+353-1-416-8900REST OF WORLD
+44-20-3973-8888REST OF WORLD
1-917-300-0470EAST COAST U.S
1-800-526-8630U.S. (TOLL FREE)

PRINTER FRIENDLY

Pulmonary Arterial Hypertension Market and Forecast Analysis to 2025

  • ID: 3797417
  • Drug Pipelines
  • March 2021
  • Region: Global
  • Datamonitor Healthcare
Disease Overview

Pulmonary arterial hypertension is one of a group of rare and life-threatening diseases collectively known as pulmonary hypertension (PH). Each PH subgroup shares similar pathophysiology, clinical presentation, and therapeutic approaches. The diseases are characterized by abnormal vascular proliferation and remodeling of the small pulmonary arteries and arterioles, vasoconstriction, and in situ thrombosis. This leads to increased pulmonary arterial pressure and localized hypertension, which can eventually result in heart failure.

Report Highlights

The publisher estimates that in 2018, there were approximately 218,500 prevalent cases of pulmonary arterial hypertension (PAH) in individuals of all ages worldwide, and forecasts that number to increase to 241,800 prevalent cases by 2027.

The publisher expects the pulmonary hypertension (PH) market to expand over the forecast period due to the approvals of novel therapies, label expansions into non-PAH subtypes, and FDA clearance of specialist devices. Moreover, the increased number of trials demonstrating improved morbidity/mortality data for marketed brands and a shift to early implementation of triple combination therapy will also drive revenue growth in the PH market.

Current treatments are solely symptomatic and function by targeting three pathways controlling vasodilation, namely the endothelin, nitric oxide, and prostacyclin pathways. For WHO FC II and III treatment-naïve patients, dual combination therapy with endothelin receptor agonists (ERAs) and phosphodiesterase 5 inhibitors (PDE5is) is the first-line treatment strategy, while monotherapy with a prostacyclin is a first-line therapy for WHO FC IV patients, with ERAs and PDE5is positioned as add-ons. Actelion’s TRITON study, which investigated the use of a triple combination of Opsumit (ERA), Adcirca (PDE5i), and Uptravi (prostacyclin) in WHO FC II and III patients, failed to achieve the primary endpoint of an improvement in pulmonary vascular resistance. Notably, there was a numerical improvement of 41% in hospitalization and all-cause death; however, this was not statistically significant. If additional larger trials reveal a significant clinical benefit in disease progression, this will allow prostacyclins to build upon the prevailing trend towards early implementation of polytherapy, which was first established through the AMBITION trial with the dual combination of an ERA and a PDE5i. Additionally, this will markedly expand the prostacyclin class’s potential by encouraging first-line uptake alongside Opsumit, which is a market leader.

Historically, ERAs have been the most commercially lucrative class; however, in 2019, prostacyclins succeeded them, with total sales of $2,675m versus $2,286m for ERAs. The publisher anticipates that the gap between prostacyclins and ERAs will widen further over the forecast period as ERAs are under rising threat from both generics and the increasing trend of prostacyclin use in triple combination therapies.

The PDE5i class, which traditionally held a significant portion of market share, has faced fierce generic competition, with both Adcirca and Revatio being subject to generic erosion since 2018. A loss of market share held by PDE5is, from $817m in 2018 to $301m in 2019, was largely attributable to this generic competition.

The guanylyl cyclase stimulator (GCS) class, which consists of a single asset, Adempas, is holding steady by stealing market share from the PDE5i class; however, this is limited to a minority of patients who respond poorly to PDE5is.

Johnson & Johnson’s two newer marketed brands, Opsumit and Uptravi, are projected to become the highest-selling products during the forecast period. Opsumit is the established market leader, with blockbuster sales of $1,639m in 2020. These two products will experience strong uptake, benefiting from physician familiarity, strong positioning in treatment guidelines, and robust efficacy data to support them. Importantly, Johnson & Johnson has generated efficacy data for morbidity/mortality, broadening the evidence base for its products. Although the results of the TRITON trial were largely negative, the triple combination therapy exhibited a numerical reduction in the risk of first disease progression event. In addition, overall mortality, an exploratory endpoint of the trial, was favorably lower in the triple combination arm, though this did not reach statistical significance. Additional studies are required to provide evidence for improved morbidity/mortality outcomes, and this would enable Uptravi to extend its reach to WHO FC II and III patients.

Despite PAH patients making up only 20% of the PH population, most therapies are targeted at this group, with off-label use of drugs for groups 2, 3, and 5 PH. The high rate of off-label use highlights a critical unmet need for approved therapies in these indications. Increasing label expansions to include group 2-4 PH subtypes is a key area of interest and will be a major driver of growth in the PH market. Market leaders Opsumit and Uptravi are being investigated in clinical trials to potentially support label expansions to chronic thromboembolic pulmonary hypertension (CTEPH). This will bring new treatment options to underserved CTEPH patients who currently only have one approved therapy option, Adempas. United Therapeutics’ application for a label expansion for injectable treprostinil, Tyvaso, to include group 3 PH patients, which will make it the first approved therapy for this subtype, is expected to receive a response in April 2021 from the FDA. Given the scarcity of competitors in non-PAH segments, there is a prominent opportunity for market penetration for these drugs.

The approval of pipeline candidates will diversify the PH market and meet an important unmet need to treat the underlying pathophysiology of the disease rather than just disease symptoms. Of the pipeline candidates in late-stage development, sotatercept, which targets the transforming growth factor beta (TGF-β) pathway to correct the underlying cause of the disease, is the most promising candidate and has been heralded as a game changer.

