Pulmonary Hypertension Due to Lung DiseasesAirFlyNCT07478094
Longitudinal Outcomes of Patients With Group 3 Pulmonary Hypertension Treated With Iloprost
An observational study of Pulmonary Hypertension Due to Lung Diseases, run by University of Sao Paulo General Hospital at 1 site in Brazil.
Trial summary
This observational real-world study aims to evaluate the longitudinal clinical outcomes of adult patients with Group 3 pulmonary hypertension (PH), associated with chronic lung diseases such as interstitial lung disease, who are treated with inhaled iloprost in routine clinical practice. Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Pulmonary Hypertension Due to Lung Diseases
- How many people
- 72 participants
- Where
- 1 siteBrazil
- Study dates
- 1 May 2026to 1 Jun 2026
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Study population: Adults with pulmonary hypertension due to chronic lung disease and/or hypoxia (group 3 pulmonary hypertension) receiving care at a specialized pulmonary circulation outpatient clinic. The study population includes patients with underlying interstitial lung disease or chronic obstructive pulmonary disease who were treated with inhaled iloprost as part of routine clinical practice. The cohort represents a real-world population followed longitudinally using data available from medical records, without additional study-specific procedures or patient contact.
Who may be able to join
- Age ≥18 years
- Diagnosis of group 3 pulmonary hypertension confirmed by right heart catheterization, defined as mean pulmonary artery pressure >20 mmHg, pulmonary artery wedge pressure ≤15 mmHg, and pulmonary vascular resistance >2 Wood units
- Presence of documented underlying chronic lung disease (e.g., interstitial lung disease confirmed by high-resolution computed tomography with restrictive pattern on pulmonary function testing, or chronic obstructive pulmonary disease)
- Use of inhaled iloprost for a minimum of 3 months, documented in medical records
- Availability of baseline clinical data (at initiation of inhaled iloprost) and at least one follow-up assessment
Who cannot join
- Pulmonary hypertension from other clinical groups (Groups 1, 2, 4, or 5) as the predominant diagnosis
- Insufficient clinical information in medical records to allow characterization or longitudinal assessment
Trial locations and contacts
1 site in 1 country.
Brazil1 site
Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo
São Paulo, São Paulo, 05403-900
About this study
Official title: AirFly: Longitudinal Outcomes of Patients With Group 3 Pulmonary Hypertension Treated With Iloprost
This observational real-world study aims to evaluate the longitudinal clinical outcomes of adult patients with Group 3 pulmonary hypertension (PH), associated with chronic lung diseases such as interstitial lung disease, who are treated with inhaled iloprost in routine clinical practice.
Treatment options for Group 3 PH remain limited, and the use of pulmonary vasodilators is controversial due to potential worsening of gas exchange. Inhaled iloprost may provide a more selective approach by targeting well-ventilated lung regions. However, real-world data on its use in this population are scarce.
Read more
The primary objective of this study is to assess changes in clinical risk status over time using the COMPERA 2.0 four-stratum risk model. Secondary objectives include describing patient characteristics, treatment patterns, and the evolution of functional parameters and biomarkers, as well as documenting relevant clinical events such as hospitalizations, treatment escalation, and discontinuation.
The study will retrospectively analyze data from approximately 50 adult patients with confirmed Group 3 PH who received inhaled iloprost as part of their routine care in a specialized pulmonary hypertension center. No additional patient contact or interventions will occur.
Read the detailed description
Group 3 pulmonary hypertension (PH), associated with chronic lung diseases such as interstitial lung disease, represents a common and clinically challenging PH phenotype, frequently associated with reduced exercise capacity, increased oxygen requirements, and higher mortality. Unlike pulmonary arterial hypertension (Group 1), where targeted therapies have demonstrated clear benefits, treatment options for Group 3 PH remain limited and are primarily focused on management of the underlying lung disease and supportive measures.
The use of systemic pulmonary vasodilators in this population is controversial due to the potential risk of worsening ventilation-perfusion mismatch and hypoxemia. Inhaled prostacyclin analogues may offer a more selective therapeutic approach by preferentially targeting well-ventilated lung regions, potentially mitigating these risks. Inhaled iloprost, approved for pulmonary arterial hypertension, has been used off-label in selected patients with Group 3 PH; however, available evidence is limited and largely derived from small or short-term studies. Data describing real-world patient profiles, treatment patterns, and longitudinal clinical outcomes remain scarce.
