Immunoadsorption in Anti-GBM Glomerulonephritis: Case Report in a Child and Literature Review

Abstract

Antiglomerular basement membrane glomerulonephritis (anti-GBM GN) is a rare autoimmune disease that is characterized by rapidly progressive glomerulonephritis that may be associated with pulmonary hemorrhage. Anti-GBM GN is caused by autoantibodies (classically type G immunoglobulin) directed against the α3 subunit of type IV collagen. Without any appropriate treatment, the disease is generally fulminant, and patient and kidney survival is poor. The current guidelines recommend the use of plasma exchanges and immunosuppressive drugs. Immunoadsorption (IA) can remove pathogenic IgGs from the circulation and do not require plasma infusions, contrary to plasma exchanges. IA has seldom been used in adult patients with good tolerance and efficiency. We report herein the first pediatric case successfully treated with IA combined with immunosuppressive drugs in a 7-year-old girl who presented acute kidney injury (estimated glomerular filtration rate 38 mL/minute/1.73 m). A kidney biopsy revealed numerous >80% glomerular crescents and linear IgG deposits along the glomerular basement membrane. Ten IA sessions led to rapid and sustained clearance of autoantibodies and improvement of kidney function until 21 months after onset (glomerular filtration rate 87 mL/minute/1.73 m). No adverse effect was noted. This report adds to the growing body of evidence suggesting IA as a therapeutic alternative to plasma exchanges in anti-GBM GN. The other 27 published pediatric cases of anti-GBM GN are reviewed.

Anti-glomerular basement membrane disease in children: a brief overview

“…A review of the apheresis protocols used within the literature in children with anti-GBM is summarised in Table 1 [8,[26][27][28][29][30][31][32]. Immunoadsorption leading to more specific removal of pathogenic immunoglobulins has been reported [33]. A comparison study 'of immunoadsorption' in adults failed to demonstrate any additional benefit over double filtration plasmapheresis [34].…”

Goodpasture's autoimmune disease — A collagen IV disorder

“…Finally, immunoadsorbtion may be employed for selective removal of pathogenic autoantibodies, leaving other plasma components intact and avoiding the risk of infection or allergic reaction [102]. Remarkably, the first pediatric case of removal of IgG by immunoadsorbtion in GP patient has been recently reported with rapid improvement and normalization of kidney function [103]. Given that GP autoantigen is well characterized and could be produced in recombinant form, development of advanced immunoadsorbtion protocol selectively targeting pathogenic autoantibodies could be envisioned in a near future.…”

Die Rasch Progessive Glomerulonephritis im Kindesalter

“…Beobachtet wird außerdem, dass das Goodpasture Syndrom eher bei Patienten < 30 Jahre und die isolierte Anti-GBM-Nephritis bei Patienten > 50 Jahre auftritt. Die Geschlechtsverteilung variiert von 1:1 bis hin zu 9:1 für das weibliche Geschlecht [7]. Anti-GBM-AK sind serologisch nachzuweisen, C3 und C4 sind meist normwertig [8].…”