Other notable pipeline candidates include novel delivery systems such as INOpulse and Trevyent, which are set to revolutionize the delivery of nitric oxide and Remodulin. However, pipeline candidates will have an initially muted effect on the market due to strong physician familiarity with the established drug classes and the static nature of the PH market.
Note: Product cover images may vary from those shown
OVERVIEW

DISEASE BACKGROUND
  • Definition
  • Etiology
  • Symptoms
  • Diagnosis and testing
  • Risk factors
  • Patient segmentation
  • Genetics
TREATMENT
  • Drug classes
  • Treatment guidelines
EPIDEMIOLOGY
  • Prevalence methodology
MARKETED DRUGS

PIPELINE DRUGS

KEY REGULATORY EVENTS
  • Keeping Track Of US FDA Decisions: Approvals For Ongentys And New Keytruda, Darzalex Dosing, But Not For Trevyent
  • Keeping Track: US FDA Approves Koselugo, Bestows BTDs on Bavencio, Sotatercept
PROBABILITY OF SUCCESS

LICENSING AND ASSET ACQUISITION DEALS
  • United Therapeutics Buys Y-mAbs' Priority Review Voucher
  • Moderna Expands Horizons With Vertex and Chiesi Deals
  • Vectura Signs Agreement With Aerami Therapeutics
CLINICAL TRIAL LANDSCAPE
  • Sponsors by status
  • Sponsors by phase
  • Recent events
DRUG ASSESSMENT MODEL
  • ERAs
  • PDE5 inhibitors
  • Guanyl cyclase stimulator
  • Prostacyclin analogs and agonists
  • Pipeline candidates
MARKET DYNAMICS

FUTURE TRENDS
  • The PH market will continue to grow due to label expansions into non-PAH subtypes
  • Positive data on morbidity/mortality endpoints will increase uptake of drugs
  • Prostacyclins will continue to be the most lucrative drug class
  • Johnson & Johnson will boast both market leaders
  • Pipeline candidates will diversify the market and introduce improved delivery systems
CONSENSUS FORECASTS

RECENT EVENTS AND ANALYST OPINION
  • INS-1009 for PAH and PH (February 19, 2021)
  • Sotatercept for PAH and PH (November 13, 2020)
  • RT234 for PAH and PH (October 16, 2020)
  • Sotatercept for PAH and PH (June 24, 2020)
  • Simdax for PAH and PH (June 2, 2020)
  • Tyvaso for PAH and PH (February 24, 2020)
  • Sotatercept for PAH and PH (January 27, 2020)
KEY UPCOMING EVENTS

UNMET NEEDS

BIBLIOGRAPHY

Prescription information

APPENDIX

LIST OF FIGURES
Figure 1: Trends in prevalent cases of pulmonary arterial hypertension, 2018–27
Figure 2: Overview of pipeline drugs for PAH and PH in the US
Figure 3: Pipeline drugs for PAH and PH, by company
Figure 4: Pipeline drugs for PAH and PH, by drug type
Figure 5: Pipeline drugs for PAH and PH, by classification
Figure 6: Probability of success in the PAH and PH pipeline
Figure 7: Clinical trials in hypertension
Figure 8: Top 10 drugs for clinical trials in hypertension
Figure 9: Top 10 companies for clinical trials in hypertension
Figure 10: Trial locations in hypertension
Figure 11: Hypertension trials status
Figure 12: Hypertension trials sponsors, by phase
Figure 13: The publisher’s drug assessment summary for pulmonary hypertension
Figure 14: Market dynamics in pulmonary hypertension
Figure 15: Future trends in pulmonary hypertension
Figure 16: Sotatercept for PAH and PH (November 13, 2020): Phase II - SPECTRA / PULSAR
Figure 17: RT234 for PAH and PH (October 16, 2020): Phase IIa - CL201 (Australia)
Figure 18: Sotatercept for PAH and PH (June 24, 2020): Phase II - PULSAR
Figure 19: Simdax for PAH and PH (June 2, 2020): Phase II - HELP (PH-HFpEF)
Figure 20: Tyvaso for PAH and PH (February 24, 2020): Phase II/III - INCREASE (ILD Patients)
Figure 21: Sotatercept for PAH and PH (January 27, 2020): Phase II - PULSAR
Figure 22: Key upcoming events in PAH and PH

LIST OF TABLES
Table 1: Pulmonary hypertension clinical classification groups
Table 2: Pulmonary hypertension clinical classification sub-groups
Table 3: Classification groups by genetic influences
Table 4: Pulmonary hypertension drug classes
Table 5: 2019 ACCP guidelines
Table 6: 2015 ESC/ERA guidelines
Table 7: Prevalent cases of pulmonary arterial hypertension, 2018–27
Table 8: Prevalent cases of pulmonary arterial hypertension, by subtype
Table 9: Prevalent cases of pulmonary arterial hypertension, by NYHA functional class
Table 10: Marketed drugs for PAH and PH
Table 11: Pipeline drugs for PAH and PH in the US
Table 12: Historical global sales, by drug ($m), 2015–19
Table 13: Forecasted global sales, by drug ($m), 2021–25
Table 14: INS-1009 for PAH and PH (February 19, 2021)
Table 15: Sotatercept for PAH and PH (November 13, 2020)
Table 16: RT234 for PAH and PH (October 16, 2020)
Table 17: Sotatercept for PAH and PH (June 24, 2020)
Table 18: Simdax for PAH and PH (June 2, 2020)
Table 19: Tyvaso for PAH and PH (February 24, 2020)
Table 20: Sotatercept for PAH and PH (January 27, 2020)
Note: Product cover images may vary from those shown
Adroll
adroll