Risk stratification has become a key component of disease monitoring in pulmonary hypertension. The COMPERA 2.0 model, which incorporates clinical and non-invasive parameters into a four-stratum risk framework, has demonstrated sensitivity to detect prognostically meaningful changes over time.
This retrospective observational study aims to characterize the clinical evolution of patients with Group 3 PH treated with inhaled iloprost in routine practice. The primary focus is the longitudinal change in risk status according to the COMPERA 2.0 four-stratum model. In addition, the study will explore changes in functional parameters such as WHO functional class and six-minute walk distance, biomarker trends when available, patterns of iloprost use (including monotherapy or combination therapy), and the occurrence of relevant clinical events such as hospitalizations, treatment escalation, and treatment discontinuation.
Data will be obtained from existing medical records of patients followed in a specialized pulmonary hypertension center, without additional patient contact or intervention.
- Keywords
- Group 3 Pulmonary Hypertension, Interstitial Lung Disease, Inhaled Iloprost, Observational Study
- Medical subject headings
- Lung Diseases, Interstitial
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| Group 3 Pulmonary Hypertension Treated With Inhaled Iloprost | Adults (≥18 years) with group 3 pulmonary hypertension (PH due to chronic lung disease and/or hypoxia) followed at a specialized pulmonary circulation outpatient clinic who received inhaled iloprost during routine clinical care. Group 3 PH was confirmed by right heart catheterization according to current hemodynamic criteria and in the presence of documented underlying lung disease (e.g., interstitial lung disease or chronic obstructive pulmonary disease). |
What is being measured
The primary outcome is the main question the study is designed to answer.
Change in Risk Stratification According to the COMPERA 2.0 Four-Stratum Model
Primary outcome is the longitudinal change in clinical risk category assessed using the COMPERA 2.0 four-stratum model (low, intermediate-low, intermediate-high, high risk). Risk status will be determined based on available clinical variables (World Health Organization functional class, 6-minute walk distance, and BNP or NT-proBNP levels, when available). The analysis will evaluate the proportion of patients who demonstrate improvement, stability, or worsening of risk category during follow-up compared with baseline (defined as initiation of inhaled iloprost).
Measured: From baseline (initiation of inhaled iloprost) through available follow-up assessments during routine clinical care (follow-up of 3 to 6 months)
Secondary outcomes (4)
Change in World Health Organization (WHO) Functional Class
Within-patient longitudinal change in World Health Organization (WHO) functional class during follow-up compared with baseline (defined as initiation of inhaled iloprost). WHO functional class ranges from I (no limitation of physical activity) to IV (inability to carry out any physical activity without symptoms), with higher classes indicating worse functional status.
Measured: From baseline (initiation of inhaled iloprost) through available follow-up assessments during routine clinical care (follow-up of 3 to 6 months)
Change in 6-Minute Walk Distance (6MWD)
Within-patient change in 6-minute walk distance (6MWD), measured in meters, comparing baseline (initiation of inhaled iloprost) and follow-up assessments during routine clinical care. Higher values indicate better exercise capacity.
Measured: From baseline (initiation of inhaled iloprost) through available follow-up assessments during routine clinical care (follow-up of 3 to 6 months)
Change in BNP or NT-proBNP Levels
Within-patient variation in B-type natriuretic peptide (BNP) or N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels during follow-up compared with baseline. BNP and NT-proBNP are biomarkers of cardiac stress; higher values indicate worse cardiac strain and prognosis.
Measured: From baseline (initiation of inhaled iloprost) through available follow-up assessments during routine clinical care (follow-up of 3 to 6 months)
Clinical Events During Follow-up
Occurrence of clinically relevant events during follow-up, including pulmonary hypertension-related hospitalizations, therapeutic escalation, discontinuation of inhaled iloprost (with reason, when available), and all-cause mortality. These events will be descriptively summarized.
Measured: From baseline (initiation of inhaled iloprost) through available follow-up assessments during routine clinical care (follow-up of 3 to 6 months)
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted17 Mar 2026
- Study start1 May 2026
- Primary completion1 Jun 2026
- Study completion1 Jun 2026
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Pulmonary Hypertension Due to Lung DiseasesStudies of the same condition, open and under way.
