Nephropathy associated with sickle cell anemia: An autologous immune complex nephritis

Prognosis of the Nephrotic Syndrome in Sickle Glomerulopathy

“…Treatment with prednisone was not beneficial. Two of the 6 patients fol lowed by Pardo et al [4] presented with normal renal function which remained unchanged 1 and 2 years later, but their nephrotic syndrome persisted. Three patients presented with impaired renal function, and died in ure mia 2, 3 and 18 months later, not having benefited from therapy with steroids and cyclophosphamide.…”

“…In 9 of these (75%) the glo merular lesion, henceforth called sickle glomerulopathy, exhibited mesangial proliferation and basement mem brane duplication, somewhat resembling membranoproliferative glomerulonephritis (MPGN). This lesion has been described in 19 of 37 adults (51 %) and 10 of 31 children (32%) with sickle cell anemia and marked pro teinuria (table I) [1][2][3][4][5][6][7][8], In this report we describe the course of 9 patients with sickle glomerulopathy and dis cuss the prognostic implications of the nephrotic syn drome.…”

“…Pardo et al [4], Ozawa et al [24], and Strauss et al [25] maintained that immune deposits of renal tubular epithelial antigen (RTE) and autologous anti-RTE anti bodies were involved in the pathogenesis of sickle glom erulopathy, while the abnormal endothelial adherence of sickled erythrocytes described by Hebbel et al [26] and Hoover et al [27] was cited by others as a possible cause for the glomerular lesions.…”

“…The 6th was admitted in uremia and died shortly thereafter. Two of the 4 who died had received hemodialysis treatment [4], Nicholson et al [5] followed 4 patients with MPGN for an average of 16 months; 3 progressed to advanced azotemia and the 4th showed a slight worsening of renal function [5], Adding our 9 patients to the 13 just cited from the literature, we find a chronic renal failure rate of 68%, a mortality rate of 50%, and a median survival of 16 months (table III). The survival rate of the 15 patients with chronic renal failure was worse than that of the 7 with stable renal function (p = 0.03), and the difference remained significant even after deleting the 5 survivors who had less than a 2 year follow-up (p = 0.01).…”

Idiopathic and Secondary Mesangiocapillary Glomerulonephritis

“…It was soon recognized that the double-track appear ance is not limited to the idiopathic MCGN, but also occurs in a variety of diseases affecting glomeruli such as shunt nephritis [6][7][8], nephritis of chronic suppurative infection [9] and of subacute bacterial endocarditis [10], lupus nephritis [I I, 12], cryoglobulinemia [13][14][15], glo merulopathy associated with hepatitis B infection [16], malarial nephropathy [17,18], schistosomal nephropathy [19,20], heroin nephropathy [21,22], sickle cell disease [23][24][25], hemolytic-uremic syndrome [26][27][28], transplant nephropathy [29,30], radiation nephritis [31,32] and nephrotic syndrome associated with tumors, especially leukemias and malignant lymphomas [33,34], Further more, minor degrees of mesangial interposition are found in many chronic, occasionally also in acute glo merular diseases of various origins'.…”