Idiopathic nodular glomerulosclerosis: Report of two cases and review of literature

Chandragiri, S; Raju, Sree Bhushan; Mukku, K K; Babu, Sashidhar; Uppin,

doi:10.4103/0971-4065.164233

Cited by 3 publications

(1 citation statement)

References 8 publications

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“…The finding of a TMA and a thrombomodulin mutation in this report may be a fortuitous discovery that sheds light on a possible connection between the alternative pathway of complement and ING. 1 – 10 , 15 – 27 The connections of ING to hyperlipidemia, obesity, and atherosclerosis are also interesting since they all represent inflammatory states, and this could be suggestive of a role for complement as well. 2 Studies of the coagulation cascade may be useful in ING and TMA presentations given that the coagulation cascade and the complement system interact.…”

Section: Discussionmentioning

confidence: 99%

Finding of pathological thrombomodulin gene variant in a patient with idiopathic nodular glomerulosclerosis and chronic thrombotic microangiopathy-like changes

Hanna

Zuckerman

Ferrey

et al. 2020

SAGE Open Medical Case Reports

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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.

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Section: Discussionmentioning

confidence: 99%

Finding of pathological thrombomodulin gene variant in a patient with idiopathic nodular glomerulosclerosis and chronic thrombotic microangiopathy-like changes

Hanna

Zuckerman

Ferrey

et al. 2020

SAGE Open Medical Case Reports

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Clinical, histopathologic and molecular features of idiopathic and diabetic nodular mesangial sclerosis in humans

Eadon

Lampe

Baig

et al. 2021

Nephrology Dialysis Transplantation

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Background Idiopathic nodular mesangial sclerosis, also called idiopathic nodular glomerulosclerosis (ING), is a rare clinical entity with an unclear pathogenesis. The hallmark of this disease is the presence of nodular mesangial sclerosis on histology without clinical evidence of diabetes mellitus or other predisposing diagnoses. To achieve insights into its pathogenesis, we queried the clinical, histopathologic and transcriptomic features of ING and nodular diabetic nephropathy (DN). Methods All renal biopsy reports accessioned at Indiana University Health from 2001 to 2016 were reviewed to identify 48 ING cases. Clinical and histopathologic features were compared between individuals with ING and DN (n = 751). Glomeruli of ING (n = 5), DN (n = 18) and reference (REF) nephrectomy (n = 9) samples were isolated by laser microdissection and RNA was sequenced. Immunohistochemistry of proline-rich 36 (PRR36) protein was performed. Results ING subjects were frequently hypertensive (95.8%) with a smoking history (66.7%). ING subjects were older, had lower proteinuria and had less hyaline arteriolosclerosis than DN subjects. Butanoate metabolism was an enriched pathway in ING samples compared with either REF or DN samples. The top differentially expressed gene, PRR36, had increased expression in glomeruli 248-fold [false discovery rate (FDR) P = 5.93 × 10−6] compared with the REF and increased 109-fold (FDR P = 1.85 × 10−6) compared with DN samples. Immunohistochemistry revealed a reduced proportion of cells with perinuclear reaction in ING samples as compared to DN. Conclusions Despite similar clinical and histopathologic characteristics in ING and DN, the uncovered transcriptomic signature suggests that ING has distinct molecular features from nodular DN. Further study is warranted to understand these relationships.

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Idiopathic nodular glomerulosclerosis (ING) in an African American (AA) man with hepatitis C

Onteddu¹,

Duggirala²,

Reddy

2018

BMJ Case Reports

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Idiopathic nodular glomerulosclerosis (ING) in a non-diabetic patient is uncommon. Nodular glomerulosclerosis is hallmark sign of diabetic nephropathy. ING is a very rare clinicopathological disease associated with smoking, obesity and hypertension, chronic obstructive pulmonary disease and metabolic syndrome. A 68-year-old non-obese African American man with hypertension, smoking and history of hepatitis C presented to the clinic with progressive worsening of lower extremity oedema and declining renal function over few months. Renal biopsy demonstrated nodular glomerulosclerosis. In this case, ING is hypothesised to be associated with hepatitis C along with smoking and hypertension (HTN). We present this case to speculate the existence of yet unknown aetiologies of ING.

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Idiopathic nodular glomerulosclerosis: Report of two cases and review of literature

Cited by 3 publications

References 8 publications

Finding of pathological thrombomodulin gene variant in a patient with idiopathic nodular glomerulosclerosis and chronic thrombotic microangiopathy-like changes

Finding of pathological thrombomodulin gene variant in a patient with idiopathic nodular glomerulosclerosis and chronic thrombotic microangiopathy-like changes

Clinical, histopathologic and molecular features of idiopathic and diabetic nodular mesangial sclerosis in humans

Idiopathic nodular glomerulosclerosis (ING) in an African American (AA) man with hepatitis C

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