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Exploring cardiovascular involvement in IgG4-related disease: a case series approach with cardiovascular magnetic resonance
  1. John Aaron Henry1,2,
  2. Roshan Xavier1,3,
  3. Emmanuel Selvaraj2,3,
  4. Matthew Burrage3,4,
  5. Katharine E Thomas1,3,
  6. Elena Lukaschuk1,3,
  7. Qiang Zhang1,3,
  8. Vanessa M Ferreira1,2,3,
  9. Stefan K Piechnik1,3,
  10. Nikant Sabharwal2,
  11. Stefan Neubauer1,2,3,5,
  12. Oliver Rider1,2,3,
  13. Emma L Culver2,5,
  14. Andrew Lewis1,2,3,5
  1. 1University of Oxford, Oxford Centre for Clinical Magnetic Resonance Research, Oxford, UK
  2. 2Oxford University Hospitals NHS Foundation Trust, Oxford, UK
  3. 3Radcliffe Department of Medicine, University of Oxford, Oxford, UK
  4. 4Faculty of Medicine, University of Queensland, Brisbane, Queensland, Australia
  5. 5NIHR Oxford Biomedical Research Centre, Oxford, UK
  1. Correspondence to Dr Andrew Lewis; andrew.lewis{at}cardiov.ox.ac.uk

Abstract

Background IgG4-related disease (IgG4-RD) is a relapsing–remitting, fibroinflammatory, multisystem disorder. Cardiovascular involvement from IgG4-RD has not been systematically characterised. In this study, we sought to evaluate consecutive patients with IgG4-RD using a detailed multiparametric cardiovascular magnetic resonance (CMR) imaging protocol.

Methods We prospectively enrolled 11 patients with histology-confirmed IgG4-RD; with active disease at time of scan. We undertook a detailed multiparametric CMR imaging protocol at 1.5T including cine imaging, native T1 and T2 mapping, stress perfusion imaging with inline quantitation of myocardial blood flow and late gadolinium enhancement (LGE) imaging.

Results All patients exhibited at least one abnormality on CMR imaging. Abnormal elevation of global or segmental left ventricular myocardial T1 and T2 values was present in four patients, suggesting myocardial oedema or inflammation. Abnormal LGE, suggesting myocardial scar fibrosis, was present in nine patients, with eight displaying a non-ischaemic pattern, and one showing an ischaemic pattern. Four patients fulfilled both Lake Louise Criteria for active myocardial inflammation, while a further six fulfilled one criterion. Myocardial perfusion reserve was normal in all evaluable patients. Ten patients had normal ventricular volumes, mass and systolic function. In addition, thoracic aortitis was identified in three patients who underwent 18F-flourodeoxyglucose PET/CT imaging, with resolution following anti-B-cell treatment.

Conclusions In this cohort of patients with histology-confirmed IgG4-RD, multiparametric CMR revealed no changes in gross cardiac structure and function, but frequent myocardial tissue abnormalities. These data suggest a plausible pathophysiological link between IgG4-RD and cardiovascular involvement.

  • Magnetic Resonance Imaging
  • Positron Emission Tomography Computed Tomography
  • Inflammation

Data availability statement

Data are available upon reasonable request. The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request.

https://doi.org/10.1136/heartjnl-2024-324785

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    Data availability statement

    Data are available upon reasonable request. The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request.

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    Footnotes

    • ELC and AL are joint senior authors.

    • X @johnaaronhenry, @@cardiolewis

    • Contributors AL and ELC conceptualised and designed the study. Data were collected by JAH, RX, ES, MB and KET. Data analysis was conducted by JAH, RX, KET, EL, QZ, VMF, SKP, and AL. NS, VMF, SKP, OR, SN, AL and ELC contributed to data interpretation. The manuscript was written by JAH. All authors contributed to the article and approved the submitted version. AL is the guarantor.

    • Funding The research was supported by the National Institute for Health Research (NIHR) Oxford Biomedical Research Centre (BRC). The views expressed are those of the author(s) and not necessarily those of the NHS, the NIHR or the Department of Health. AL acknowledges funding support from the BHF Oxford Centre for Research Excellence (RE/18/3/34214), and the British Heart Foundation (FS/ICRF/24/26111).

    • Competing interests ELC consults for Horizon Therapeutics, Zenus BioPharma and Sanofi for IgG4-RD. AL consults for Abbott Laboratoies and acknowledges speaker fees from Novartis.

    • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

    • Provenance and peer review Not commissioned; externally peer-reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.