IgA Nephropathy Market Insight, Epidemiology and Market Forecast - 2034

Key Highlights

• According to the IgA Nephropathy Foundation, IgA nephropathy is an autoimmune disease that attacks the kidneys and affects blood filtration in the kidneys' small blood vessels. IgA nephropathy occurs when an abnormal protein damages the filtering unit (glomerulus) inside the kidneys. It is estimated that 20-40% of people with IgA nephropathy will develop end-stage kidney disease, which means they will need dialysis or kidney transplantation to survive.
• The diagnosis of IgA nephropathy relies on revealing IgA as the dominant or co-dominant immunoglobulin in the glomerular mesangium by kidney biopsy. Although renal biopsy remains the gold standard for IgA nephropathy diagnosis, recent efforts have sought new biomarkers to predict IgA nephropathy without needing a biopsy. However, unclear pathogenesis complicates noninvasive diagnosis.
• Conventional major treatments to manage IgA nephropathy include ACE inhibitors (angiotensin-converting enzyme inhibitors) and ARBs (angiotensin II receptor blockers) to manage symptoms like high blood pressure. Other treatments include corticosteroids to slow the progression of the disease and other immunosuppressants like mycophenolate mofetil (MMF), cyclophosphamide, etc.
• Corticosteroids are the most commonly used immunosuppressive therapy for IgA nephropathy. Recent studies have shown that lower doses of systemic corticosteroids when paired with antimicrobial prophylaxis, can be effective. However, concerns about treatment-related toxicity and poor tolerability persist, even at these reduced doses. A new approach involves targeting corticosteroid delivery to the ileum, which may enhance efficacy while minimizing systemic toxicity.
• The latest Kidney Disease Improving Global Outcomes (KDIGO) guidelines advise that patients with IgA nephropathy receive optimized supportive care to maintain kidney function and lower cardiovascular risk. This includes effectively managing blood pressure, utilizing the highest tolerable doses of Renin-angiotensin System Inhibitors (RASi), limiting dietary sodium, achieving a healthy body weight, and quitting smoking. However, RASi are frequently not used to their full potential.
• In addition to RASi, new treatment options for the chronic kidney disease aspect of IgA nephropathy include Sodium-glucose Cotransporter-2 (SGLT2) inhibitors, Dual Endothelin-angiotensin Receptor antagonists (DEARAs [FILSPARI]), Complement inhibitor (FABHALTA) endothelin receptor antagonists (ERAs), and Mineralocorticoid Receptor Antagonists (MRAs).
• Few therapies are being investigated for the treatment of IgA nephropathy. Some key players involved in the development are Novartis, Visterra (a subsidiary of Otsuka Pharmaceutical), Vera Therapeutics, Ionis Pharmaceuticals/Roche, Vertex Pharmaceuticals/Alpine Immune Sciences, AstraZeneca, Vera Therapeutics, Biogen, and others.
• Emerging therapies such as Atrasentan, Sibeprenlimab (VIS-649), Zigakibart (BION-1301), IONIS-FB-LRx/RG6299, Povetacicept, ULTOMIRIS (ravulizumab), Atacicept (VT-001), Felzartamab, and others are under different phases of clinical trial for the treatment of IgA nephropathy.
• The growth of the IgA nephropathy market is expected to be mainly driven by innovations in treatment options, the rise in prevalence, technological advancements in the healthcare industry, etc.
• Delays in diagnosis, limited Treatment Options, economic burden, and lack of proper understanding of the disease will be going to hit the IgA nephropathy market.

This report delivers an in-depth understanding of IgA nephropathy, historical and forecasted epidemiology as well as IgA nephropathy market trends in the United States.

The IgA nephropathy market report provides current treatment practices, emerging drugs, market share of individual therapies, and current and forecasted IgA nephropathy market size in the United States from 2020 to 2034. The report also covers current IgA nephropathy treatment practices/algorithms and unmet medical needs to curate the best opportunities and assess the market’s potential.

Geography Covered

• The United States

IgA Nephropathy Understanding and Treatment Algorithm

IgA Nephropathy Overview

According to the IgA Nephropathy Foundation, IgA nephropathy is an autoimmune disease that attacks the kidneys and affects blood filtration in the kidneys' small blood vessels. IgA nephropathy occurs when an abnormal protein damages the filtering unit (glomerulus) inside the kidneys. It is estimated that 20-40% of people with IgA nephropathy will develop end-stage kidney disease, which means they will need dialysis or kidney transplantation to survive.

