High blood pressure in the lungs in patients with systemic sclerosis: a screening for diagnosis and follow-up to see how it behaves
| ISRCTN | ISRCTN72968188 |
|---|---|
| DOI | https://doi.org/10.1186/ISRCTN72968188 |
| Secondary identifying numbers | CAAE16739013.4.0000.5259 |
- Submission date
- 07/07/2021
- Registration date
- 08/07/2021
- Last edited
- 18/08/2023
- Recruitment status
- No longer recruiting
- Overall study status
- Completed
- Condition category
- Skin and Connective Tissue Diseases
Plain English summary of protocol
Background and study aims
Systemic sclerosis (SSc) is a rare autoimmune disease, caused by the immune system attacking the connective tissue under the skin and around internal organs and blood vessels. This causes scarring and thickening of the tissue in these areas.
Pulmonary hypertension is high blood pressure in the blood vessels that supply the lungs (pulmonary arteries). It's a serious condition that can damage the right side of the heart. The walls of the pulmonary arteries become thick and stiff, and cannot expand as well to allow blood through.
Pulmonary hypertension is the leading cause of death in systemic sclerosis (SSc) and affects up to 12% of all patients with SSc.
The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population.
Who can participate?
Patients aged 18 years and above with SSc.
What does the study involve?
The study involves the performance of a systematic screening procedure for the diagnosis of systemic sclerosis-related pulmonary hypertension. Then, the study performed a three-year follow-up of the included patients. Patients with positive screening underwent right heart catheterization.
What are the possible benefits and risks of participating?
Potential benefits: patients who participate have the chance to receive an early diagnosis of pulmonary hypertension. This affords early treatment and may alleviate symptoms, improve quality of life. We speculate that early treatment could improve survival.
Potential risks: to confirm the diagnosis of pulmonary hypertension, the patients referred by the screening had to undergo right heart catheterization. This is an invasive haemodynamic procedure. Right heart catheterization has the risks of bleeding, heart arrhythmias, vessel perforation, allergic reaction to iodine contrast, heart failure and very rarely death.
Where is the study run from?
University Hospital Pedro Ernesto (Brazil)
When is the study starting and how long is it expected to run for?
June 2012 to July 2020
Who is funding the study?
Fundação Carlos Chagas Filho de Amparo à Pesquisa do Estado do Rio de Janeiro (Foundation to Sponsor Research in Rio de Janeiro) (Brazil)
Who is the main contact?
Rogério Rufino, rrufino.uerj@gmail.com
Contact information
Public
Boulevard 28 de Setembro 77
Vila Isabel
Rio de Janeiro
20551-030
Brazil
 ORCID ID |
0000-0002-8676-4268 |
|---|---|
| Phone | +55 212868-8216 |
| veronica.vilela@hupe.uerj.br |
Study information
| Study design | Single centre observational study |
|---|---|
| Primary study design | Observational |
| Secondary study design | Longitudinal study |
| Study setting(s) | Hospital |
| Study type | Screening |
| Participant information sheet | Not available in web format, please use the contact details to request a patient information sheet. |
| Scientific title | Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after a three-year follow-up |
| Study objectives | A systematic screening to achieve early diagnosis of systemic sclerosis-related pulmonary hypertension can be performed. The profile and prognosis of the patients diagnosed by this screening should be described. |
| Ethics approval(s) | Approved 24/03/2013, Local ethics committee University Hospital Pedro Ernesto (Boulevard 28 de Setembro 77 Vila Isabel CePeM – Centro de Pesquisa Clínica Multiusuário – 2º andar/sala 28, prédio anexo ao Hospital Universitário Pedro Ernesto, Rio de Janeiro, Brazil; +55 21 2868-8253; cep@hupe.uerj.br), ref: 314.092 |
| Health condition(s) or problem(s) studied | Pulmonary hypertension related to systemic sclerosis |
| Intervention | All patients with systemic sclerosis in treatment at our referral centre were invited to be included between 01/07/2014 until 01/07/2017. Included patients underwent a systematic screening for pulmonary hypertension that included transthoracic echocardiography and the multiple tools required by the DETECT algorithm. Patients were referred to right heart catheterization if the screening was positive. The profile of the patients diagnosed by this screening was described. The prognosis was evaluated by a three-year follow-up. |
| Intervention type | Procedure/Surgery |
| Primary outcome measure | Diagnosis of pulmonary hypertension at baseline (yes/no) measured using patient records |
| Secondary outcome measures | 1. Survival measured using patient records at 3 years 2. Worsening of condition from baseline measured using patient records at 3 years |
| Overall study start date | 15/06/2012 |
| Completion date | 01/07/2020 |
Eligibility
| Participant type(s) | Patient |
|---|---|
| Age group | Adult |
| Lower age limit | 18 Years |
| Sex | Both |
| Target number of participants | 83 |
| Total final enrolment | 65 |
| Key inclusion criteria | 1. Aged ≥ 18 years 2.Systemic sclerosis diagnosis according to the 2013 European League Against Rheumatism and American College of Rheumatology criteria |
| Key exclusion criteria | 1. Severe left ventricle dysfunction (left ventricular fraction ejection <55%) 2. Severe restrictive lung disease (forced vital capacity <40%) 3. Unwillingness to undergo right heart catheterization |
| Date of first enrolment | 01/07/2014 |
| Date of final enrolment | 01/07/2017 |
Locations
Countries of recruitment
- Brazil
Study participating centre
Vila Isabel
Rio de Janeiro
20551-030
Brazil
Sponsor information
Government
Edifício Estado de Sá - 6º Andar
Av. Erasmo Braga 118 - Centro
Rio de Janeiro
20020-000
Brazil
| Phone | +55 21 2333-2001 |
|---|---|
| central.atendimento@faperj.br | |
| Website | http://www.faperj.br/ |
"ROR" |
https://ror.org/03kk0s825 |
Funders
Funder type
Charity
Government organisation / Trusts, charities, foundations (both public and private)
- Alternative name(s)
- Carlos Chagas Filho Foundation for Research Support in Rio de Janeiro, Research Support Foundation for the State of Rio de Janeiro, FAPERJ
- Location
- Brazil
Results and Publications
| Intention to publish date | 31/12/2021 |
|---|---|
| Individual participant data (IPD) Intention to share | Yes |
| IPD sharing plan summary | Available on request |
| Publication and dissemination plan | Planned publication in a high-impact peer-reviewed journal. |
| IPD sharing plan | The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request: vilelavs@gmail.com Type of data: demographic, screening tools, screening results, clinica, serum and immunological data, haemodynamic data. All datasets are on Excel sheets where anonymisation was made by replacing patients dentifications by numbers (codes). The criteria for the access to the data is to send e-mail for the main author with reasonable request. Data will be available as soon as requested by email (within one or two weeks). Once shared, data will be permanently available. As patient identifications are not disclosed in the sheets, consent from participants to share data was not obtained. There are no legal and ethical restrictions for this data sharing. |
Study outputs
| Output type | Details | Date created | Date added | Peer reviewed? | Patient-facing? |
|---|---|---|---|---|---|
| Results article | 29/07/2021 | 02/08/2021 | Yes | No | |
| Dataset | 18/08/2023 | No | No |
Editorial Notes
18/08/2023: A link to the dataset was added.
02/08/2021: Publication reference added.
08/07/2021: Trial's existence confirmed by University Hospital Pedro Ernesto
