Immunotactoid glomerulopathy in sickle cell anemia

A 12-year-old African American male with homozygous sickle cell disease (SC D) was admitted with insidious onset of periorbital and scrotal edema. The initial evaluation failed to reveal any underlying monoclonal gammopathy, o r cryoglobulinemia, or other systemic causes for the renal disease. A percu taneous renal biopsy was consistent with immunotactoid glomerulopathy (ITG) , which is rare in children and is characterized histologically by fibrilla r deposits in the glomeruli. Children can present with symptoms of nephroti c syndrome and progress to end stage renal disease. Our patient was treated with an ACE inhibitor and is currently free of edema and with normal renal function on followup at 1 year. Immunotactoid glomerulopathy should be con sidered in the differential diagnosis of nephrotic syndrome in children wit h sickle cell disease. Renal biopsy is indicated in children with sickle ce ll disease and nephrotic syndrome and ITG should be considered as potential cause. Although there is no effective treatment for this condition, ACE in hibitors can decrease the proteinuria and possibly delay the progression to end stage renal disease. The side effects related to the use of ACE inhibi tors should be monitored. These include renal impairment, hyperkalemia, ane mia, neutropenia, and angioedema. Since we have a short follow-up in our pa tient, the role and safety of ACE inhibitors in the management of ITG need further evaluation.