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The causal association between pulmonary arterial hypertension (PAH) and autoimmune diseases remains uncertain. This study aimed to assess the causal associations between PAH and autoimmune diseases using bidirectional Mendelian randomization (MR) analyses. Genome-wide association summary statistics for PAH, asthma, myasthenia gravis, rheumatoid arthritis (RA), systemic lupus erythematosus, and type 1 diabetes mellitus were obtained from publicly accessible databases. The primary MR approach used was the inverse variance weighted method. Sensitivity analyses were conducted to test the robustness of the MR findings, including tests for heterogeneity, horizontal pleiotropy, and leave-one-out analysis, ensuring the reliability and validity of the results. Ultimately, transcriptome analysis was used for GO, KEGG enrichment analysis and protein interaction network. Bidirectional Mendelian randomization analysis found a causal relationship between PAH and RA (OR [95% CI] > 1; P < .05). Enrichment analysis further revealed the common molecular mechanisms of these 2 diseases, especially the dysfunction of chemokine pathway and other inflammation-related signaling pathways. Additionally, the study uncovered the core genes within the co-morbidity-associated protein-protein interaction network, including CCL5, CCL9, and VCAM1. Transcription factor (TF) network analysis showed that TFs such as GATA1, JUN and RELA were significantly up-regulated in PAH, and they play a key role in regulating cell proliferation and immune response. The study found a bidirectional positive causal link between PAH and RA. Dysregulation of the chemokine pathway and other inflammation-related signaling pathways may be momentous factors driving the progression of PAH and RA.
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http://dx.doi.org/10.1097/MD.0000000000041737 | DOI Listing |
JACC Case Rep
August 2025
Department of Cardiology, Hospital de Santa Maria (ULSSM), CAML, CCUL@RISE, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal.
Background: Pulmonary arterial hypertension (PAH) is a severe and potentially life-threatening complication of systemic lupus erythematosus.
Case Summary: We present the case of a young woman with dyspnea at rest, hospitalized with a suspected systemic lupus erythematosus flare. Right heart catheterization confirmed pre-capillary pulmonary hypertension with a low cardiac index.
Arthritis Res Ther
July 2025
Rheumatology Unit, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Via S. Pansini 5, Naples, 80131, Italy.
Objective: To assess the contribution of Systemic sclerosis (SSc)-specific features on type II diabetes mellitus (T2D) in a large cohort of Italian SSc patients.
Methods: A total of 613 SSc patients from 11 tertiary Rheumatology Units across Italy were included. All patients underwent full history taking, clinical examination, and relevant laboratory and radiological evaluations.
Turk J Med Sci
July 2025
Division of Cardiology, Faculty of Medicine, Çukurova University, Adana, Turkiye.
Background/aim: Systemic sclerosis (SSc) is a complex autoimmune disease marked by vascular abnormalities and fibrosis. This study aimed to compare pulmonary artery wall thickness (PAWT) among SSc patients without PAH, patients with idiopathic PAH (IPAH), and healthy controls, and to explore the clinical implications of increased PAWT in SSc patients.
Materials And Methods: This cross-sectional study included 60 SSc patients, 30 patients with IPAH, and 40 age- and sex-matched healthy controls.
Future Cardiol
August 2025
Department of Rheumatology, The First Affiliated Hospital with Nanjing Medical University, Nanjing, China.
Right ventricular (RV) function is the primary determinant of survival in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). A "double hit" hypothesis suggests that RV suffers not only from pressure overload common to all PAH but also from direct insults related to the underlying systemic autoimmunity and inflammation. This likely drives distinct maladaptive remodeling (fibrosis, inflammation) and contributes to the poorer prognosis observed in CTD-PAH compared to idiopathic PAH (IPAH).
View Article and Find Full Text PDFGlob Heart
July 2025
Department of Cardiothoracic Surgery, The Third Affiliated Hospital of Soochow University, Changzhou, Jiangsu Province 213003, China.
Background: Pulmonary arterial hypertension (PAH) is a severe vascular disorder with a multifactorial etiology, including potential genetic predispositions. Understanding the causal relationship between autoimmune diseases and the risk of developing PAH can inform clinical strategies for prevention and treatment.
Methods: We conducted a two-sample Mendelian Randomization (MR) analysis to evaluate the causal effect of genetic predisposition to five autoimmune diseases (systemic lupus erythematosus [SLE], rheumatoid arthritis [RA], inflammatory bowel disease [IBD], multiple sclerosis [MS], and type 1 diabetes [T1D]) on the risk of PAH.