- More trials run by University of Sao Paulo General HospitalThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Retrospective
- Enrolment
- 72 participants (actual)
Sponsor and investigators
- Lead sponsor
- University of Sao Paulo General Hospital
- Sponsor type
- University, hospital or other
- Responsible party
- Principal investigator, Caio Júlio César dos Santos Fernandes, University of Sao Paulo General Hospital
- Investigators
- Caio Fernandes, Principal investigator, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT07478094
- Sponsor's protocol ID
- SGP 28054
- Acronym
- AirFly
- Status last verified
- Jun 2026
- First submitted
- 27 Feb 2026
- Last updated
- 24 Jun 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- Undecided
Study at a glance
From the registry record, last updated by the sponsor on 24 Jun 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- AirFly: Longitudinal Outcomes of Patients With Group 3 Pulmonary Hypertension Treated With Iloprost
- ClinicalTrials.gov ID
- NCT07478094
- Acronym / sponsor ID
- AirFly / SGP 28054
- Keywords
- Group 3 Pulmonary Hypertension, Interstitial Lung Disease, Inhaled Iloprost, Observational Study
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 72 participants (actual)
- Lead sponsor
- University of Sao Paulo General HospitalUniversity, hospital or other
- Study start
- 1 May 2026 (actual)
- Primary completion
- 1 Jun 2026 (actual)
- Study completion
- 1 Jun 2026 (actual)
- First posted
- 17 Mar 2026 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
This observational real-world study aims to evaluate the longitudinal clinical outcomes of adult patients with Group 3 pulmonary hypertension (PH), associated with chronic lung diseases such as interstitial lung disease, who are treated with inhaled iloprost in routine clinical practice.
Treatment options for Group 3 PH remain limited, and the use of pulmonary vasodilators is controversial due to potential worsening of gas exchange. Inhaled iloprost may provide a more selective approach by targeting well-ventilated lung regions. However, real-world data on its use in this population are scarce.
The primary objective of this study is to assess changes in clinical risk status over time using the COMPERA 2.0 four-stratum risk model. Secondary objectives include describing patient characteristics, treatment patterns, and the evolution of functional parameters and biomarkers, as well as documenting relevant clinical events such as hospitalizations, treatment escalation, and discontinuation.
The study will retrospectively analyze data from approximately 50 adult patients with confirmed Group 3 PH who received inhaled iloprost as part of their routine care in a specialized pulmonary hypertension center. No additional patient contact or interventions will occur.
Detailed description
Group 3 pulmonary hypertension (PH), associated with chronic lung diseases such as interstitial lung disease, represents a common and clinically challenging PH phenotype, frequently associated with reduced exercise capacity, increased oxygen requirements, and higher mortality. Unlike pulmonary arterial hypertension (Group 1), where targeted therapies have demonstrated clear benefits, treatment options for Group 3 PH remain limited and are primarily focused on management of the underlying lung disease and supportive measures.
The use of systemic pulmonary vasodilators in this population is controversial due to the potential risk of worsening ventilation-perfusion mismatch and hypoxemia. Inhaled prostacyclin analogues may offer a more selective therapeutic approach by preferentially targeting well-ventilated lung regions, potentially mitigating these risks. Inhaled iloprost, approved for pulmonary arterial hypertension, has been used off-label in selected patients with Group 3 PH; however, available evidence is limited and largely derived from small or short-term studies. Data describing real-world patient profiles, treatment patterns, and longitudinal clinical outcomes remain scarce.
Risk stratification has become a key component of disease monitoring in pulmonary hypertension. The COMPERA 2.0 model, which incorporates clinical and non-invasive parameters into a four-stratum risk framework, has demonstrated sensitivity to detect prognostically meaningful changes over time.
This retrospective observational study aims to characterize the clinical evolution of patients with Group 3 PH treated with inhaled iloprost in routine practice. The primary focus is the longitudinal change in risk status according to the COMPERA 2.0 four-stratum model. In addition, the study will explore changes in functional parameters such as WHO functional class and six-minute walk distance, biomarker trends when available, patterns of iloprost use (including monotherapy or combination therapy), and the occurrence of relevant clinical events such as hospitalizations, treatment escalation, and treatment discontinuation.
