NCT07768644

Brief Summary

Pulmonary hypertension (PH) is an uncommon condition and can often be difficult to diagnose. It means elevated blood pressure in the lungs which can rapidly lead to failure of the right side of the heart and is ultimately fatal. Diagnosing PH currently involves a number of scans, breathing tests and often an invasive test called right heart catheterisation \[RHC\] (which involves inserting a tube into a large vein to measure the lung blood pressure from the). The investigators propose using a scan of the nailbeds called "Nailfold (Video) Capillaroscopy" to see whether this measurements correspond to the pressures that the investigators measure at the time of RHC and whether these measures change with treatment for PH. The investigators wish to answer the hypotheses that:

  1. 1.The measurements of the nailbed blood vessels are correlated with the invasive right heart catheter values across all groups of PH
  2. 2.The abnormalities seen in systemic sclerosis are reversible with treatment with pulmonary vasodilator drugs
  3. 3.This change will be significantly more than any change seen in other types of pulmonary hypertension with these drugs
  4. 4.Changes in the nailbed blood vessels with treatment will reflect changes in the usual parameters used to measure treatment response in PH:

Trial Health

77
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
60

participants targeted

Target at P25-P50 for all trials

Timeline
22mo left

Started May 2026

Typical duration for all trials

Geographic Reach
1 country

1 active site

Status
recruiting

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Progress17%
May 2026Aug 2028

First Submitted

Initial submission to the registry

May 20, 2026

Completed
Same day until next milestone

Study Start

First participant enrolled

May 20, 2026

Completed
3 months until next milestone

First Posted

Study publicly available on registry

August 17, 2026

Completed
1.8 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

May 20, 2028

Expected
2 months until next milestone

Study Completion

Last participant's last visit for all outcomes

August 2, 2028

Last Updated

August 17, 2026

Status Verified

August 1, 2026

Enrollment Period

2 years

First QC Date

May 20, 2026

Last Update Submit

August 11, 2026

Conditions

Pulmonary Arterial Hypertension (PAH) (WHO Group 1 PH)Pulmonary Hypertension (World Health Organization Group 3)Chronic Thromboembolic Pulmonary Hypertension

Keywords

Nailfold VideocapillaroscopyPulmonary vasodilatorPulmonary Hypertension

Outcome Measures

Primary Outcomes (1)

  • Linear Capillary Density

    Change in Linear Capillary Density over time on Pulmonary Vasodilator Therapy (in capillary loops/mm)

    4 - 6 months into therapy

Secondary Outcomes (22)

  • NVC Capillary Diameter

    Baseline and first follow up visit (4-6 months)

  • Angular dispersion index

    Baseline and first follow up visit (4-6 months)

  • Overall capillaroscopic pattern

    Baseline and first follow up visit (4-6 months)

  • Pulmonary Vascular Resistance

    Baseline only

  • Mean Pulmonary Arterial Pressure, Pulmonary Artery Systolic Pressure, Right Atrial Pressure

    Baseline only

  • +17 more secondary outcomes

Study Arms (6)

SSc-PAH at diagnosis

Recruited at diagnosis with SSc-spectrum disorder fulfilling either ACR/EULAR 2013 or VEDOSS (very early diagnosis of SSc) criteria.

Drug: Standard of Care therapy

SSc-PAH at follow up

SSc-PAH patient undergoing treatment intensification in clinic. SSc-spectrum disorder fulfilling either ACR/EULAR 2013 or VEDOSS (very early diagnosis of SSc) criteria

Drug: Standard of Care therapy

Non-SSc Group 1 PAH at diagnosis

Patients with Group 1 PAH other than SSc, as determined by National MDT decision recruited at the point of diagnosis

Drug: Standard of Care therapy

Non-SSc Group 1 PAH at follow up

Patients with Group 1 PAH other than SSc, as determined by National MDT decision recruited at the point of intensification of therapy in clinic

Drug: Standard of Care therapy

Group 3 PH at diagnosis

Patients with group 3 PH (made by national MDT decision) recruited at the point of diagnosis

Drug: Standard of Care therapy

Group 4 PH at diagnosis

Patients with group 4 PH (made by national MDT decision), recruited at the point of diagnosis

Drug: Standard of Care therapy

Interventions

Standard of Care therapyDRUG

Pulmonary Vasodilator therapy which may include: * Phosphodiesterase Inhibitors * Prostanoids * Endothelin Receptor Antagonists * Activin Ligand Traps

