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

Abstract

Anti-glomerular basement membrane disease (Anti-GBM), previously known as Goodpasture syndrome, is an extremely rare cause of rapidly progressive glomerulonephritis and chronic kidney disease stage 5 (CKD5) in children. It is associated with acute pulmonary haemorrhage and it has a poor prognosis. It is classified as an autoimmune, small-vessel vasculitis caused by autoantibody formation against the alpha-3 chain in type IV collagen found in the glomerular basement membrane. Evidence of anti-GBM antibodies in serum or histologically are required for diagnosis. Treatment in children is based on very limited adult data and often involves the use of acute apheresis to rapidly remove circulating factors coupled with intensive immunosuppression such as cyclophosphamide and intravenous corticosteroids. There is also an emerging role for the use of biologic agents such as B cell depletion. The evidence base in children with anti-GBM disease is extremely limited. Multi-centre international collaboration is required to provide insight into this disease, better describe its prognosis and work towards improving outcomes. This review article summarises the key features of this disease in children, highlights treatment options and considers areas of unmet need.

Hydralazine-Induced Antineutrophilic Cytoplasmic Antibody (ANCA)-Associated Vasculitis Presenting as Crescentic Glomerulonephritis

“…Anti-glomerular basement disease is characterized by the autoimmune development of antibodies towards type IV collagen in the GBM leading to rapidly progressive glomerulonephritis, but there should be evidence of anti-GBM antibodies in serum or histology, which was negative for our patient [ 6 , 7 ]. On the other hand, anti-histone and anti-dsDNA usually are absent in idiopathic AAV [ 8 ].…”

Anti-glomerular basement membrane disease in children: can Sars-Cov-2 be a trigger?

“…The age range mentioned is in line with the patient age in this case report. Most patients present with rapidly progressive glomerulonephritis, and concomitant alveolar hemorrhage may occur in some cases 1,2 . Kidney manifestations are characterized by acute kidney injury with urinalysis showing proteinuria, usually not in the nephrotic range, and sediment with dysmorphic red cells, white cells, and red cells and granular casts 3 .…”

Case report: A study on the de novo KMT2D variant of Kabuki syndrome with Goodpasture’s syndrome by whole exome sequencing

“…Goodpasture's syndrome is a severe and extremely rare autoimmune disorder characterized by the presence of circulating autoantibodies directed against the noncollagenous domain of the α3 chain of type IV collagen (9,10). Antibodies found in both glomerular and alveolar basement membranes resulting in glomerulonephritis (GN) and acute pulmonary hemorrhage are usually from the IgG class, with IgG1 and IgG3 subclasses predominating, while rare cases of IgA-and IgG4-mediated disease have been reported (10,25,26). Unlike reports in adults, very few cases of childhood Goodpasture's syndrome are available.…”