Pemphigus Vulgaris (PV)-Market Insights, Epidemiology, and Market Forecast–2030

DelveInsight’s ‘Pemphigus Vulgaris (PV)-Market Insights, Epidemiology, and Market Forecast–2030’ report deliver an in-depth understanding of the PV, historical and forecasted epidemiology as well as the PV market trends in the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom) and Japan.
The PV market report provides current treatment practices, emerging drugs, and market share of the individual therapies, current and forecasted 7MM PV market size from 2017 to 2030. The Report also covers current PV treatment practice/algorithm, market drivers, market barriers, unmet medical needs, SWOT analysis to curate the best of the opportunities and assesses the underlying potential of the market.
Geography Covered
• The United States
• EU5 (Germany, France, Italy, Spain, and the United Kingdom)
• Japan

Study Period: 2017–2030
Pemphigus Vulgaris (PV) Disease Understanding and Treatment Algorithm
Pemphigus Vulgaris (PV) Overview
Pemphigus Vulgaris (PV) is defined as a potentially fatal, autoimmune and intraepithelial disease characterized by flaccid blisters, and erosions of the skin and mucous membrane. It is histologically marked by acantholysis and is mediated by circulating desmoglein-reactive autoantibodies, directed against keratinocyte cell surfaces.
PV is subdivided into two subtypes: mucosal-dominant type and mucocutaneous type. The mucosal-dominant type of PV shows blisters mainly and erosive lesions on the oral mucosa while mucocutaneous type also shows flaccid bullae and intractable erosions of the skin. PV is further segmented based on the severity of the disease as mild, moderate and severe.
Pemphigus Vulgaris (PV) Diagnosis
Generally, patients with unexplained chronic mucosal ulceration, particularly with bullous skin lesions, have been suspected of having PV. It is essential to differentiate PV from other bullous dermatoses. Two clinical findings that are specific for PV include the Nikolsky sign and Asboe-Hansen sign. The diagnosis of PV is confirmed by biopsy of lesion and surrounding (perilesional) normal skin. Immunofluorescence testing shows IgG autoantibodies against the keratinocyte's cell surface. Serum autoantibodies to desmoglein 1 and desmoglein 3 transmembrane glycoproteins can be identified via direct immunofluorescence, indirect immunofluorescence, and enzyme-linked immunosorbent assay (ELISA).
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Pemphigus Vulgaris (PV) Treatment
There is currently no cure for the treatment of PV, but treatment can help keep the symptoms under control. The majority of the therapies aim to improve the symptoms associated with pemphigus by the reduction of serum autoantibodies, either directly or through generalized immune suppression. The goal of the initial phase of therapy is disease control that means preventing the formation of new blisters and starting the healing process of the existing blisters. This initial phase ends when there is no blister appearing for 2 weeks, and the existing lesions have been healed.
The first line of treatment during the initial phase of therapy is corticosteroids (prednisolone)., owing to their rapid effect (within days). Completed remission with minimal treatment needs months while complete treatment remission off treatment requires several months or years of therapy. Systemic corticosteroids can also be used in combination with immunosuppressive agents (Azathioprine, Mycophenolate mofetil, Rituximab), in the cases where there are expected complications of hypertension, diabetes mellitus, or osteoporosis due to prolonged use (>4 months) of the steroids.
The second line of treatment in the cases of contraindications to glucocorticosteroids or complications due to prolonged use consists of the combined or single use of immunosuppressants such as azathioprine, mycophenolate mofetil (MMF), dapsone, methotrexate, cyclophosphamide, and cyclosporine.
Other treatment options include intravenous immunoglobulins (IVIG), immunoadsorption, plasmapheresis, and extracorporeal photochemotherapy.
Pemphigus Vulgaris (PV) Epidemiology
The disease epidemiology covered in the report provides historical as well as forecasted epidemiology segmented by Total Diagnosed Prevalent Population of PV, Gender-specific Diagnosed Prevalent Population of PV, Severity-specific Diagnosed Prevalent Population of PV, and Age-specific Diagnosed Prevalent Population of PV scenario of PV in the 7MM covering the United States, EU5 countries (Germany, France, Italy, Spain, and the United Kingdom) and Japan from 2017 to 2030.
Key Findings
• The increasing geriatric population and rising prevalence of PV generates a large patient population in the 7MM. In 2017, the diagnosed prevalent population of PV in the 7MM was 41,217.
• The United States contributes the largest share with 13,526 cases in 2017 acquiring near about 32.81% of the totals 7MM share. On the other hand, EU5 and Japan account for 60.10% and 7.09% diagnosed prevalent population share respectively in 2017.
• A female predominance in PV is well established. However, the genetic and physiological basis for this gender bias is not well understood. In 2017, males and female contribute to 5,275 and 8,251 diagnosed prevalent cases in the US.