A family history of chronic nephritis, susceptibility to the common cold, preference for salty foods, frequent consumption of raw eggs, and a high intake of carbohydrates, including rice, are significantly associated with an increased risk for IgA nephropathy.

IgA Nephropathy Diagnosis

IgA nephropathy might be suspected when a patient has bloody (red) or dark urine following a respiratory tract illness such as a sore throat or a cold. Although a doctor may have a high index of suspicion that their patient has IgA nephropathy based on history, physical exam, urine tests, and blood tests, the only way to truly diagnose IgA nephropathy is through a kidney biopsy.

A kidney biopsy is when a small needle is inserted into a person’s kidney to take a small sample of tissue. This tissue is then examined under a microscope to look for certain characteristic markers of the disease.

IgA Nephropathy Treatment

The management of IgA nephropathy begins with confirming the diagnosis, typically through a renal biopsy, while ruling out secondary causes. Key considerations for formulating the management plan include assessing proteinuria, eGFR, blood pressure, and histological findings. The primary goals of treatment are to induce remission and avert the onset of complications.

First-line agents for managing proteinuria and lowering blood pressure in IgA nephropathy patients typically involve ACE inhibitors or angiotensin receptor blockers (ARBs). The target blood pressure is typically set at 130/80 mm Hg. If proteinuria is more than 1 g/d, the systolic blood pressure target is less than 125 mm Hg.

The treatment of IgA nephropathy includes corticosteroids, novel agents (sparsentan, budesonide, sodium-glucose cotransporter-2 (SGLT2) inhibitors, combination therapy, tonsillectomy, renal transplant, other immunosuppressive agents (mycophenolate mofetil [MMF], rituximab, and other medications (cyclophosphamide, azathioprine, calcineurin inhibitors, and leflunomide).

IgA Nephropathy Epidemiology

The IgA nephropathy epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented by Total Diagnosed Prevalent Cases of IgA Nephropathy, Age-specific Cases of IgA Nephropathy, Gender-specific Cases of IgA Nephropathy, Treatment Rate of IgA Nephropathy, and Patient at Risk of Progression in IgA Nephropathy in the United States from 2020 to 2034.

• In 2023, the diagnosed prevalent cases of IgA nephropathy in the United States was approximately 130,000.
• The incidence of IgA nephropathy in men is around two times higher than in females, worldwide. The onset of IgA nephropathy is often in the second or third decades of life (18-65 years of age), affecting teens and young adults more than other age groups.

IgA Nephropathy Drug Chapters

The section dedicated to drugs in the IgA nephropathy report provides an in-depth evaluation of pipeline drugs (Phase III, and Phase II) related to IgA nephropathy.

The drug chapters section provides valuable information on various aspects related to clinical trials of IgA nephropathy, such as the pharmacological mechanisms of the drugs involved, designations, approval status, patent information, and a comprehensive analysis of the pros and cons associated with each drug. Furthermore, it presents the most recent news updates and press releases on drugs targeting IgA nephropathy.

Marketed Therapies

TARPEYO (TRF budesonide): Asahi Kasei (Calliditas Therapeutics)

TARPEYO is a patented oral formulation of a potent and well-known active substance of budesonide for targeted release. The formulation delivers the drug to the Peyer’s patch region of the lower small intestine, where the disease originates, as per the predominant pathogenesis models. TARPEYO is derived from the TARGIT technology, which allows the substance to pass through the stomach and intestine without being absorbed and released in a pulse-like fashion only when it reaches the lower small intestine.

In addition to its potent local effect, another advantage of using this active substance is its very low bioavailability, i.e., around 90% of it is inactivated in the liver before it reaches systemic circulation. This means that a high concentration can be applied locally where needed but with only very limited systemic exposure and side effects.

In March 2024, Calliditas Therapeutics announced that the FDA had granted an orphan drug exclusivity period of seven years for TARPEYO, expiring in December 2030, based on when the company obtained full approval with a new indication for this drug product.

In August 2023, the FDA granted priority review for full approval of TARPEYO for the treatment of IgA Nephropathy.

