Proteinuria and the nephrotic syndrome in homozygous sickle cell anaemia

Thirteen patients with homozygous sickle cell anaemia (SCA) and clinical and laboratory evidence of renal glomerular disease were studied in an attempt to determine the pathogenesis of proteinuria and the nephrotic syndrome in this group of patients. Correlation was made between the clinical presentation and the renal histological changes in assigning the patients to various groups. Patients with active leg ulcers and acute presentations showed evidence of poststreptococcal glomerulonephritis and spontaneous resolution of the renal disease in the majority of cases. Other groups showed persistent proteinuria and/or progressive deterioration of renal function. Serological evidence of streptococcal infection was present in all groups regardless of whether the renal histology showed a proliferative or membranoproliferative glomerulonephritis. It is concluded that streptococcal glomerulonephritis is an important cause of proteinuria and the nephrotic syndrome in SCA and that chronic leg ulceration may facilitate the acquisition of such diseases.