):

Glomerular Filtration Rate ◽

Renal Disease ◽

Pulse Pressure ◽

End Stage Renal Disease ◽

Filtration Rate ◽

Interstitial Fibrosis ◽

Calcium Content ◽

Tissue Calcification ◽

Stage Renal Disease ◽

End Stage

Clinical studies suggest a strong link between tissue calcification and pressure hyperpulsatility in end stage renal disease patients. Using a Wistar rat model of arterial elastocalcinosis and hyperpulsatility [vitamin D and nicotine (VDN) treatment], we evaluated the relative importance of tissue calcification and hyperpulsatility in the etiology of renal failure. VDN rats showed significant increases in aortic wall calcium content (50 times; 992 ± 171 vs. control 19 ± 1 μmol/g dry wt) and pulse pressure (1.5 times; 61 ± 4 vs. control 40 ± 2 mmHg). Significant renal calcification (16 times; 124 ± 27 vs. control 8.1 ± 0.7 μmol/g dry wt) occurred mainly within the media of the preglomerular vasculature and in the areas of interstitial fibrosis in VDN. Extensive renal damages (5 times; 26 ± 5% of collapsed-atrophic or sclerotic glomeruli, or glomerular cysts vs. control 5.2 ± 0.3%; 28 times; 61 ± 12% areas of focal, cortical areas exhibiting interstitial fibrosis per section vs. control 2.2 ± 0.6%) were observed histologically. The glomerular filtration rate significantly decreased (880 ± 40 vs. control 1,058 ± 44 μl·min−1·g kidney wt−1). Albuminuria increased six times (1.6 ± 0.4 vs. control 0.27 ± 0.04 mg/24 h). There were significant linear relationships between albuminuria and pulse pressure ( r2 = 0.408; n = 24) or renal calcium content ( r2 = 0.328; n = 24; P < 0.05) and between glomerular filtration rate and pulse pressure ( r2 = 0.168; n = 27). To our knowledge, this study provides the first evidence of links between both 1) hyperpulsatility and renal dysfunction, and 2) renal calcification and renal dysfunction. Given the increasing frequency of end-stage renal disease, this model could prove useful for preclinical evaluation of drugs that prevent or attenuate hyperpulsatility and/or tissue calcification.

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