Guillain Barré syndrome (GBS) is a rare, although serious, autoimmune condition that affects the peripheral nervous system, yet the underlying risk factors remain unknown. Consequently, there is a need for more substantial and reliable data to identify clinical and biological determinants and predictors of GBS to ultimately improve patient care.
Each year, GBS affects 1-2 people in every 100,000. Although thought of as rare, this equates to a lifetime risk to each of us of 1 in 1000. Weakness usually begins in the legs before spreading to other parts of the body. In severe cases, it spreads to and impairs the muscles required for swallowing and breathing. In this instance, about 1 in 4 patients require artificial ventilation, and 1 in 20 patients die from the condition. Recovery can take many months or even years, and may be incomplete.
The rarity of the disease makes it difficult to generate meaningful data in a single centre; even large neuroscience hospitals only admit around 10-15 GBS patients per year at most. For this reason, the Inflammatory Neuropathy Consortium established the International GBS Outcome Study (IGOS). IGOS provides a platform for researchers to collect and share clinical information and blood samples over multiple time points to assist large scale research projects. There are currently co-ordinating centres in 18 countries, each with multiple assessment centres. All data collected is uploaded to a central repository where it can be accessed online by the network of researchers involved. Already more than 1000 patients have been entered into the study worldwide, by far the largest collection of GBS patients ever assembled.
IGOS brings together researchers across many disciplines to investigate a diverse range of topics, from treatment interventions to genetic markers and biomarkers for predicting disease susceptibility and outcome. Identifying such markers is particularly important in order for new therapies to be targeted to those patients who will benefit the most. One field of research, which I am involved in, is investigating the disease mechanisms of GBS. In particular, the way in which the immune system causes damage to peripheral nerves. Using IGOS samples, it is possible to investigate the various types of antibodies present in individual patients at different stages of their disease. Using a model system, we can investigate the causes of nerve injury and what might be done to prevent damage or enhance repair.
Predictors and determinants of rare diseases, such as GBS, are difficult to understand due to the lack of high quality data. IGOS enables the accumulation and sharing of data on a global scale, holding promise for the improvement of GBS case detection, management and treatment. Importantly, IGOS sets a precedent for a research framework that could be translated into research for other rare disease conditions.
See https://www.gbsstudies.org for further information on the IGOS study.