Researchers in Trinity College Dublin have published the first study to report national incidence rates of a rare kidney disease, anti-glomerular basement membrane (anti-GBM) disease. Their study, published in the Clinical Journal of the American Society of Nephrology, also showed clustering of cases in time and space, which suggests that there may be an unidentified environmental trigger for the onset of the disease.
Anti-GBM disease is an autoimmune condition that affects the kidneys and lungs, causing rapidly progressive kidney failure and lung bleeding. The cause of anti-GBM disease is unknown. From 2003-2014, seventy nine cases were identified in Ireland, equating to 1.6 cases per million Irish inhabitants each year. Ten cases were identified during a three month period in early 2013, with a concentration of cases in the South-East. A separate geographical cluster involving seven cases was observed in Donegal during the 11 year study period. These disease clusters suggest an environmental influence in the development of the disease.
The study is unique as it is the first to formally investigate patterns of incidence of anti-GBM disease at a country wide level, and will help further the understanding of this rare but important disease.
Because of the rarity of the disease, this first comprehensive national study represents a very valuable resource for further understanding of anti-GBM disease.
Speaking about the significance of the research, lead author of the study, Dr Mark Canney, a nephrologist and HRB research fellow at the Trinity Health Kidney Centre said: “Because of the rarity of the disease, this first comprehensive national study represents a very valuable resource for further understanding of anti-GBM disease. As well as finding that the disease may be triggered by unknown environmental factors, we also found that the disease behaved differently in the clusters compared to the other cases. What we conventionally think of as a single disease entity may in fact be several diseases.”
Professor and Consultant of Nephrology at Trinity’s School of Medicine, Mark Little said: “This research was the first all-Ireland collaborative study under the auspices of the new ‘Vasculitis Ireland Network’ rare disease centre of expertise. It involved collaboration across several disciplines including nephrology, immunology, histopathology and bioinformatics. It also demonstrates the value of such research networks for generation of robust Irish data at a national level. The Trinity Health Kidney Centre has recently been designated as the coordinating centre of expertise in Ireland for rare immune kidney disease by the new European Reference Network initiative for rare diseases. This will provide an ideal platform from which to build a national infrastructure for researching rare conditions such as anti-GBM disease in Ireland.”