In February 2023, Calliditas Therapeutics received CMA from the MHRA of the United Kingdom for KINPEYGO to treat IgA nephropathy in adults at risk of rapid disease progression with a urine protein-to-creatinine-ratio (UPCR) =1.5 g/g.

FILSPARI (sparsentan): Travere Therapeutics

FILSPARI (sparsentan) is a once-daily oral medication that selectively targets two critical pathways in the disease progression of IgA nephropathy (endothelin-1 and angiotensin II or DEARA) is the first and only non-immunosuppressive therapy approved for the treatment of this condition. Endothelin I and angiotensin II lead to a decline in kidney function by contributing to inflammation and fibrosis in the kidney, changes to the shape of podocytes, podocyte loss, and increased permeability of the glomerular filtration barrier. Furthermore, endothelin I and angiotensin II are vasoconstrictive, causing blood vessel narrowing and increased pressure in the glomeruli.

In May 2024, the FDA granted a Priority Review of the company’s supplemental New Drug Application (sNDA) to convert FILSPARI from accelerated approval to full approval for the treatment of IgA nephropathy in the US.

Emerging Therapies

Atrasentan: Novartis

Atrasentan is an investigational potent and selective oral ETA receptor antagonist, currently in Phase III development for IgA nephropathy and early-stage development for other rare kidney diseases. Activation of the ETA receptor contributes to elevated proteinuria, which is associated with kidney damage, fibrosis, and loss of kidney function in IgA nephropathy. Atrasentan has the potential to be added to current supportive therapy to reduce persistent proteinuria and preserve kidney function for a broad patient population. Preclinical models have also suggested that atrasentan may reduce inflammation and fibrosis in IgA nephropathy.

In the second quarter of 2024, Novartis received FDA filing acceptance for atrasentan for treating IgA nephropathy.

Novartis is expecting that the final analysis from the ALIGN (CHK01-01) trial, including the key secondary endpoint of change from baseline in eGFR at 136 weeks, and the results in participants receiving an SGLT2 inhibitor as background care in an exploratory cohort, is expected in 2026.

Sibeprenlimab (VIS-649): Visterra (a subsidiary of Otsuka Pharmaceutical)

Sibeprenlimab (formerly VIS649) is an investigational humanized IgG2 monoclonal antibody that reduces the production of Gd-IgA1 by binding to a specific signaling molecule called APRIL (proliferation-inducing ligand), which has been demonstrated to be a driver of IgA and Gd-IgA1 production. By binding and neutralizing APRIL, sibeprenlimab may reduce the amount of IgA and Gd-IgA1. Lower levels of Gd-IgA1 may then result in reduced auto-antibody production, which in turn may result in fewer immune complexes, decreased immune complex deposits in the kidney, and reduced kidney inflammation. By reducing the production of Gd-IgA1, sibeprenlimab is believed to prevent further kidney damage and the progression to end-stage kidney disease.

As a first-in-class and easy-to-use drug, sibeprenlimab is actively being developed for indications other than IgA nephropathy as well in its LCM program. Subcutaneous injection with a dosing interval of once every 4 weeks is expected to have a safer and better tolerability profile without excessive immunosuppressive effect.

In February 2024, the FDA granted a Breakthrough Therapy Designation (BTD) for the investigational drug sibeprenlimab for the treatment of IgA nephropathy.

Drug Class Insight

Complement inhibitor

The complement system is an important component of innate immunity and can be activated through three different pathways: the classical pathway, lectin pathway, and alternative pathway, all of them converging in the terminal pathway. Several studies have provided evidence for the activation of alternative pathways, lectin pathways, and terminal pathways as effector mechanisms of kidney injury in IgA nephropathy. It should be noted that alternative pathway plays an important role in complement amplification even when other pathways are primarily activated. Conversely, the absence of C1q in most IgA nephropathy kidney biopsies suggests that the classical pathway is not significantly involved in its pathogenesis.

All the breakthroughs in the understanding of IgA nephropathy and the development of novel complement-targeted therapies are likely to usher in a new era of personalized treatment. With the advent of precision medicine, the possibilities for tailored approaches based on a patient's characteristics offer hope for improved outcomes in IgA nephropathy patients.