• DelveInsight’s epidemiology model for PV estimates that out of the total diagnosed prevalent population of 13,526 cases in the US for PV in 2017, 5,814 cases are of mild, 3,678 cases are of moderate, and 4,034 accounted for severe cases, respectively.
• The majority of the PV cases were found in adults between 60–70 years of age. In 2017, 3,014 cases, 2,449 cases, were contributed by patients age 60–69 years, 70–79 years, and lowest cases observed in the age-group of 0–17 years (406 cases) in 2017, respectively in the US.
Pemphigus Vulgaris (PV) Epidemiology
The epidemiology segment also provides the PV epidemiology data and findings across the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom) and Japan.
Pemphigus Vulgaris (PV) Drug Chapters
The drug chapter segment of the PV report encloses the detailed analysis of PV marketed drugs, mid-phase, and late-stage pipeline drugs. It also helps to understand the PV clinical trial details, expressive pharmacological action, agreements and collaborations, approval and patent details of each included drug and the latest news and press releases.
Pemphigus Vulgaris (PV) Marketed Drugs
Mabthera/Rituxan: Genentech/Roche Group
MabThera (Rituxan in the US) in combination with methotrexate is indicated for the treatment of adults with severe active rheumatoid arthritis (RA) who has had an inadequate response or intolerance to other disease-modifying antirheumatic drugs (DMARD) including one or more tumor necrosis factor (TNF) inhibitor therapies.
Rituxan is the first biologic therapy approved by the FDA for PV and the first major advancement in the treatment of the disease in more than 60 years. Also, the FDA has granted Priority Review, Breakthrough Therapy Designation, and Orphan Drug Designation to Rituxan for the treatment of PV.
Products detail in the report…
Pemphigus Vulgaris (PV) Emerging Drugs
Rilzabrutinib (PRN1008): Principia Biopharma
Rilzabrutinib/PRN1008 is being evaluated in patients with pemphigus. It is an oral, reversible covalent BTK inhibitor. This investigational, small molecule therapy acts by blocking BTK (Bruton’s Tyrosine Kinase—a component of B-cell signaling). Principia believes Rilzabrutinib can potentially lead to positive outcomes in IgG4-RD by impacting many of the driving features of the disease, including inflammation, allergic components (IgE and eosinophils), monocytes, macrophages (involved in fibrosis), and B-cells implicated in initiation and maintenance of disease.
Principia has an ongoing phase III clinical trial in patients with moderate to severe pemphigus. It has completed a phase II part A trial in patients with pemphigus and, in conjunction with this trial, the company has an ongoing phase II part B trial to increase the active treatment period from 3 to 6 months. Between the phase II parts A and B of the pemphigus trial, the company has been able to observe consistent safety and efficacy data in approximately 40 pemphigus patients
Products detail in the report…
Ianalumab (VAY736): Novartis Pharmaceuticals/MorphoSys
Ianalumab (VAY736) is a human IgG1/κ mAb designed to target human BAFF-R and to competitively inhibit binding of BAFF to BAFF-R, thereby blocking BAFF-R-mediated signaling in B cells. Also, Ianalumab was engineered to effectively eliminate B cells from circulation in vivo by antibody-dependent cellular cytotoxicity (ADCC). ADCC activity of Ianalumab is greatly enhanced by the elimination of fucose residues from the carbohydrate moiety attached to the Fc part of the antibody. Accordingly, Ianalumab shows potent ADCC activity in vitro with an EC50 of 2.0 pM.
Thus, Ianalumab eliminates BAFF-R + mature and immature B cells via dual mechanisms: antibody-dependent cytotoxicity (ADCC) and induction of B cell apoptosis by blocking BAFF: BAFF-R interaction and downstream survival pathway in B cells. BAFF-R expression is limited to immature and mature B cells up to the lymphoblast stage. Thus, earlier stage pro-B and pre-B cells are not directly affected by Ianalumab. MorphoSys and Novartis are investigating the molecule for the treatment of PV. The molecule has completed a phase II clinical study for PV.
Products detail in the report…
Efgartigimod (ARGX-113): Argenx BVBA
Efgartigimod (ARGX-113) is an investigational therapy for IgG-mediated autoimmune diseases and was designed to exploit the natural interaction between IgG antibodies and the recycling receptor FcRn. The company has designed Efgartigimod to degrade circulating disease-causing autoimmune antibodies and has potential in many large and orphan indications namely multiple sclerosis, PV, systemic lupus erythematosus, myasthenia gravis, and skin blistering diseases. Efgartigimod is currently being tested in Phase II clinical trials for PV.
Products detail in the report…
List to be continued in the report…
Pemphigus Vulgaris (PV) Market Outlook
Treatment of the disease is difficult and sometimes unsafe. For decades, the mainstay of treatment has been glucocorticoids, followed by other drugs.
