Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
Cardiovascular, Case Report
Cardiovascular, Editorial
Cardiovascular, Images in Cardiology
Cardiovascular, Interventional Round
Cardiovascular, Original Article
Cardiovascular, Preface
Cardiovascular, Review Article
Cardiovascular, Student’s Corner
Case Report
Case Reports
Clinical Discussion
Clinical Rounds
CPC
Debate
Dedication
Editorial
Expert Comments
Expert's Opinion
Genetic Autopsy
Genetics Autopsy
Image in Cardiology
Images in Cardiology
Interventional Round
Interventional Rounds
Letter to the Editor
Media and news
Original Article
Practice in Medicine
Preface
Review Article
Scientific Paper
Short Communication
Student's Corner
WINCARS Activities
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
Cardiovascular, Case Report
Cardiovascular, Editorial
Cardiovascular, Images in Cardiology
Cardiovascular, Interventional Round
Cardiovascular, Original Article
Cardiovascular, Preface
Cardiovascular, Review Article
Cardiovascular, Student’s Corner
Case Report
Case Reports
Clinical Discussion
Clinical Rounds
CPC
Debate
Dedication
Editorial
Expert Comments
Expert's Opinion
Genetic Autopsy
Genetics Autopsy
Image in Cardiology
Images in Cardiology
Interventional Round
Interventional Rounds
Letter to the Editor
Media and news
Original Article
Practice in Medicine
Preface
Review Article
Scientific Paper
Short Communication
Student's Corner
WINCARS Activities
View/Download PDF

Translate this page into:

Editorial
06 (
02
); 106-107
doi:
10.1055/s-0041-1732503

Pulmonary Artery Hypertension in SLE

Department of Cardiology, CARE Hospitals, Hyderabad, Telangana, India
Address for correspondence B K S Sastry, MD, DM. FACC Department of Cardiology CARE Hospitals, Nampally, Hyderabad, Telangana, 500001 India bkssastry@hotmail.com
Licence
This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
Disclaimer:
This article was originally published by Thieme Medical and Scientific Publishers Pvt. Ltd. and was migrated to Scientific Scholar after the change of Publisher; therefore Scientific Scholar has no control over the quality or content of this article.

Pulmonary hypertension (PH) is characterized by pulmonary artery mean pressure more than 20 mm Hg. It is caused by diverse diseases. It is classified into five categories, based on etiology, pathophysiology, natural history, and response to treatment. Group 1 PH, also called pulmonary artery hypertension (PAH), includes many different diseases including PH due to connective tissue disease (CTD). The most common CTD to cause PAH is systemic sclerosis. PAH is also commonly seen in mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), and rheumatoid arthritis. There is no uniform pathophysiologic mechanism for PH. Molecular pathogenesis of PAH is not completely known, and thus the therapeutic targets in the management of PAH are limited. This makes the prognosis and treatment of PAH due to CTD more difficult.

In this context, the paper published by Baisya et al, which provides insights into the molecular mechanism of PAH in SLE, is a step in the right direction, and I congratulate them for that. Baisya et al conducted a cluster analysis of auto antibodies in 71 patients with SLE-associated PAH. They concluded that the cluster with no definite antibody association had the highest mean right ventricular systolic pressure (RVSP) or severe PAH.1

One of the limitations of their study, as rightly acknowledged by the authors, is that the diagnosis of PAH is based on echocardiography alone while the gold standard is right heart catheterization. It is quite understandable that patients’ acceptance of cardiac catheterization would be quite low in the Indian context. Instead of diagnosing PH solely on the basis of tricuspid regurgitation (TR) jet velocity, they could have made the diagnosis of PH, based on the guidelines provided by the American Society of Echocardiography.2 However, it is likely that the diagnosis of PAH is valid in this cohort of patients, considering the criteria they used.