IgA Nephropathy Market Outlook

IgA nephropathy is the most common primary glomerulonephritis, potentially progressing to renal failure. The pathogenesis involves the overproduction of aberrantly glycosylated IgA1, recognized by glycan-specific IgA and IgG autoantibodies, leading to glomerular immune complex deposition and mesangial cell activation. This process triggers cytokine and complement overproduction, resulting in podocyte and tubulointerstitial injury. Clinical progression varies, with 30-40% of patients reaching end-stage renal disease 20-30 years post-diagnosis. Treatment focuses on supportive care to manage symptoms, while the role of immunosuppression, primarily corticosteroids, remains controversial and is generally considered in specific cases or with caution due to potential adverse effects, with mycophenolate mofetil showing promise in certain patient populations.

FILSPARI (sparsentan), developed by Travere Therapeutics/Vifor Pharma, is another landmark introduced in the IgA nephropathy treatment space in February 2023 through an accelerated approval gateway. Sparsentan is a first-in-class, orally active, single-molecule functioning as a high-affinity dual-acting antagonist of ETA and AT1 receptors associated with kidney disease progression. It was granted accelerated approval based on interim results from the ongoing pivotal Phase III PROTECT study.

In a nutshell, only two FDA-approved therapies are available for managing IgA nephropathy. Though recent drug approvals offer new treatment options for IgA nephropathy, high demand for more efficacious and targeted therapies still exists in the IgA nephropathy therapeutic landscape. As per our analysis, it is estimated that the IgA nephropathy treatment space will experience significant changes during the forecast period of 2024-2034, owing to the launch of upcoming novel therapies and a robust pipeline of drugs in development for the management of IgA nephropathy.

Various key players such as Novartis, Visterra (a subsidiary of Otsuka Pharmaceutical), Vera Therapeutics, Ionis Pharmaceuticals/Roche, Vertex Pharmaceuticals/Alpine Immune Sciences, AstraZeneca, Vera Therapeutics, Biogen, and others, are involved in developing therapies for IgA nephropathy.

• In 2023, the total market size of IgA nephropathy was around ~USD 400 million which is expected to increase by 2034 during the study period (2020-2034) in the US.
• During the forecast period (2024-2034), pipeline candidates such as Atrasentan, Sibeprenlimab (VIS-649), Zigakibart (BION-1301), IONIS-FB-LRx/RG6299, Povetacicept, ULTOMIRIS (ravulizumab), Atacicept (VT-001), Felzartamab, and others, are expected to drive the rise in IgA nephropathy market size.
• By 2034, FABHALTA is expected to garner the largest market share in the United States.

Key Updates

• In September 2024, Vera Therapeutics announced it completed enrollment ahead of schedule for the pivotal Phase III ORIGIN 3 trial of atacicept in IgA nephropathy patients. The trial has enrolled 200 participants, and data from this cohort will support the 36-week primary efficacy endpoint for regulatory approval.
• Calliditas Therapeutics expects TARPEYO’s inclusion in updated KDIGO guidelines in 2024. The company expects that it will further drive the acceptance and appropriate use of TARPEYO.
• In April 2024, Novartis announced that the company filed the application using the APPLAUSE-IgA nephropathy study data for accelerated FDA approval and was accepted for priority review.
• At the World Congress of Nephrology 2024, Novartis presented the trial design of the BEYOND trial - a Phase III, randomized, double-blind, placebo-controlled trial of zigakibart in adults with IgA nephropathy.
• In April 2024, Vertex Pharmaceuticals Incorporated and Alpine Immune Sciences announced that the companies have entered into a definitive agreement under which Vertex will acquire Alpine for USD 65 per share or approximately USD 4.9 billion in cash. The transaction was unanimously approved by both the Vertex and Alpine Boards of Directors.

IgA Nephropathy Drugs Uptake

• This section focuses on the uptake rate of potential drugs expected to be launched in the market during 2020-2034. The landscape of IgA nephropathy treatment has experienced a profound transformation with the uptake of novel drugs. These innovative therapies are redefining standards of care. Furthermore, the increased uptake of these transformative drugs is a testament to the unwavering dedication of physicians, oncology professionals, and the entire healthcare community in their tireless pursuit of advancing cancer care. This momentous shift in treatment paradigms is a testament to the power of research, collaboration, and human resilience.
• Given the label expansion and better efficacy results, FINTEPLA is expected to capture the largest market share in the United States with a medium-fast uptake.

