Autoimmune diseases
Autoimmune diseases can affect any tissue and organ, at any age, with a greater prevalence among women.1,2
There are approximately 80 recognized autoimmune diseases, with evidence of more than 100 existing.3
Autoimmune diseases are caused by the immune system mistakenly mounting an attack against the body’s own cells and tissues often through the action of autoantibodies, antibodies that participate in the autoimmune attack.1,4,5
Our commitment
Hansa is conducting clinical trials to evaluate the potential for imlifidase to treat rare autoimmune diseases where IgG plays a significant role including in anti-glomerular basement membrane (anti-GBM) disease, and Guillain-Barré Syndrome. Additionally, imlifidase is being evaluated in an investigator-initiated trial in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis.
Anti-GBM disease
Anti-GBM, also known as Goodpasture’s syndrome, is an acute and very severe inflammatory disease in which IgG-antibodies attack an antigen intrinsic to the glomerular basement membrane, resulting in an acute immune attack on the kidneys and, in some patients, on the lungs.6 Severe anti-GBM can be life-threatening, sometimes causing kidney failure and bleeding in the lungs7,8. The acute autoimmune attacks can become fatal in up to one in eight patients in the first year,9 while the majority of patients lose their kidney function and end up on dialysis.7,10 In anti-GBM, it is estimated that only one in three patients will have a preserved renal function after six months with current standard of care.8
Our commitment
Imlifidase has been granted orphan drug designation in anti-GBM antibody disease by both the US Food and Drug Administration and European Commission. Hansa Biopharma is currently conducting a global pivotal phase 3 trial, GOOD-IDES-02 to assess the use of imlifidase in combination with standard of care versus standard of care alone in the treatment of patients affected by severe anti-GBM disease.
For more information: NCT05679401
“Given the severity of Anti-GBM’s acute phase, the autoimmune reaction needs to be counteracted and stopped as quickly as possible if we want to limit the damage to the organs. Only if treatment is instituted early, there is a chance of salvaging the organ’s function.”
Mårten Segelmark, Professor of Nephrology at Lund University.
patients' perspective
Suspended time: a journey from diagnosis to transplant
"Dialysis may sound simple, but it isn’t, and over the course of his treatment Ron underwent several different kinds, all of which made him feel awful."
Guillain-Barré Syndrome
Guillain-Barré Syndrome (GBS) is an acute, rare, paralyzing, inflammatory disease of the peripheral nervous system usually preceded by an infection or other immune stimulation that induces an aberrant autoimmune response targeting peripheral nerves and their spinal roots. GBS is a rapidly progressive monophasic disorder often leading to a severe paresis of the arms and legs.
Approximately 25% of patients require mechanical ventilation for days to months following the acute autoimmune attack.11 Even with subsequent recovery from the acute condition, many patients suffer from long-term complications, leaving 20% unable to walk after six months12 and many patients experiencing residual clinical deficits, including weakness, sensory signs, fatigue, and pain.
Our commitment
Imlifidase has been granted orphan drug designation for treating GBS from the US Food and Drug Administration. We have conducted an open-label, single arm, multi-center Phase 2 study to evaluate the safety, tolerability, and efficacy of imlifidase in combination with standard of care IVIg in GBS patients.
For more information: NCT03943589
“In my career, I have seen cases where patients had to make three visits to the emergency room before the diagnosis of GBS was made. This complicates things, as a timely diagnosis and treatment is crucial to reduce the severity of the symptoms and minimize long term damage.”
Professor Shahram Attarian, Head of Department of Neuromuscular Diseases and ALS, Hopitaux Universitaires de Marseille (APHM).
References
1. Angum F, et al. The Prevalence of Autoimmune Disorders in Women: A Narrative Review. Cureus. 2020 May 13;12(5):e8094. doi: 10.7759/cureus.8094.
2. Pisetsky, D.S. Pathogenesis of autoimmune disease. Nat Rev Nephrol 19, 509–524 (2023). https://doi.org/10.1038/s41581-023-00720-1
3. “List of Autoimmune Diseases". Autoimmune Registry Inc. https://www.autoimmuneregistry.org/autoimmune-diseases
4. Wang L, et al. Human autoimmune diseases: a comprehensive update. J Intern Med. 2015 Oct;278(4):369-95. doi: 10.1111/joim.12395.
5. Ma H, Murphy C, Loscher CE and O’Kennedy R (2022) Autoantibodies - enemies, and/or potential allies? Front. Immunol. 13:953726. doi: 10.3389/fimmu.2022.953726
6. UNC Kidney Center: Anti-GBM Disease. Available at: https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/anti-gbm-disease/.
7. Kluth DC, Rees AJ. Anti-glomerular basement membrane disease. J Am Soc Nephrol. 1999 Nov;10(11):2446-53. doi: 10.1681/ASN.V10112446.
8. Hellmark T, Segelmark M. Diagnosis and classification of Goodpasture's disease (anti-GBM). J Autoimmun. 2014 Feb-Mar;48-49:108-12. doi: 10.1016/j.jaut.2014.01.024.
9. Sánchez-Agesta M, et al. (2022) Anti-glomerular Basement Membrane Glomerulonephritis: A Study in Real Life. Front. Med. 9:889185. doi: 10.3389/fmed.2022.889185
10. McAdoo SP, Pusey CD. Anti-Glomerular Basement Membrane Disease. Clin J Am Soc Nephrol. 2017 Jul 7;12(7):1162-1172. doi: 10.2215/CJN.01380217.
11. Fletcher DD et al. Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation. Neurology. 2000 Jun 27;54(12):2311-5. doi: 10.1212/wnl.54.12.2311
12. Van Doorn PA. Diagnosis, treatment and prognosis of Guillain-Barré syndrome (GBS). Presse Med. 2013 Jun;42(6 Pt 2):e193-201. doi: 10.1016/j.lpm.2013.02.328.