Nephropathology Since 2006
   
Case 168
Diagnosis
 
     
Versión en Español

Go back to clinical information and images

Diagnosis: Focal and Segmental Glomerulosclerosis, Perihilar Variant

This variant is defined by the presence of at least one glomerulus with perihilar hyalinosis with or without sclerotic lesions at the glomerular vascular pole (perihilar) in more than 50% of glomeruli with segmental lesions. This form has been described in both primary FSGS and secondary adaptive forms stemming from nephron loss or glomerular hypertension (i.e., due to obesity, reflux nephropathy, hypertension, and sickle cell disease), usually accompanied by glomerular hypertrophy. In the adaptive conditions, reflex dilatation of the afferent arteriole leading to glomerular hypertension may cause particular stress on the perihilar segment. Foot process effacement is usually focal and relatively mild (Han MH, Kim YJ. Biomed Res Int. 2016;9375753. [PubMed link]).

The glomerular alteration (and proteinuria) in our case was clearly preceded by a long-standing systemic hypertension, then, we could also call it as "hypertensive nephropathy", however, this term is often used to speak of chronic scarring lesions with fibrosis, tubular atrophy, and nephroangiosclerosis assumed to be caused for chronic systemic arterial hypertension.

See the chapter: Focal and Segmental Glomerulosclerosis of our Tutorial.

Go back to clinical information and images

References

  • Nephro , 143(1), 38-42; 2019 Consequences of Glomerular Hyperfiltration: The Role of Physical Forces in the Pathogenesis of Chronic Kidney Disease in Diabetes and Obesity Avry Chagnac, Boris Zingerman, Benaya Rozen-Zvi, Michal Herman-Edelstein [PubMed link]
  • Saudi J Kidney Dis Transpl, 26(1), 173-81; 2015 Focal and Segmental Glomerulosclerosis: Does Prognosis Vary With the Variants? Guditi Swarnalatha, R Ram, Kiran Mai Ismal, Sharmas Vali, Manisha Sahay, K V Dakshinamurty. [PubMed link]
  • Ultrastruct Pathol, 35(4), 176-82; 2011 Clinicopathological Characteristics of Obesity-Associated Focal Segmental Glomerulosclerosis Sihem Darouich, Rym Goucha, Mohamed Habib Jaafoura, Semy Zekri, Hédi Ben Maiz, Adel Kheder [PubMed link]
  • Han MH, Kim YJ. Practical Application of Columbia Classification for Focal Segmental Glomerulosclerosis. Biomed Res Int. 2016;9375753. [PubMed link]
  • J Bras Nefrol, 35(2), 112-9; 2013 Histologic Variants of Primary Focal Segmental Glomerulosclerosis: Presentation and Outcome Luis F Arias, Carlos A Jiménez, Mariam J Arroyave [PubMed link]

[Top]

Go back to clinical information and images