Skip to content
2000
Volume 5, Issue 1
  • ISSN: 0250-6882
  • E-ISSN: 0250-6882
file format pdf download PDF
side by side viewer icon HTML

Abstract

Pulmonary arterial hypertension (PAH) is a frequent complication of connective tissue diseases (CTDs), such as systemic sclerosis (SSc), mixed connective tissue disease, systemic lupus erythematosus (SLE), and, to a lesser extent, dermatomyositis, rheumatoid arthritis, and Sjögren syndrome. Connective tissue disease-associated PAH (CTD-PAH) is the second most common cause of PAH, following the idiopathic form of PAH. Nearly 15%–30% of patients with SSc develop PAH and have a very poor prognosis and survival rates compared to idiopathic PAH. Also, the majority of these patients present with substantial functional impairment and severe hemodynamics at diagnosis. Therefore, an early diagnosis of CTD-PAH through annual systematic screening and appropriate treatment is crucial in order to achieve optimal clinical outcomes and has been strongly recommended with the highest level of evidence in rheumatology, cardiology, and pulmonary guidelines since 2014. Unfortunately, annual screening in at-risk CTD-PAH patients is not being performed in the majority of patients, leading to substantial delays in therapy.

© 2024 The Author(s). Published by Bentham Science Publisher.
This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Loading

Article metrics loading...

/content/journals/nemj/10.2174/0102506882333536241105102119
2024-01-01
2025-10-31

  • From This Site

    /content/journals/nemj/10.2174/0102506882333536241105102119
    dcterms_title,dcterms_subject,pub_keyword
    -contentType:Contributor -contentType:Concept -contentType:Institution
    10
    5
Loading full text...

Full text loading...

/deliver/fulltext/nemj/5/1/NEMJ-5-E02506882333536.html?itemId=/content/journals/nemj/10.2174/0102506882333536241105102119&mimeType=html&fmt=ahah

