The Expression of Heat Shock Proteins 27 and 70 in Lupus Nephritis

Background: Heat shock protein (HSP ) up-regulation is a cytoprotective response following stress insults (toxic, ischemic, inflammatory and oxidative). Ob ject ive: To study the localization of HSP 27 and HSP 70 in the renal tissue of patients with lupus nephritis (LN) and correlate our findings with the severity of histological involvement (activity and chronicity indices) and the degree of renal function impairment. Pat ients and Methods: Seventy patients with LN (diffuse proliferative n=31, focal proliferative n=20, and membranous n=19) were included in the study. The distribution of HSP 27/HSP 70 was studied by immuno-histochemistry in renal biopsy sections. A double staining method for vimentin, a-smooth muscle actin, CD 34 and CD 68 (+) cells were performed to identify the type of glomerular cells expressing HSP s. The severity of immunostaining for HSP 27/70 was evaluated semiquantitively. Results : HSP 27 and HSP 70 were identified within the cytoplasm of tubular epithelial cells of all patients. Increased HSP 27 expression was noted within intrinsic glomerular cells in diffuse lupus nephritis whereas no glomerular expression was observed in focal proliferative and membranous LN. A significant positive correlation was found between HSP 27 expression in diffuse proliferative nephritis and the activity and total (activity + chronicity) indices. The severity of histological involvement was also related to the degree of renal function impairment. Conclusions: Up-regulation of heat shock protein expression was identified in patients with various types of LN, especially those with diffuse proliferative nephritis. The severity of HSP 27 expression was related to the activity and total indices. These results suggest a possible defensive role for HSP 27 in severe lupus nephritis

Littoral cell angioma of the spleen accompanied by haemophagocytic syndrome in a dialysis patient suffering from aa amyloidosis

Littoral cell angioma (LCA) is a rare form of vascular tumor unique to the spleen that arises from the specialized endothelial cells that line the splenic sinuses (littoral cells). Haemophagocytic syndrome (HS) is also a rare hematologic disorder that some times accompanies LCA. The authors describe a young dialysis patient with a history of familiar mediteranean fever and secondary amyloidosis who was found to have this rare association of AA amyloidosis with LCA and haemophagocytic syndrome

COL4A3/COL4A4 Mutations Producing Focal Segmental Glomerulosclerosis and Renal Failure in Thin Basement Membrane Nephropathy

<p>Mutations in the COL4A3/COL4A4 genes of type IV collagen have been found in ~40% of cases of thin basement membrane nephropathy, which is characterized by microscopic hematuria and is classically thought to cause proteinuria and chronic renal failure rarely. Here we report our observations of 116 subjects from 13 Cypriot families clinically affected with thin basement membrane nephropathy. These families first came to our attention because they segregated microscopic hematuria, mild proteinuria, and variable degrees of renal impairment, but a dual diagnosis of focal segmental glomerulosclerosis (FSGS) and thin basement membrane nephropathy was made in 20 biopsied cases. Molecular studies identified founder mutations in both COL4A3 and COL4A4 genes in 10 families. None of 82 heterozygous patients had any extrarenal manifestations, supporting the diagnosis of thin basement membrane nephropathy. During follow-up of up to three decades, 31 of these 82 patients (37.8%) developed chronic renal failure and 16 (19.5%) reached end-stage renal disease. Mutations G1334E and G871C were detected in seven and three families, respectively, and were probably introduced by founders. We conclude that these particular COL4A3/COL4A4 mutations either predispose some patients to FSGS and chronic renal failure, or that thin basement membrane nephropathy sometimes coexists with another genetic modifier that is responsible for FSGS and progressive renal failure. The findings presented here do not justify the labelling of thin basement membrane nephropathy as a benign condition with excellent prognosis.</p&gt