Anti-glomerular basement membrane disease (anti-GBM disease), formerly known as Goodpasture’s syndrome, is a rare, autoimmune condition that impacts the kidneys and lungs. A recent study published in the Journal of the American Society of Nephrology investigates this disease, specifically the protein laminin-521’s role. Through a retrospective analysis, researchers evaluated whether or not laminin-521 is a target of autoimmunity in anti-GBM disease.
About Anti-GBM Disease
Anti-GBM disease, formerly known as Goodpasture’s syndrome, is a rare, autoimmune condition that occurs when autoantibodies attack the basement membranes of the small blood vessels in the lungs and kidneys. Medical professionals are unsure as to why the immune system begins to attack the body, but they believe that environmental factors and genetics play a role. In about half of cases, only the kidneys are impacted. If this is the case, the disease is referred to as anti-GBM glomerulonephritis. It is a very rare condition, impacting fewer than one of every million people. Caucasian males aged 15-35 are at the highest risk of this condition, followed by those above the age of 50.
Symptoms associated with the lungs include chest pain, chronic cough, coughing up blood, difficulty breathing, and even respiratory failure. Kidney-related symptoms include blood in the urine, foamy urine caused by proteinuria, fatigue, nausea, poor appetite, unintentional weight loss, confusion, and other symptoms associated with low kidney function. It is possible that affected individuals will experience kidney failure as well. In terms of treatment, early intervention yields the best results. Treatment options include dialysis, oxygen, immunosuppressive treatment, plasmapheresis, and avoiding possible triggers of the disease.
About the Study
In order to fully understand if laminin-521 is the target of autoantibodies, a team of researchers evaluated the circulating autoantibodies of 101 anti-GBM disease patients and 85 healthy controls. Through a solid-phase immunoassay, they were able to measure the IgG binding to human recombinant laminin-521.
They found that while one-third of the anti-GBM disease patients contained circulating IgG autoantibodies that were binding to laminin-521, none of the controls nor patients with other glomerular diseases did. After further investigation, the researchers discovered that those with anti-GBM disease with lung hemorrhaging had more active autoantibodies than those who only have kidney-related symptoms.
To conclude their research, the team of experts stated that laminin-521 is a major target of autoantibodies, just like α345(IV) collagen. They stated that the targeting of this protein may promote injury to the lungs in anti-GBM disease.
You can find the full study here.
