Progressive idiopathic nodular glomerulosclerosis mimicking diabetic nephropathy without abnormal glycemic metabolism

Nephrology ◽  
2016 ◽  
Vol 21 (12) ◽  
pp. 1074-1075
Author(s):  
Atsushi Tanaka ◽  
Tsukasa Nakamura ◽  
Eiichi Sato ◽  
Yoshihiko Ueda ◽  
Koichi Node
2020 ◽  
Vol 8 ◽  
pp. 2050313X2094051
Author(s):  
Ramy Hanna ◽  
Jonathan E Zuckerman ◽  
Antoney Ferrey ◽  
Everado Arias Torres ◽  
Sam Tonthat ◽  
...  

Idiopathic nodular glomerulosclerosis is an unusual histopathological finding that has commonly been observed in male smokers with hypertension. It has remained an enigmatic condition and is best described as a diabetic pattern of glomerular injury seen in non-diabetic patients. It is also one of the few nicotine (smoking)-associated/smoking-associated patterns of renal injury. We present an even more unusual manifestation of this pathological finding in a 59-year-old Hispanic female who presented with chronic kidney disease approaching need for renal replacement therapy. The patient had idiopathic nodular glomerulosclerosis on kidney biopsy, despite no prior history of diabetes, nor smoking history, including no secondhand smoking exposure. The patient did have hypertension. The renal biopsy also showed evidence of chronic thrombotic-microangiopathic changes within arteries and arterioles. Genetic testing of the alternative pathway revealed an unusual and likely pathological variant of thrombomodulin supporting complement dysfunction as having a role in the presentation.


2002 ◽  
Vol 33 (8) ◽  
pp. 826-835 ◽  
Author(s):  
Glen S. Markowitz ◽  
Julie Lin ◽  
Anthony M. Valeri ◽  
Cecilia Avila ◽  
Samih H. Nasr ◽  
...  

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Aurélie Sannier ◽  
Valentin Maisons ◽  
Mickael Bobot ◽  
Francois Vrtovsnik ◽  
Noemie Jourde-Chiche ◽  
...  

Abstract Background and Aims Kidney Biopsies (KB) performed in patients with Type-2 diabetes (T2D) usually aim at differentiating diabetic nephropathy (DN) from other kidney diseases. However, KB could also help refining patients’ prognosis, both in terms of renal survival, and in terms of patient survival. In 2010, the Renal Pathology Society developed a pathological classification of DN, but the prognostic value of the described items , is still imperfectly documented. We aimed to assess the prognostic performances of these items to predict renal and patient survival. Method Native KBs with diabetic and/or hypertensive nephropathy (DN/HN) performed in patients with T2D in four French centers were analyzed and scored according to the classification developed by the Renal Pathology Society. Clinical and biological data was collected from the patients’ records. Survival analyses were performed for renal survival (time to first dialysis or preemptive transplantation) and death after dichotomization of continuous data). For each of the analyses, we first established a model comprising clinical data only. We then assessed the benefit of adding each of the pathological item to the clinical model. Finally, we performed a backward stepwise analysis to identify items predictive of renal and/or patient survival. Results We analyzed 165 biopsies with DN/HN from patients with T2D and with at least 12 months of follow-up (unless they reached an endpoint during the first year). Among them, 73 (44%) were male, 155 (94%) had hypertension, 53 (34%) hematuria, 22 (15%) had proliferative diabetic retinopathy (DR), 33 (23%) had non-proliferative DR, 90 (62%) had no DR (20 had missing data). Mean (SD) age was 63 (11), median [IQR] eGFRCKD-EPI was 29 [18;45] ml/min/1.73m², urinary protein-to-creatinine ratio was 0.38 [0.14;0.83] g/mmol, HbA1c was 7 [6.2;8.2] % and diabetes duration before KB was 10 [5;19] years. The median [IQR] follow-up was 33 months[18;57]. During the follow-up, 43 (26%) patients died and 69 (42%) required renal replacement therapy (RRT). The percentage of ischemic glomeruli, and presence of more than one area of arteriolar hyalinosis (ah=2), were predictive of renal survival and improved the predictive value of the model when added to clinical parameters. Presence of at least one convincing Kimmelstiel–Wilson lesion (nodular glomerulosclerosis or Class III DN) was predictive of death and similarly improved the predictive model (See figure). Conclusion Pathological findings on KB, as classified by the Renal Pathology Society, carry significant prognostic value in patients with T2D and DN/HN. Vascular lesions (presence of arteriolar hyalinosis and less than 7% of ischemic glomeruli) predicted the need for RRT, while nodular glomerulosclerosis was predictive of death. 


2017 ◽  
Vol 7 (1) ◽  
pp. 48-54 ◽  
Author(s):  
Nao Nakamura ◽  
Kensei Taguchi ◽  
Yoshihiro Miyazono ◽  
Keiichiro Uemura ◽  
Kiyomi Koike ◽  
...  

2013 ◽  
Vol 137 (3) ◽  
pp. 351-359 ◽  
Author(s):  
Kirtee Raparia ◽  
Irtaza Usman ◽  
Yashpal S. Kanwar

Context.—Nodular, intercapillary glomerulosclerotic lesions resembling Kimmelstiel-Wilson nodules commonly observed in diabetic nephropathy can also be seen in patients without any clinical history or evidence of diabetes. Objectives.—To discuss the pathobiology of lesions reminiscent of diabetes nephropathy, including light-chain deposition disease, amyloidosis, immunotactoid nephropathy, the membranoproliferative form of glomerulonephritis, and idiopathic nodular glomerulosclerosis, and how to differentiate them from diabetic nephropathy. Data Sources.—Published literature and authors' personal experience. Conclusions.—The well-formed, intercapillary, nodular mesangial lesions, along with thickened glomerular basement membranes and tubular basement membranes, and hyaline arteriolosclerosis are virtually pathognomic of diabetic nephropathy. However, the pathologist must exclude lesions reminiscent of diabetic nephropathy by performing special stains on histologic sections, immunofluorescence, and electron microscopic studies.


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