Data will be obtained from existing medical records of patients followed in a specialized pulmonary hypertension center, without additional patient contact or intervention.
Study design
- Observational model
- Cohort
- Time perspective
- Retrospective
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Group 3 Pulmonary Hypertension Treated With Inhaled Iloprost | – | –Adults (≥18 years) with group 3 pulmonary hypertension (PH due to chronic lung disease and/or hypoxia) followed at a specialized pulmonary circulation outpatient clinic who received inhaled iloprost during routine clinical care. Group 3 PH was confirmed by right heart catheterization according to current hemodynamic criteria and in the presence of documented underlying lung disease (e.g., interstitial lung disease or chronic obstructive pulmonary disease). |
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- Adults with pulmonary hypertension due to chronic lung disease and/or hypoxia (group 3 pulmonary hypertension) receiving care at a specialized pulmonary circulation outpatient clinic. The study population includes patients with underlying interstitial lung disease or chronic obstructive pulmonary disease who were treated with inhaled iloprost as part of routine clinical practice. The cohort represents a real-world population followed longitudinally using data available from medical records, without additional study-specific procedures or patient contact.
Key inclusion criteria (5 listed)
- Age ≥18 years
- Diagnosis of group 3 pulmonary hypertension confirmed by right heart catheterization, defined as mean pulmonary artery pressure >20 mmHg, pulmonary artery wedge pressure ≤15 mmHg, and pulmonary vascular resistance >2 Wood units
- Presence of documented underlying chronic lung disease (e.g., interstitial lung disease confirmed by high-resolution computed tomography with restrictive pattern on pulmonary function testing, or chronic obstructive pulmonary disease)
- Use of inhaled iloprost for a minimum of 3 months, documented in medical records
- Availability of baseline clinical data (at initiation of inhaled iloprost) and at least one follow-up assessment
Key exclusion criteria (2 listed)
- Pulmonary hypertension from other clinical groups (Groups 1, 2, 4, or 5) as the predominant diagnosis
- Insufficient clinical information in medical records to allow characterization or longitudinal assessment
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
Change in Risk Stratification According to the COMPERA 2.0 Four-Stratum Model
Time frame: From baseline (initiation of inhaled iloprost) through available follow-up assessments during routine clinical care (follow-up of 3 to 6 months)
Primary outcome is the longitudinal change in clinical risk category assessed using the COMPERA 2.0 four-stratum model (low, intermediate-low, intermediate-high, high risk). Risk status will be determined based on available clinical variables (World Health Organization functional class, 6-minute walk distance, and BNP or NT-proBNP levels, when available). The analysis will evaluate the proportion of patients who demonstrate improvement, stability, or worsening of risk category during follow-up compared with baseline (defined as initiation of inhaled iloprost).
4 secondary outcomes
Change in World Health Organization (WHO) Functional Class
Time frame: From baseline (initiation of inhaled iloprost) through available follow-up assessments during routine clinical care (follow-up of 3 to 6 months)
Change in 6-Minute Walk Distance (6MWD)
Time frame: From baseline (initiation of inhaled iloprost) through available follow-up assessments during routine clinical care (follow-up of 3 to 6 months)
Change in BNP or NT-proBNP Levels
Time frame: From baseline (initiation of inhaled iloprost) through available follow-up assessments during routine clinical care (follow-up of 3 to 6 months)
Clinical Events During Follow-up
Time frame: From baseline (initiation of inhaled iloprost) through available follow-up assessments during routine clinical care (follow-up of 3 to 6 months)
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 24 Jun 2026.
- Individual participant dataSharing planned: Undecided
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Caio Fernandes principal investigator
Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Pulmonary Hypertension Due to Lung Diseases, run by University of Sao Paulo General Hospital at 1 site in Brazil.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 5 inclusion and 2 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 24 Jun 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in Brazil. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Change in Risk Stratification According to the COMPERA 2.0 Four-Stratum Model (time frame: From baseline (initiation of inhaled iloprost) through available follow-up assessments during routine clinical care (follow-up of 3 to 6 months)). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 24 Jun 2026.