ArticleEuropean journal of immunology2018
Circulating plasmablasts are elevated and produce pathogenic anti-endothelial cell autoantibodies in idiopathic pulmonary arterial hypertension.
Article in European journal of immunology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
What it found
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Who cites it
22 citing papers in PubMed, 39 citations in OpenAlex.
- Macrophage κ-opioid receptor inhibits hypoxic pulmonary hypertension progression and right heart dysfunction via an SCD1-dependent anti-inflammatory response.Genes & diseases · 2026Article
- Research progress on the mechanisms of cGAS-STING signaling in immune cell infiltration associated with vascular remodeling.Journal of translational medicine · 2025Review
- B-cells in pulmonary arterial hypertension: friend, foe or bystander?The European respiratory journal · 2024Review
- Biomarkers in Pulmonary Arterial Hypertension.Diagnostics (Basel, Switzerland) · 2022Review
- Autoimmunity Is a Significant Feature of Idiopathic Pulmonary Arterial Hypertension.American journal of respiratory and critical care medicine · 2022Article
- Peripheral Blood T Cells of Patients with IPAH Have a Reduced Cytokine-Producing Capacity.International journal of molecular sciences · 2022Article
- Article
- Endothelin B Receptor Immunodynamics in Pulmonary Arterial Hypertension.Frontiers in immunology · 2022Article
- Loss of immune homeostasis in patients with idiopathic pulmonary arterial hypertension.Thorax · 2021Article
- Role of the Immune System Elements in Pulmonary Arterial Hypertension.Journal of clinical medicine · 2021Review
- Immune Cells Profiling in ANCA-Associated Vasculitis Patients-Relation to Disease Activity.Cells · 2021Article
- Plasma markers in pulmonary hypertension subgroups correlate with patient survival.Respiratory research · 2021Observational
- Novel Experimental Therapies for Treatment of Pulmonary Arterial Hypertension.Journal of experimental pharmacology · 2021Review
- Modeling human adaptive immune responses with tonsil organoids.Nature medicine · 2021Article
- Autoimmunity in Pulmonary Arterial Hypertension: Evidence for Local Immunoglobulin Production.Frontiers in cardiovascular medicine · 2021Review
- Management of Pulmonary Arterial Hypertension.Current cardiovascular risk reports · 2021Review
- Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: An Immunological Perspective.Journal of clinical medicine · 2020Review
- Inflammatory Basis of Pulmonary Arterial Hypertension: Implications for Perioperative and Critical Care Medicine.Anesthesiology · 2019Review
- Single-cell technologies - studying rheumatic diseases one cell at a time.Nature reviews. Rheumatology · 2019Review
- Affinity Maturation of the Anti-Citrullinated Protein Antibody Paratope Drives Epitope Spreading and Polyreactivity in Rheumatoid Arthritis.Arthritis & rheumatology (Hoboken, N.J.) · 2019Article
Corrections and comments
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Authors and funding
14 authors at 2 institutions in 1 country.
Funding
Abstract
Idiopathic pulmonary arterial hypertension (IPAH) is a devastating pulmonary vascular disease in which autoimmune and inflammatory phenomena are implicated. B cells and autoantibodies have been associated with IPAH and identified as potential therapeutic targets. However, the specific populations of B cells involved and their roles in disease pathogenesis are not clearly defined. We aimed to assess the levels of activated B cells (plasmablasts) in IPAH, and to characterize recombinant antibodies derived from these plasmablasts. Blood plasmablasts are elevated in IPAH, remain elevated over time, and produce IgA autoantibodies. Single-cell sequencing of plasmablasts in IPAH revealed repertoires of affinity-matured antibodies with small clonal expansions, consistent with an ongoing autoimmune response. Recombinant antibodies representative of these clonal lineages bound known autoantigen targets and displayed an unexpectedly high degree of polyreactivity. Representative IPAH plasmablast recombinant antibodies stimulated human umbilical vein endothelial cells to produce cytokines and overexpress the adhesion molecule ICAM-1. Together, our results demonstrate an ongoing adaptive autoimmune response involving IgA plasmablasts that produce anti-endothelial cell autoantibodies in IPAH. These antibodies stimulate endothelial cell production of cytokines and adhesion molecules, which may contribute to disease pathogenesis. These findings suggest a role for mucosally-driven autoimmunity and autoimmune injury in the pathogenesis of IPAH.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.