References

  1. DistlerO. OfnerC. HuscherD. JordanS. UlrichS. StählerG. GrünigE. HeldM. GhofraniH.A. ClaussenM. LangeT.J. KloseH. RosenkranzS. NoordegraafV.A. VizzaC.D. DelcroixM. OpitzC. PauschC. ScelsiL. NeurohrC. OlssonK.M. CoghlanJ.G. HalankM. SkowaschD. BehrJ. MilgerK. RemppisB.A. SkrideA. JurevicieneE. GumbieneL. MiliauskasS. RaggL.J. WilkensH. PittrowD. HoeperM.M. EwertR. Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: A COMPERA analysis.Rheumatology20246341139114610.1093/rheumatology/kead360
     [Google Scholar]
  2. AdrovicA. OztuncF. BarutK. KokaA. GojakR. SahinS. The frequency of pulmonary hypertension in patients with juvenile scleroderma.Bosn. J. Basic Med. Sci.20151543035
     [Google Scholar]
  3. AdrovicA. DedeogluR. SahinS. BarutK. KokaA. CengizD. Evaluation of pulmonary artery pressure in patients with juvenile systemic lupus erythematosus (jSLE).Bosn. J. Basic Med. Sci.20181816671
     [Google Scholar]
  4. GalièN. HumbertM. VachieryJ.L. GibbsS. LangI. TorbickiA. SimonneauG. PeacockA. NoordegraafV.A. BeghettiM. GhofraniA. SanchezG.M.A. HansmannG. KlepetkoW. LancellottiP. MatucciM. McDonaghT. PierardL.A. TrindadeP.T. ZompatoriM. HoeperM. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.Eur. Heart J.20163716711910.1093/eurheartj/ehv317
     [Google Scholar]
  5. HaoY. ThakkarV. StevensW. MorrisroeK. PriorD. RabusaC. YoussefP. GabbayE. RoddyJ. WalkerJ. ZochlingJ. SahharJ. NashP. LesterS. RischmuellerM. ProudmanS.M. NikpourM. A comparison of the predictive accuracy of three screening models for pulmonary arterial hypertension in systemic sclerosis.Arthritis Res. Ther.2015171710.1186/s13075‑015‑0517‑5
     [Google Scholar]
  6. HassounP.M. BoueizA. Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: Pulmonary arterial hypertension associated with connective tissue diseases.Ann. Thorac. Med.2014952610.4103/1817‑1737.134016
     [Google Scholar]
  7. SteenV.D. FollansbeeW.P. ConteC.G. MedsgerT.A.Jr Thallium perfusion defects predict subsequent cardiac dysfunction in patients with systemic sclerosis.Arthritis Rheum.199639467768110.1002/art.1780390421
     [Google Scholar]
  8. CondonD.F. NickelN.P. AndersonR. MirzaS. PerezJ.V.A. The 6th World symposium on pulmonary hypertension: What's old is new.F1000Res.20198F100010.12688/f1000research.18811.1
     [Google Scholar]
  9. QuatredeniersM. FerreiraM.P. RibeiroS.D. NakhlehM.K. GhignaM.R. KaminskyC.S. PerrosF. Iron deficiency in pulmonary arterial hypertension: A deep dive into the mechanisms.Cells202110247710.3390/cells10020477
     [Google Scholar]
  10. ViethenT. GerhardtF. DumitrescuD. BuschK.S. FreyhausT.H. RudolphT.K. BaldusS. RosenkranzS. Ferric carboxymaltose improves exercise capacity and quality of life in patients with pulmonary arterial hypertension and iron deficiency: A pilot study.Int. J. Cardiol.2014175223323910.1016/j.ijcard.2014.04.233
     [Google Scholar]
  11. LeardL.E. HolmA.M. ValapourM. GlanvilleA.R. AttawarS. AversaM. CamposS.V. ChristonL.M. CypelM. DellgrenG. HartwigM.G. KapnadakS.G. KolaitisN.A. KotloffR.M. PattersonC.M. ShlobinO.A. SmithP.J. SoléA. SolomonM. WeillD. WijsenbeekM.S. WillemseB.W.M. ArcasoyS.M. RamosK.J. Consensus document for the selection of lung transplant candidates: An update from the international society for heart and lung transplantation.J. Heart Lung Transplant.202140111349137910.1016/j.healun.2021.07.005
     [Google Scholar]
  12. HwalekA. RosenheckJ.P. WhitsonB.A. Lung transplantation for pulmonary hypertension.J. Thorac. Dis.202113116708671610.21037/jtd‑2021‑20
     [Google Scholar]
  13. FDA UTracleer® (bosentan) tablets, for oral use 2001.Available from: https://www.accessdata.fda.gov/drugsatfda_docs/ label/2017/209279s000lbl.pdf. [cited as 08 October 2021].2001
  14. FDA UMethotrexate tablets, USP.Available from: https://www.accessdata.fda.gov/drugsatfda_docs/label/2016/008085s066lbl.pdf. [cited as 08 October 2021].2021
  15. FDA PiCELLCEPT↓ Intravenous (mycophenolate mofetil) for injection, for intravenous use 2018.Available from: https://www.accessdata.fda.gov/drugsatfda_docs/label/2018/050722s035,050723s035,050758s033,050759s041lbl.pdf 2018
/content/journals/nemj/10.2174/0102506882333536241105102119
Loading
Diagnosis and Management of the Connective Tissue Disease-associated PAH in the UAE
NEW Emirates Medical J 5, e02506882333536 (2024); https://doi.org/10.2174/0102506882333536241105102119
/content/journals/nemj/10.2174/0102506882333536241105102119
/content/journals/nemj/10.2174/0102506882333536241105102119
Loading

Data & Media loading...


  • Article Type:     Review Article
Keyword(s): ASIG screening algorithm; Connective tissue diseases; Dermatomyositis; DETECT algorithm; Systemic lupus erythematosus; Systemic sclerosis

Most Cited Most Cited RSS feed

This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error
Please enter a valid_number test