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Autoimmunity Is a Significant Feature of Idiopathic Pulmonary Arterial Hypertension.
Jones, Rowena J; De Bie, Eckart M D D; Groves, Emily; Zalewska, Kasia I; Swietlik, Emilia M; Treacy, Carmen M; Martin, Jennifer M; Polwarth, Gary; Li, Wei; Guo, Jingxu; Baxendale, Helen E; Coleman, Stephen; Savinykh, Natalia; Coghlan, J Gerry; Corris, Paul A; Howard, Luke S; Johnson, Martin K; Church, Colin; Kiely, David G; Lawrie, Allan; Lordan, James L; Mackenzie Ross, Robert V; Pepke Zaba, Joanna; Wilkins, Martin R; Wort, S John; Fiorillo, Edoardo; Orrù, Valeria; Cucca, Francesco; Rhodes, Christopher J; Gräf, Stefan; Morrell, Nicholas W; McKinney, Eoin F; Wallace, Chris; Toshner, Mark.
Afiliación
  • Jones RJ; Heart and Lung Research Institute.
  • De Bie EMDD; Heart and Lung Research Institute.
  • Groves E; Heart and Lung Research Institute.
  • Zalewska KI; Heart and Lung Research Institute.
  • Swietlik EM; Royal Papworth Hospital, and.
  • Treacy CM; Heart and Lung Research Institute.
  • Martin JM; Royal Papworth Hospital, and.
  • Polwarth G; Heart and Lung Research Institute.
  • Li W; Heart and Lung Research Institute.
  • Guo J; Royal Papworth Hospital, and.
  • Baxendale HE; Heart and Lung Research Institute.
  • Coleman S; Heart and Lung Research Institute.
  • Savinykh N; Royal Papworth Hospital, and.
  • Coghlan JG; Medical Research Council Biostatistics Unit.
  • Corris PA; Cambridge Biomedical Research Centre Phenotyping Hub, Department of Medicine, University of Cambridge.
  • Howard LS; Royal Free London National Health Service Foundation Trust, London, United Kingdom.
  • Johnson MK; Freeman Hospital, Newcastle-upon-Tyne, United Kingdom.
  • Church C; Hammersmith Hospital, London, United Kingdom.
  • Kiely DG; Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, United Kingdom.
  • Lawrie A; Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, United Kingdom.
  • Lordan JL; Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom.
  • Mackenzie Ross RV; Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, United Kingdom.
  • Pepke Zaba J; Freeman Hospital, Newcastle-upon-Tyne, United Kingdom.
  • Wilkins MR; Royal United Hospitals Bath National Health Service Foundation Trust, Bath, United Kingdom.
  • Wort SJ; Royal Papworth Hospital, and.
  • Fiorillo E; National Heart and Lung Institute, Faculty of Medicine, Imperial College London, London, United Kingdom.
  • Orrù V; Royal Brompton Hospital, London, United Kingdom.
  • Cucca F; Istituto di Ricerca Genetica e Biomedica, Consiglio Nazionale delle Ricerche, Cagliari, Italy.
  • Rhodes CJ; Istituto di Ricerca Genetica e Biomedica, Consiglio Nazionale delle Ricerche, Cagliari, Italy.
  • Gräf S; University of Sassari, Sassari, Italy; and.
  • Morrell NW; National Heart and Lung Institute, Faculty of Medicine, Imperial College London, London, United Kingdom.
  • McKinney EF; Heart and Lung Research Institute.
  • Wallace C; Heart and Lung Research Institute.
  • Toshner M; Cambridge Institute of Therapeutic Immunology and Infectious Disease, Jeffrey Cheah Biomedical Centre, Cambridge Biomedical Campus, Cambridge, United Kingdom.
Am J Respir Crit Care Med ; 206(1): 81-93, 2022 07 01.
Article en En | MEDLINE | ID: mdl-35316153
Rationale: Autoimmunity is believed to play a role in idiopathic pulmonary arterial hypertension (IPAH). It is not clear whether this is causative or a bystander of disease and if it carries any prognostic or treatment significance. Objectives: To study autoimmunity in IPAH using a large cross-sectional cohort. Methods: Assessment of the circulating immune cell phenotype was undertaken using flow cytometry, and the profile of serum immunoglobulins was generated using a standardized multiplex array of 19 clinically validated autoantibodies in 473 cases and 946 control subjects. Additional glutathione S-transferase fusion array and ELISA data were used to identify a serum autoantibody to BMPR2 (bone morphogenetic protein receptor type 2). Clustering analyses and clinical correlations were used to determine associations between immunogenicity and clinical outcomes. Measurements and Main Results: Flow cytometric immune profiling demonstrates that IPAH is associated with an altered humoral immune response in addition to raised IgG3. Multiplexed autoantibodies were significantly raised in IPAH, and clustering demonstrated three distinct clusters: "high autoantibody," "low autoantibody," and a small "intermediate" cluster exhibiting high concentrations of ribonucleic protein complex. The high-autoantibody cluster had worse hemodynamics but improved survival. A small subset of patients demonstrated immunoglobulin reactivity to BMPR2. Conclusions: This study establishes aberrant immune regulation and presence of autoantibodies as key features in the profile of a significant proportion of patients with IPAH and is associated with clinical outcomes.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Autoinmunidad / Hipertensión Pulmonar Tipo de estudio: Observational_studies / Prevalence_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: Am J Respir Crit Care Med Asunto de la revista: TERAPIA INTENSIVA Año: 2022 Tipo del documento: Article Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Autoinmunidad / Hipertensión Pulmonar Tipo de estudio: Observational_studies / Prevalence_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: Am J Respir Crit Care Med Asunto de la revista: TERAPIA INTENSIVA Año: 2022 Tipo del documento: Article Pais de publicación: Estados Unidos