Immune Complex Membranoproliferative Glomerulonephritis (IC-MPGN), a rare and progressive kidney disease, currently lacks approved treatments, but emerging therapies are showing promise. A recent report highlights the epidemiology, market trends, and potential future treatments for IC-MPGN across the United States, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan.
Disease Burden and Current Treatment Landscape
In 2023, approximately 7,100 diagnosed prevalent cases of IC-MPGN were reported across the 7MM, with the United States accounting for approximately 55% of these cases. The disease is more prevalent in adults, with about 90% of cases linked to chronic infections, autoimmune disorders, or underlying conditions like hepatitis C or lupus. These conditions lead to persistent immune complex formation, triggering the complement system and causing kidney damage.
Currently, treatment for IC-MPGN primarily involves off-label prescription drugs, including immunosuppressants, steroids, and renin-angiotensin-aldosterone system (RAAS) inhibitors. These treatments aim to manage symptoms and slow disease progression, but they do not address the underlying cause of the disease.
Emerging Therapies: Targeting the Complement System
The report identifies two promising therapies in late-stage clinical development: iptacopan (Novartis) and pegcetacoplan (Apellis Pharmaceuticals), both of which target the complement system. These drugs represent a potential shift towards more targeted and effective treatments for IC-MPGN.
Iptacopan (Novartis)
Iptacopan is an investigational, first-in-class, orally administered factor B inhibitor of the alternative complement pathway. It is currently in Phase III clinical trials and is being developed for several complement-driven renal diseases (CDRDs), including IC-MPGN. Novartis plans to seek regulatory approval for iptacopan by 2026, with preliminary Phase III trial results expected the same year.
Pegcetacoplan (Apellis Pharmaceuticals)
Pegcetacoplan is a targeted C3 inhibitor designed to regulate excessive complement activation. It is currently approved in the United States for paroxysmal nocturnal hemoglobinuria and is being investigated for IC-MPGN in a Phase III trial (VALIANT). In August 2024, Apellis and Sobi announced positive topline results from the VALIANT study in patients with C3 glomerulopathy (C3G) or primary IC-MPGN.
Market Outlook and Future Trends
The IC-MPGN market in the United States was valued at approximately $12 million in 2023 and is projected to increase by 2034. This growth is attributed to the rising diagnosed prevalence of IC-MPGN and the anticipated entry of emerging therapies with premium pricing.
The development of complement inhibitors faces challenges, including the complexity of the complement system and its ability to adapt to pathway blockades. However, the potential for targeted therapies to address the root cause of IC-MPGN represents a significant advancement in the treatment landscape.
Key opinion leaders (KOLs) emphasize the importance of understanding the evolving treatment landscape and patient needs to ensure successful drug uptake and accessibility. The report provides insights into market access, reimbursement scenarios, and the potential impact of emerging therapies on the IC-MPGN market.
