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Diagnosis and management of autoimmune diseases in the ICU

  • Narrative Review
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Abstract

Autoimmune diseases encompass a broad spectrum of disorders characterized by disturbed immunoregulation leading to the development of specific autoantibodies, resulting in inflammation and multiple organ involvement. A distinction should be made between connective tissue diseases (mainly systemic lupus erythematosus, systemic scleroderma, inflammatory muscle diseases, and rheumatoid arthritis) and vasculitides (mainly small-vessel vasculitis such as antineutrophil cytoplasmic antibody-associated vasculitis and immune-complex mediated vasculitis). Admission of patients with autoimmune diseases to the intensive care unit (ICU) is often triggered by disease flare-ups, infections, and organ failure and is associated with high mortality rates. Management of these patients is complex, including prompt disease identification, immunosuppressive treatment initiation, and life-sustaining therapies, and requires multi-disciplinary involvement. Data about autoimmune diseases in the ICU are limited and there is a need for multicenter, international collaboration to improve patients’ diagnosis, management, and outcomes. The objective of this narrative review is to summarize the epidemiology, clinical features, and selected management of severe systemic autoimmune diseases.

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Acknowledgements

We are grateful to Chantal Dumestre-Perard, Adrien Mirouse, and Jean-Rémi Lavillegrand for sharing their personal collection of pictures. Figures have been designed using Biorender.

Funding

FS reports obtained funding by NeuroCure Clinical Research Center (NCRC), funded by the Deutsche Forschungsgemeinschaft (DFG, German Research Foundation) under Germany´s Excellence Strategy EXC 2049-89829218.

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GD has no conflict of interest to disclose. YA has no conflict of interest to disclose. RB has received honararium from the Difficulty Airway Course. OR has no conflict of interest to disclose. MD reports having received consulting fees from Gilead-Kite, research support from MSD, and speaker fees from MSD, Gilead-Kite and Astellas. JH has received honoraria for lectures from Diagnostica Stago, Pfizer PFE France, Sanofi Aventis France, Inotrem, MSD and Shionogi, all unrelated to this work and paid to her institution.

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Dumas, G., Arabi, Y.M., Bartz, R. et al. Diagnosis and management of autoimmune diseases in the ICU. Intensive Care Med 50, 17–35 (2024). https://doi.org/10.1007/s00134-023-07266-7

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