Idiopathic collapsing glomerulopathy in children

Abstract

Idiopathic collapsing glomerulopathy (ICG) is a clinically and pathologically distinct variant of focal segmental glomerulosclerosis, characterized clinically by rapid progression of renal insufficiency, a male and African-American racial predominance, and pathologically by segmental glomerular collapse, visceral epithelial cell hypertrophy and hyperplasia, and the absence of endothelial tubuloreticular inclusions. Pathologically similar lesions have been reported in adult and pediatric patients with human immunodeficiency virus (HIV) infection and/or intravenous (IV) drug abuse. Most patients with ICG who have been reported in the literature are adults. Six children with ICG were retrospectively identified (two from East Carolina University, four from University of North Carolina-Chapel Hill). Clinical data and renal biopsy findings were reviewed for all patients. All six patients were male; five African-American and one Hispanic. Ages ranged from 2 to 17 years (mean 12 years). Steroid-resistant nephrotic syndrome was the presenting clinical finding. Average 24-h urine protein excretion was 6.3 g (range 3.2-15 g). Five patients were serologically negative for HIV infection (one patient not tested) and none had a history of IV drug abuse or known HIV risk factors. Progression to end-stage renal insufficiency in two patients within 1 year of biopsy required renal transplantation, and within 1 month of biopsy one patient required dialysis. We report a series of pediatric patients with ICG, an aggressive variant of focal segmental glomerulosclerosis. ICG in children is similar clinically and pathologically to this disease in adult patients.

Rituximab Treatment for Adult Patients with Focal Segmental Glomerulosclerosis

“…Compared with other variants of FSGS, collapsing FSGS is less responsive to steroids and other immunosuppressive drugs. According to most previous reports, the prognosis is usually poor, and patients are more likely to be in end-stage renal disease within a few years in spite of therapy (13,14). Kaito et al reported a case with steroid-and cyclosporin-resistant collapsing FSGS who went into complete remission with a combination rituximab and steroid pulse-therapy while receiving CyA (15).…”

A 20-year history of childhood HIV-associated nephropathy

“…However, today we know that under certain circumstances both adults and children not infected with HIV-1 can develop similar lesions [144,145]. Thus, it is tempting to speculate that other infectious agents may be involved in the pathogenesis of collapsing FSGS.…”

Collapsing Focal Segmental Glomerulosclerosis in a Young Child

“…In general, there is a poor response to steroids [12,13,14]. However, remission is achievable in some patients with other immunosuppressive therapy [14,15,16]. While there have been reports of efficacy for rituximab in FSGS (for example, Kaito et al [17] reported a case of a 2-year-old boy with steroid- and CsA-resistant collapsing FSGS who went into complete remission with a combination of intravenous rituximab and MP pulse therapy while receiving oral CsA), we were unable to offer rituximab in this case.…”

“…Our patient progressed very quickly to ESRF. It was reported previously that the prognosis is usually poor and that patients are likely to be in ESRF within a few years despite receiving treatment [15]. …”