IgA Nephropathy Pipeline Development Activities

The report provides insights into therapeutic candidates in Phase III, Phase IIb/III, Phase II, and Phase I. It also analyzes key players involved in developing targeted therapeutics.

Pipeline Development Activities

The report covers information on collaborations, acquisitions and mergers, licensing, and patent details for IgA nephropathy emerging therapy.

KOL Views

To keep up with current market trends, we take KOLs and SMEs’ opinions working in the domain through primary research to fill the data gaps and validate our secondary research. Industry Experts contacted for insights on IgA nephropathy evolving treatment landscape, patient reliance on conventional therapies, patient therapy switching acceptability, and drug uptake, along with challenges related to accessibility, including oncologists, radiation oncologists, surgical oncologists, and others.

The analysts connected with 30+ KOLs to gather insights; however, interviews were conducted with 15+ KOLs in the United States. Centers such as - Washington University, University of Alabama at Birmingham Medical Center, University of Alabama at Birmingham, University of Louisville, Stanford University Medical Center, Loma Linda University, Columbia University Vagelos College of Physicians and Surgeons, etc., were contacted. Their opinion helps understand and validate current and emerging therapy treatment patterns or IgA nephropathy market trends. This will support the clients in potential upcoming novel treatments by identifying the overall scenario of the market and the unmet needs.

Qualitative Analysis

We perform Qualitative and market Intelligence analysis using various approaches, such as SWOT analysis. In the SWOT analysis, strengths, weaknesses, opportunities, and threats in terms of disease diagnosis, patient awareness, patient burden, competitive landscape, cost-effectiveness, and geographical accessibility of therapies are provided. These pointers are based on the Analyst’s discretion and assessment of the patient burden, cost analysis, and existing and evolving treatment landscape.

Market Access and Reimbursement

? Coverage for TARPEYO - The first drug to be approved for IgA nephropathy patients in the USTARPEYO (budesonide) delayed-release capsules are the first and only FDA-approved drug to treat IgA nephropathy. The drug was granted accelerated approval as it would cater to a high unmet need of IgA nephropathy patients at risk of disease progression.

However, the market success of any newly approved therapy is influenced by access to the market. Hence, Calliditas planned commercial readiness from 3Q21. The US geography was divided into approximately 40 sales territories upon FDA approval, and a field force was designed to optimize the reach and frequency of top-tier nephrologists. The national account managers were placed in 3Q 2021. The training materials were finalized, and the product was launched in Q1 of 2022.

To minimize the time from prescription to patient, Calliditas streamlined the marketing channels and announced a Wholesale Acquisition Cost (WAC) of USD 14,160 for a 30-day supply of TARPEYO.

• TARPEYO touchpoints: To assist patients and their healthcare providers who prescribe TARPEYO, Calliditas has launched a comprehensive patient support program - TARPEYO Touchpoints. It is a full-service patient and provider support program designed to accelerate and streamline access to TARPEYO. It utilizes biologics by McKesson’s Pharmacy Elite model integrated hub and exclusive specialty pharmacy under one roof. It is staffed by care navigators and a designated rare pod team (nurses, pharmacists, fulfillment, and distribution team).
• PharmacyElite program: TARPEYO may help address an unmet need for patients with primary IgA nephropathy who currently lack a treatment option designed to target their disease. Biologics by McKesson, an independent specialty pharmacy specializing in oncology and rare disease areas, was selected by Calliditas Therapeutics as the exclusive specialty pharmacy provider for TARPEYO delayed-release capsules. With its innovative PharmacyElite program, Biologics will help patients start on TARPEYO quickly by efficiently managing access, reimbursement, and clinical services. PharmacyElite is powered by a dedicated team focused on removing common points of redundancy and improving outcomes throughout the TARPEYO patient journey.

TARPEYO touchpoints co-pay assistance program

If the patient is commercially insured, they may be eligible to pay as little as USD 0 per prescription.*† Ninety-seven percent of TARPEYO patients have paid less than USD 10 per prescription. Eighty-eight percent of TARPEYO patients have paid USD 0 per prescription.