Group 3 PH at diagnosisGroup 4 PH at diagnosisNon-SSc Group 1 PAH at diagnosisNon-SSc Group 1 PAH at follow upSSc-PAH at diagnosisSSc-PAH at follow up

Eligibility Criteria

Age18 Years+
Sexall
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)
Sampling MethodNon-Probability Sample
Study Population

Spanning the entire pulmonary population of Scotland

You may qualify if:

  • For inpatient enrolment:
  • Suspected diagnosis of group 1,3 or 4 pulmonary hypertension undergoing inpatient assessment at nationally designated centre
  • Age \> 18 years
  • For follow up scanning of those enrolled as an inpatient:
  • Confirmed diagnosis of pulmonary hypertension assessed at nationally designated centre fulfilling criteria for group 1, 3 or 4 disease
  • Commencing PH targeted therapy, specifically one or more of: a phosphodiesterase 5 inhibitor (PDE5i), an ET receptor antagonist (ERA), parenteral prostanoid therapy, selexipag, riociguat, sotatercept.
  • For outpatient enrolment
  • Confirmed diagnosis of pulmonary hypertension assessed at nationally designated centre fulfilling criteria for group 1, 3 or 4 disease
  • Age \> 18 years
  • Escalating PH targeted therapy, specifically one or more of: a phosphodiesterase 5 inhibitor (PDE5i), an ET receptor antagonist (ERA), parenteral prostanoid therapy, selexipag, riociguat, sotatercept.
  • Target of \> 25% of patients recruited at diagnosis and to have an SSc-spectrum disorder fulfilling either ACR/EULAR 2013 or VEDOSS (very early diagnosis of SSc) criteria.
  • Target of \> 25% of patients recruited at diagnosis and to have non-SSc group 1 (PAH) diagnosis (as determined by national MDT decision).

You may not qualify if:

  • Patients with confirmed group 2 or group 5 pulmonary hypertension
  • Patients \< 18 years of age
  • Patients unable to participate in nailbed microscopy (through e.g. amputation, severe contractures or other musculoskeletal issues).
  • (From follow up) Patients not being commenced on pulmonary vasodilator therapy at the point of diagnosis.
  • Patients already on maximal PH therapy
  • Patients lacking capacity to consent to participation
  • Patients who are non-fluent English speakers, where suitable translation cannot be found within a time frame that makes the study feasible.
  • Patients with a life expectancy \< 4 months
  • Patients who have consumed nicotine or caffeine within the last 4 - 6 hours
  • Patients wearing nail polish / varnish/ extensions that preclude adequate nailbed visualisation.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (1)

NHS Golden Jubilee

Clydebank, West Dunbartonshire, G81 4DY, United Kingdom

RECRUITING

Related Publications (9)

  • Moore TL, Vail A, Herrick AL. Assessment of digital vascular structure and function in response to bosentan in patients with systemic sclerosis-related Raynaud's phenomenon. Rheumatology (Oxford). 2007 Feb;46(2):363-4. doi: 10.1093/rheumatology/kel383. Epub 2006 Nov 20. No abstract available.

    PMID: 17116656BACKGROUND

  • Santana-Goncalves M, Zanin-Silva D, Henrique-Neto A, Moraes DA, Kawashima-Vasconcelos MY, Lima-Junior JR, Dias JBE, Bragagnollo V, de Azevedo JTC, Covas DT, Malmegrim KCR, Ramalho L, Oliveira MC. Autologous hematopoietic stem cell transplantation modifies specific aspects of systemic sclerosis-related microvasculopathy. Ther Adv Musculoskelet Dis. 2022 Mar 28;14:1759720X221084845. doi: 10.1177/1759720X221084845. eCollection 2022.

    PMID: 35368373BACKGROUND

  • Bharathi PG, Berks M, Dinsdale G, Murray A, Manning J, Wilkinson S, Cutolo M, Smith V, Herrick AL, Taylor CJ. A deep learning system for quantitative assessment of microvascular abnormalities in nailfold capillary images. Rheumatology (Oxford). 2023 Jun 1;62(6):2325-2329. doi: 10.1093/rheumatology/kead026.