Management of acute attacks and original management of the disease are enabled with systemic glucocorticoids such as prednisolone, 1–1.5 mg/kg/day PO. The idea is to bring the patient into remission with steroids, using the smallest dosage possible; the reason for the smallest possible dosages is to avoid the possible comorbidities, such as Cushing’s disease, osteoporosis, hypertension, and diabetes. As the patient begins to take systemic glucocorticoids, they can also be prescribed an adjunct treatment protocol with nonsteroidal but immunosuppressant drugs such as azathioprine and mycophenolate.
Many patients go on to have recalcitrant PV after long periods of successful treatment with the medications mentioned above. A vast majority do achieve remission for at least 6 months if they have been treated with glucocorticoids and adjunct medications for close to 10 years; however, around 20–40% of patients do not ever go into remission and are considered refractory. Rituximab can curb the disease, especially in refractory cases. Rituxan (rituximab) was approved by the US FDA for the treatment of adults with moderate to severe PV in July 2018. In 2019, it was also approved in the EU as MabThera for the treatment of moderate to severe PV.
Cyclophosphamide is also used in refractory cases, but it has an unsafe side effect profile. It can cause hemorrhagic cystitis. Because of its safety profile, it requires regular monitoring by the patient, which is an inconvenience. Plasmapheresis is also used occasionally by nonspecifically removing proteins from the blood, but it is costly and has its unfavorable side effects, depending on the patient’s condition. Also, intravenous immunoglobulins can be used to dilute the autoantibodies with regular immunoglobulin.
According to DelveInsight, PV seven major market is expected to change in the study period 2017–2030.
Key Findings
• The market size of PV in the 7MM was found to be USD 181.78 million in 2017.
• The United States accounts for the highest market size of PV, in comparison to the other major markets i.e., EU5 countries, and Japan
• Among the EU5 countries, Germany had the highest market size with USD 26.24 million in 2017, while France had the lowest market size of PV with USD 8.75 million in 2017.
• The market size of PV in Japan was USD 10.23 million in 2017.
• The upcoming therapies Ianalumab (VAY736) and Efgartigimod (ARGX-113) are anticipated to reach the US market by 2024, and Rilzabrutinib (PRN1008), is expected to enter the US market in year 2023.
The United States Market Outlook
This section provides the total PV market size and; market size by therapies in the United States.
EU-5 Market Outlook
The total PV market size and market size by therapies in Germany, France, Italy, Spain, and the United Kingdom are provided in this section.
Japan Market Outlook
The total PV market size and market size by therapies in Japan are provided.
Pemphigus Vulgaris (PV) Drugs Uptake
This section focusses on the rate of uptake of the potential drugs recently launched in the PV market or expected to get launched in the market during the study period 2017–2030. The analysis covers PV market uptake by drugs; patient uptake by therapies; and sales of each drug.
This helps in understanding the drugs with the most rapid uptake, reasons behind the maximal use of new drugs and allows the comparison of the drugs based on market share and size which again will be useful in investigating factors important in market uptake and in making financial and regulatory decisions.
Pemphigus Vulgaris (PV) Development Activities
The report provides insights into different therapeutic candidates in phase II, and phase III stage. It also analyzes key players involved in developing targeted therapeutics.
Pipeline Development Activities
The report covers the detailed information of collaborations, acquisition, and merger, licensing and patent details for PV emerging therapies.
Reimbursement Scenario in Pemphigus Vulgaris (PV)
The US Centers for Medicare & Medicaid Services has assigned J code ‘J9312’ for Rituxan which is effective from effective January 1, 2019. This coding information assists the patients to complete the payer forms for Rituxan. The J codes are used by physicians, hospitals and other healthcare professionals for billing for Medicare. Rituxan is covered under Medicare Part B and Medicare Advantage plan. Patients with Medicare Advantage plan will be covered by Medicare Part B. Also some Medicare Advantage plans include coverage for other prescription drugs that may not be covered by Original Medicare (MedicareAdvantage.com, n.d.). Medicare coverage of Rituxan can vary. If the treatment is covered by Medicare Part B, patients have to pay the Part B coinsurance or copayment (20% of the Medicare-approved amount) after they meet the Part B deductible, which was USD 185 per year in 2019.
As per the International Pemphigus Pemphigoid Foundation (IPPF) registry, 22% of pemphigoid and pemphigus patients have Medicare coverage, 61% commercial insurance, and 17% other insurance (National Organization for Rare Disorders, 2019).
In January 2020, the National Authority for Health (Haute Autorité de Santé - HAS) in France has given a favorable opinion to reimburse MabThera for the treatment of patients with moderate to severe PV (Haute Autorité de Santé, 2020).