The private insurance pharmacy and therapeutic (P&T) committees meet either quarterly or monthly to decide on including TARPEYO in their formulary of covered products. Calliditas is actively engaging with these committees, and the second quarter shows a significant improvement in TARPEYO's status, with over 80% of listings now reflecting an enhanced TARPEYO label.
? FILSPARI
Product access and reimbursement are strong with 96% of the US lives having a pathway to FILSPARI reimbursement, and Travere Therapeutics is very pleased with the claims approval rates it is seeing, also reflecting the strong authorization criteria for FILSPARI payer plans and formularies. The company is also driving additional efficiencies in the pull-through process, which is supporting the increasing prescriber base and the growing number of patients initiating therapy having a positive experience with FILSPARI.

Travere TotalCare

Travere TotalCare is a free support program that can provide information and help guide patients through treatment with FILSPARI.

Travere TotalCare offers solutions to make treatment more affordable for patients:

• USD 0 co-pay for eligible commercially insured patients.*
• Free, at-home REMS lab testing for qualified patients.
• If the patient does not have insurance or if the insurance does not cover enough for FILSPARI, additional support may be available.

*Co-pay terms and conditions:

• The program is only valid for patients with commercial or private insurance.
• Must be a US resident.
• Not valid for patients insured by a federal or state government-funded health plan, including Medicare, Medicare Advantage, Medicaid, and TRICARE.
• Void where the program is prohibited by law.
• Not valid for uninsured patients.
• The program does not replace prescription drug coverage or insurance and is not intended to substitute for coverage.

Travere Therapeutics reserves the right to terminate or modify this program at any time without notice.

Scope of the Report

• The report covers a segment of key events, an executive summary, and a descriptive overview of IgA nephropathy, explaining its causes, signs, symptoms, pathogenesis, and currently used therapies.
• Comprehensive insight into the epidemiology segments and forecasts, disease progression, and treatment guidelines has been provided.
• Additionally, an all-inclusive account of the emerging therapies and the elaborative profiles of late-stage and prominent therapies will impact the current treatment landscape.
• A detailed review of the IgA nephropathy market, historical and forecasted market size, market share by therapies, detailed assumptions, and rationale behind our approach is included in the report, covering the United States drug outreach.
• The report provides an edge while developing business strategies, by understanding trends, through SWOT analysis and expert insights/KOL views, patient journey, and treatment preferences that help shape and drive the United States IgA nephropathy market.

IgA Nephropathy Report Insights

• Patient Population
• Therapeutic Approaches
• IgA nephropathy Market Size and Trends
• Existing Market Opportunity

IgA Nephropathy Report Key Strengths

• Eleven-year Forecast
• The United States Coverage
• IgA nephropathy Epidemiology Segmentation
• Key Cross Competition

IgA Nephropathy Report Assessment

• Current Treatment Practices
• Reimbursements
• Market Attractiveness
• Qualitative Analysis (SWOT, Conjoint Analysis, Unmet needs)

FAQs

• Would there be any changes observed in the current treatment approach?
• Will there be any improvements in IgA nephropathy management recommendations?
• Would research and development advances pave the way for future tests and therapies for IgA nephropathy?
• Would the diagnostic testing space experience a significant impact and lead to a positive shift in the treatment landscape of IgA nephropathy?
• What kind of uptake will the new therapies witness in the coming years in IgA nephropathy patients?

Reasons to Buy

• The report will help develop business strategies by understanding the latest trends and changing treatment dynamics driving the IgA nephropathy market.
• Insights on patient burden/disease prevalence, evolution in diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years.
• Understand the existing market opportunities in varying geographies and the growth potential over the coming years.
• Distribution of historical and current patient share based on real-world prescription data along with reported sales of approved products in the United States.
• Identifying strong upcoming players in the market will help devise strategies to help get ahead of competitors.
• Detailed analysis and ranking of class-wise potential current and emerging therapies under the Analyst view section to provide visibility around leading classes.
• Highlights of access and reimbursement policies of current therapies, barriers to accessibility of expensive off-label therapies, and patient assistance programs.
• To understand Key Opinion Leaders’ perspectives around the accessibility, acceptability, and compliance-related challenges of existing treatment to overcome barriers in the future.
• Detailed insights on the unmet needs of the existing market so that the upcoming players can strengthen their development and launch strategy.