Cluster analysis is useful in identifying the risk factors and mechanisms of the disease and developing therapeutic targets. Reported incidence of PAH in SLE and autoantibody associations with SLE PAH are somewhat divergent and appear to be based on geographic location and ethnicity.3 4 Incidence of PAH in SLE is relatively less in Taiwanese and Caucasians, while it is more in Pakistanis and African Americans.5 6 7 There are significant interethnic differences observed in the REVEAL registry done in the US.8 Similarly, different antibodies are associated with SLE PAH, as reported in different studies. In a UK study, association with lupus anticoagulant was reported, while the French reported association with anti-Ro and anti-La antibodies and the Chinese reported association with anti-RNP and anticardiolipin antibodies.9 10 11 12 This kind of data strongly suggests the possible role played by the underlying genetic factors, and this study has to be seen as part of a bigger picture. Often there is overlap between different autoimmune rheumatological diseases, their phenotypes and prevalent antibodies.13 Keeping in mind wide variations and smaller size of each cohort, one may consider a pooled analysis of PAH associated with different autoimmune diseases.

Antibodies interact closely with cytokines in pathogenesis of diseases. In an interesting study, researchers from the Stanford University and University of Sheffield carried out cluster analysis of circulating cytokines and chemokines in patients with PAH.14 They identified four clusters and established an association with prognosis, although there were no difference in phenotypes. Larger studies that include patients with PAH associated with different CTD, and analyze antibody clusters as well as cytokine profiles, may throw more light on pathogenesis and prognosis and help identify therapeutic targets. A truly personalized precision medicine may not be far off.

Conflict of Interests

None.

Source of Funding None

References

  1. Baisya R, Devarasetti PK, GSR Murthy, Rajasekhar L . Auto-antibody clustering in systemic lupus erythematosus associated pulmonary arterial hypertension. Ind J Car Dis Wom 2021. doi: 10.1055/s-0041-1732510
  2. , , , et al . Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr. 2010;23(07):685-713.
    [Google Scholar]
  3. , , , et al . Baseline characteristics and risk factors of pulmonary arterial hypertension in systemic lupus erythematosus patients. Medicine (Baltimore. 2016;95(10):e2761.
    [Google Scholar]
  4. , , , , , . Pulmonary arterial hypertension: the clinical syndrome. Circ Res. 2014;115(01):115-130.
    [Google Scholar]
  5. , , , et al . Incidence and survival impact of pulmonary arterial hypertension among patients with systemic lupus erythematosus: a nationwide cohort study. Arthritis Res Ther. 2019;21(01):82.
    [Google Scholar]
  6. , , , , . Frequency and predictors of pulmonary hypertension in patients with Systemic Lupus Erythematosus. Pak J Med Sci. 2019;35(01):86-89.
    [Google Scholar]
  7. , , , , . Autoantibody clustering of lupus-associated pulmonary hypertension. Lupus Sci Med. 2019;6(01):e000356.
    [Google Scholar]
  8. , , , et al . Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest. 2010;138(06):1383-1394.
    [Google Scholar]
  9. , , , et al . Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus. Rheumatology (Oxford. 2009;48(12):1506-1511.
    [Google Scholar]
  10. , , , et al . CSTAR co-authors. Chinese SLE Treatment and Research group (CSTAR) registry: II. Prevalence and risk factors of pulmonary arterial hypertension in Chinese patients with systemic lupus erythematosus. Lupus. 2014;23(10):1085-1091.
    [Google Scholar]
  11. , , , et al . Clinical features and independent predictors of pulmonary arterial hypertension in systemic lupus erythematosus. Rheumatol Int. 2012;32(06):1727-1731.
    [Google Scholar]
  12. , , , et al , , , . Pulmonary arterial hypertension associated with systemic lupus erythematosus: results from the French pulmonary hypertension registry. Chest. 2018;153(01):143-151.
    [Google Scholar]
  13. , , , et al . Cluster analysis of autoimmune rheumatic diseases based on autoantibodies. New insights for polyautoimmunity. J Autoimmun. 2019;98:24-32.
    [Google Scholar]
  14. , , , et al . Discovery of distinct immune phenotypes using machine learning in pulmonary arterial hypertension. Circ Res. 2019;124(06):904-919.
    [Google Scholar]

Fulltext Views
17

PDF downloads
0
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections