Role of Endothelial Protein C Receptor in Pathogenesis of Lupus Nephritis

Asmaa Ahmed El-Sayed Ahmed Shaaban


SUMMARY AND CONCLUSION L upus nephritis is a frequent and serious manifestation of SLE with remarkable higher morbidity and mortality. The main pathogenesis of renal damage in LN is the immune complex formation and deposition in the glomeruli either in situ or circulating preformed complexes generated by autoantibodies inducing inflammatory process and damage. Other pathogenic mechanisms play a role in LN as the complement, coagulation pathway and vasculopathy. This was neglected in the histopathological classification that treatment options rely mainly on it. EPCR is a transmembrane endothelial receptor with both anti-thrombotic and anti-inflammatory properties through its regulation of protein C activation. In pathological states, as inflammatory status, EPCR is shed into a soluble form which has proinflammatory and procoagulant properties. Studies on the role of EPCR in SLE revealed its participation in the pathobiology of the disease. This is evidenced by alter distribution, of both soluble and membrane-bound forms which may reflect its role in vasculopathyof SLE. We conducted this study on 30 SLE patients (further subdivided into 2 subgroups; patients with LN and patients without LN) and 40 controls subdivided into two subgroup; 30 apparently healthy subjects serving as a control group to serum studies and 10 renal biopsies from surgical nephrectomies as controls for immunohistochemistry study. SLE patients were studied at two visits, 3 months apart. All SLE patients were subjected to history taking, clinical examination, laboratory investigations as CBC, ESR, Anti-dsDNA autoantibody, serum C3, C4 and renal profile. Assessment of disease activity by SLEDAI score, damage index, prognosis and response to treatment were measured. EPCR was assessed in the renal biopsy for the membranous form and in the serum for the soluble form. The study revealed:  The sEPCR was significantly higher in SLE patients than controls. Also, it was higher in those with LN and with vasculitic rash, but no statistical significant difference in other clinical manifestations.  Soluble EPCR (sEPCR) percentage of change was statistically significantly positive correlated to the percentage of change of disease activity in SLE patients with & without LN, but no relation was found to the SLICC/ACR damage index.  Soluble EPCR (sEPCR) change was statistically significantly related to prognosis grades, and response to treatment in SLE patients with LN which render it a reflection for these indices and indicator of bad prognosis and poor response to treatment in LN patients.  Negative immunohistochemistry staining of the mEPCR in biopsies of LN patients with no relation to the pathological classification.

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Other Titles دور مستقبلات البروتين الغشائي ج في التسبب فى الإلتهاب الكلوى فى مرضى الذئبة الحمراء
Issue Date 2015

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