    PMID: 36651676BACKGROUND

  • Bellando-Randone S, Del Galdo F, Lepri G, Minier T, Huscher D, Furst DE, Allanore Y, Distler O, Czirjak L, Bruni C, Guiducci S, Avouac J, Cutolo M, Smith V, Matucci-Cerinic M; Very Early Diagnosis of Systemic Sclerosis collaborators. Progression of patients with Raynaud's phenomenon to systemic sclerosis: a five-year analysis of the European Scleroderma Trial and Research group multicentre, longitudinal registry study for Very Early Diagnosis of Systemic Sclerosis (VEDOSS). Lancet Rheumatol. 2021 Dec;3(12):e834-e843. doi: 10.1016/S2665-9913(21)00244-7.

    PMID: 38287630BACKGROUND

  • Arvanitaki A, Giannakoulas G, Triantafyllidou E, Feloukidis C, Boutou AK, Garyfallos A, Karvounis H, Dimitroulas T. Peripheral microangiopathy in precapillary pulmonary hypertension: a nailfold video capillaroscopy prospective study. Respir Res. 2021 Jan 21;22(1):27. doi: 10.1186/s12931-021-01622-1.

    PMID: 33478514BACKGROUND

  • Hofstee HM, Vonk Noordegraaf A, Voskuyl AE, Dijkmans BA, Postmus PE, Smulders YM, Serne EH. Nailfold capillary density is associated with the presence and severity of pulmonary arterial hypertension in systemic sclerosis. Ann Rheum Dis. 2009 Feb;68(2):191-5. doi: 10.1136/ard.2007.087353. Epub 2008 Mar 28.

    PMID: 18375538BACKGROUND

  • van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Muller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Ellen Csuka M, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55. doi: 10.1136/annrheumdis-2013-204424.

    PMID: 24092682BACKGROUND

  • Denton CP, De Lorenzis E, Roblin E, Goldman N, Alcacer-Pitarch B, Blamont E, Buch MH, Carulli M, Cotton C, Del Galdo F, Derrett-Smith E, Douglas K, Farrington S, Fligelstone K, Gompels L, Griffiths B, Herrick A, Hughes M, Pain C, Pantano G, Pauling JD, Prabu A, O'Donoghue N, Renzoni EA, Royle J, Samaranayaka M, Spierings J, Tynan A, Warburton L, Ong VH. The 2024 British Society for Rheumatology guideline for management of systemic sclerosis. Rheumatology (Oxford). 2024 Nov 1;63(11):2956-2975. doi: 10.1093/rheumatology/keae394.

    PMID: 39255973BACKGROUND

  • Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Muller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumanovics G, Coleiro B, Feierl E, Szucs G, Von Muhlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kotter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Stork J, Morovic-Vergles J, Walker UA. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010 Oct;69(10):1809-15. doi: 10.1136/ard.2009.114264. Epub 2010 Jun 15.

    PMID: 20551155BACKGROUND

MeSH Terms

Conditions

Pulmonary Arterial HypertensionHypertension, Pulmonary

Condition Hierarchy (Ancestors)

Lung DiseasesRespiratory Tract DiseasesHypertensionVascular DiseasesCardiovascular Diseases

Study Officials

  • Alistair C Church, PhD, BSc, MB ChB, FRCP

    University of Glasgow / NHS Greater Glasgow and Clyde

    PRINCIPAL INVESTIGATOR

Central Study Contacts

William J Kerrigan, BA BMBCh MRCP

CONTACT

0141 951 5497w.kerrigan.1@research.gla.ac.uk

Alistair C Church, PhD, BSc, MB ChB, FRCP

CONTACT

0141 951 5497alistair.church@glasgow.ac.uk

Study Design

Study Type
observational
Observational Model
COHORT
Time Perspective
PROSPECTIVE
Sponsor Type
OTHER
Responsible Party
PRINCIPAL INVESTIGATOR
PI Title
PhD Student

Study Record Dates

First Submitted

May 20, 2026

First Posted

August 17, 2026

Study Start

May 20, 2026

Primary Completion (Estimated)

May 20, 2028

Study Completion (Estimated)

August 2, 2028

Last Updated

August 17, 2026

Record last verified: 2026-08

Data Sharing

IPD Sharing
Will share

Digital research data will be archived in the University of Glasgow Enlighten repository for 10 years As well as study specific consent for this project, broad and enduring consent will be sought from participants to facilitate sharing and future use of suitably de-identified and appropriately redacted data for research projects. In the consenting process it will be made clear that failure to provide broad and enduring consent will not preclude participation in the study. Sharing of data would be subject to appropriate research ethics committee approval and data sharing agreements for any future work.

Time Frame
After submission of and defense of my research degree. \~September 2030 for at least 10 years
Access Criteria
As described above

Locations

GB(1)