Competitive Intelligence Analysis
We perform competitive and market Intelligence analysis of the PV market by using various competitive intelligence tools that include–SWOT analysis, PESTLE analysis, Porter’s five forces, BCG Matrix, Market entry strategies, etc. The inclusion of the analysis entirely depends upon the data availability.
Scope of the Report
• The report covers the descriptive overview of PV, explaining its causes, signs and symptoms, pathophysiology and currently available therapies.
• Comprehensive insight has been provided into the PV epidemiology and treatment.
• Additionally, an all-inclusive account of both the current and emerging therapies for PV is provided, along with the assessment of new therapies, which will have an impact on the current treatment landscape.
• A detailed review of the PV market; historical and forecasted is included in the report, covering the 7MM drug outreach.
• The report provides an edge while developing business strategies, by understanding trends shaping and driving the 7MM PV market.
Report Highlights
• The increasing prevalence of the disease along with promising emerging pipeline therapies will positively drive the PV market.
• The companies and academics are working to assess challenges and seek opportunities that could influence PV R&D.
• Major players are involved in developing therapies for PV. Launch of emerging therapies will significantly impact the PV market.
• Our in-depth analysis of the pipeline assets across different stages of development (phase III and phase II), different emerging trends and comparative analysis of pipeline products with detailed clinical profiles, key cross-competition, launch date along with product development activities will support the clients in the decision-making process regarding their therapeutic portfolio by identifying the overall scenario of the research and development activities.
Pemphigus Vulgaris (PV) Report Insights
• Patient Population
• Therapeutic Approaches
• PV Pipeline Analysis
• PV Market Size and Trends
• Market Opportunities
• Impact of Upcoming Therapies
Pemphigus Vulgaris (PV) Report Key Strengths
• Eleven Years Forecast
• 7MM Coverage
• PV Epidemiology Segmentation
• Key Cross Competition
• Highly Analyzed Market
• Drugs Uptake
Pemphigus Vulgaris (PV) Report Assessment
• Current Treatment Practices
• Unmet Needs
• Pipeline Product Profiles
• Market Attractiveness
• Market Drivers and Barriers
• SWOT Analysis
• Market Access and Reimbursement
Key Questions
Market Insights:
• What was the PV market share (%) distribution in 2017 and how it would look like in 2030?
• What would be the PV total market size as well as market size by therapies across the 7MM during the forecast period (2020–2030)?
• What are the key findings pertaining to the market across the 7MM and which country will have the largest PV market size during the forecast period (2020–2030)?
• At what CAGR, the PV market is expected to grow at the 7MM level during the forecast period (2020–2030)?
• What would be the PV market outlook across the 7MM during the forecast period (2020–2030)?
• What would be the PV market growth till 2030 and what will be the resultant market size in the year 2030?
• How would the market drivers, barriers and future opportunities affect the market dynamics and subsequent analysis of the associated trends?
Epidemiology Insights:
• What is the disease risk, burden and unmet needs of PV?
• What is the historical PV patient pool in the United States, EU5 (Germany, France, Italy, Spain, and the UK) and Japan?
• What would be the forecasted patient pool of PV at the 7MM level?
• What will be the growth opportunities across the 7MM with respect to the patient population pertaining to PV?
• Out of the above-mentioned countries, which country would have the highest prevalent population of PV during the forecast period (2020–2030)?
• At what CAGR the population is expected to grow across the 7MM during the forecast period (2020–2030)?
Current Treatment Scenario, Marketed Drugs, and Emerging Therapies:
• What are the current options for the treatment of PV along with the approved therapy?
• What are the current treatment guidelines for the treatment of PV in the US, Europe and Japan?
• What are the PV marketed drugs and their MOA, regulatory milestones, product development activities, advantages, disadvantages, safety, and efficacy, etc.?
• How many companies are developing therapies for the treatment of PV?
• How many emerging therapies are in the mid-stage and late stages of development for the treatment of PV?
• What are the key collaborations (Industry–Industry, Industry-Academia), Mergers and acquisitions, licensing activities related to the PV therapies?
• What are the recent novel therapies, targets, mechanisms of action and technologies developed to overcome the limitation of existing therapies?
• What are the clinical studies going on for PV and their status?
• What are the key designations that have been granted for the emerging therapies for PV?
• What are the 7MM historical and forecasted market of PV?
Reasons to buy
• The report will help in developing business strategies by understanding trends shaping and driving the PV.
• To understand the future market competition in the PV market and Insightful review of the key market drivers and barriers.
• Organize sales and marketing efforts by identifying the best opportunities for PV in the US, Europe (Germany, Spain, Italy, France, and the United Kingdom) and Japan.
• Identification of strong upcoming players in the market will help in devising strategies that will help in getting ahead of competitors.
• Organize sales and marketing efforts by identifying the best opportunities for the PV market.
• To understand the future market competition in the PV market.