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A spotlight on anti-GBM disease

Anti-glomerular basement membrane disease (anti-GBM), also known as Goodpasture ́s disease, is an acute and very severe inflammatory disease impacting the kidneys and affecting around 1.5 in a million people annually1. This ultrarare condition is part of the set of glomerulonephritis (GNN), and it often leads to dialysis and the need for kidney transplant.

What is anti-GBM?

Anti-GBM is an autoinflammatory disease characterized by the development of IgG-antibodies that attack a specific part of the kidney and lung called the glomerular basement membrane (GBM). This results in damage of the organs.

Anti-GBM disease is one form of glomerulonephritis (GNN), a set of inflammatory conditions of the kidney and one of the leading causes for kidney disease. In glomerulonephritis, the inflammation starts in the glomeruli, the filtering units of the kidney, and the small blood vessels. As the inflammation progresses, damaging the tissues, the renal function is progressively lost.

What is the impact of anti-GBM?

Many patients with anti-GBM disease lose kidney function and require chronic dialysis and kidney transplantation2 and/or may experience bleeding from the lungs. In one out of six patients, anti-GBM can become fatal during the acute phase, while the majority of patients ends up on dialysis3, waiting for the opportunity for a kidney transplant. Only one in three anti-GBM patients will have a preserved renal function after six months with current treatment4.

A disease challenging to diagnose

One of the most problematic aspects in the diagnosis of these conditions is that patients with glomerulonephritis, including those diagnosed with anti-GBM disease, usually experience quite diffuse symptoms in the initial stages. When the symptoms become apparent, the damage of the organ is often in an advanced state. This is what happened to Ron, when he was eventually diagnosed with anti-GBM (link to Ron’s story).

1. Henderson R et al. Nephrology Dialysis Transplantation 2018. (33) 2: 196-202. 

2. McAdoo S et al. Anti-GBM disease. Clin J Am Soc Nephrol 2017. 12: 1162–1172.

3. Cohort of 13 studies (661 patients in anti-GBM 1993-2017) Treating anti-GBM disease with imlifidase Mårten Segelmark, Professor OF Nephrology

4. Kluth et al. J Am Soc Nephrol. 1999 Nov;10(11):2446-53 and Hellmark et al. J Autoimmun. 2014 Feb-Mar;48-49:108-12