Idiopathic nodular glomerulosclerosis and differential diagnosis

ABSTRACT Introduction: Idiopathic nodular glomerulosclerosis (ING) is a condition that has a vasculopathic glomerular histological pattern. Case presentation: The authors present the case of a 44-year-old Hispanic smoker female with hypertension and peripheral arterial disease who presented nephrotic syndrome for 2 weeks. The patient was diagnosed with ING by percutaneous renal biopsy results, which showed global nodular mesangial matrix expansion, with linear staining accentuation of glomerular and tubular basement membrane for Immunoglobulin G (IgG) and albumin on immunofluorescence. Conclusions: ING is a rare disease with a poor renal prognosis and wide diagnostic approach; we highlight the importance of analyzing every piece of detail together to reach a definitive diagnosis.

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

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.

Other primary glomerular diseases

This chapter covers the other primary glomerular diseases, including their pathologies, treatment options for their management, and risk factors. Some of these primary glomerular diseases are quite rare. This chapter covers fibrillary glomerulonephritis (FGN), collagenofibrotic glomerulopathy, thin basement membrane nephropathy (TBMN), lipoprotein glomerulopathy (LPG), ‘pure’ mesangial proliferative glomerulonephritis (MesPGN), IgM nephropathy, C1q nephropathy, idiopathic nodular glomerulosclerosis, and C4 glomerulopathy. It describes the use of light microscopy, immunofluorescence, electron microscopy, and immunochemistry where applicable. For each disease, the natural history, clinical presentation, pathogenesis, and pathology are described, and, where applicable, specific studies are discussed. Any specific treatments are outlined for each.