Severe COVID-19 in an APS1 patient with interferon autoantibodies treated with plasmapheresis

doi:10.1016/j.jaci.2021.03.034

Case Reports

. 2021 Jul;148(1):96-98.

doi: 10.1016/j.jaci.2021.03.034. Epub 2021 Apr 16.

Severe COVID-19 in an APS1 patient with interferon autoantibodies treated with plasmapheresis

Affiliations

¹ Department of Pediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Rheumatology and Inflammation Research, Institute of Medicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. Electronic address: andri.lemarquis@gu.se.
² Centre for Hematology and Regenerative Medicine, Department of Medicine, Karolinska Institute, Stockholm, Sweden; Division of Clinical Immunology and Transfusion Medicine, Karolinska University Hospital, Stockholm, Sweden.
³ Centre for Molecular Medicine, Department of Medicine (Solna), Karolinska Institute, Stockholm, Sweden.
⁴ Department of Pediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Rheumatology and Inflammation Research, Institute of Medicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
⁵ Science for Life Laboratory, Department of Women's and Children's Health, Karolinska Institute, Solna, Sweden.
⁶ Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, the Sahlgrenska Academy at the University of Gothenburg, Mölndal, Sweden; Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital, Mölndal, Sweden.
⁷ Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, the Sahlgrenska Academy at the University of Gothenburg, Mölndal, Sweden; Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital, Mölndal, Sweden; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, United Kingdom; UK Dementia Research Institute at UCL, London, United Kingdom.
⁸ Department of Infectious Diseases, Institute of Biomedicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Clinical Microbiology, Sahlgrenska University Hospital, Gothenburg, Sweden.
⁹ Department of Rheumatology and Inflammation Research, Institute of Medicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
¹⁰ Centre for Molecular Medicine, Department of Medicine (Solna), Karolinska Institute, Stockholm, Sweden; Department of Medical Biochemistry and Microbiology, Uppsala University, Uppsala, Sweden.
¹¹ Centre for Hematology and Regenerative Medicine, Department of Medicine, Karolinska Institute, Stockholm, Sweden; Division of Clinical Immunology and Transfusion Medicine, Karolinska University Hospital, Stockholm, Sweden; Broegelmann Research Laboratory, Department of Clinical Sciences, University of Bergen, Bergen, Norway.
¹² Department of Pediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Pediatrics, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden.
¹³ Department of Pediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Rheumatology and Inflammation Research, Institute of Medicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. Electronic address: olov.ekwall@gu.se.

PMID: 33892926
PMCID: PMC8051851
DOI: 10.1016/j.jaci.2021.03.034

Case Reports

Severe COVID-19 in an APS1 patient with interferon autoantibodies treated with plasmapheresis

Andri Lemarquis et al. J Allergy Clin Immunol. 2021 Jul.

. 2021 Jul;148(1):96-98.

doi: 10.1016/j.jaci.2021.03.034. Epub 2021 Apr 16.

Authors

Affiliations

¹ Department of Pediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Rheumatology and Inflammation Research, Institute of Medicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. Electronic address: andri.lemarquis@gu.se.
² Centre for Hematology and Regenerative Medicine, Department of Medicine, Karolinska Institute, Stockholm, Sweden; Division of Clinical Immunology and Transfusion Medicine, Karolinska University Hospital, Stockholm, Sweden.
³ Centre for Molecular Medicine, Department of Medicine (Solna), Karolinska Institute, Stockholm, Sweden.
⁴ Department of Pediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Rheumatology and Inflammation Research, Institute of Medicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
⁵ Science for Life Laboratory, Department of Women's and Children's Health, Karolinska Institute, Solna, Sweden.
⁶ Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, the Sahlgrenska Academy at the University of Gothenburg, Mölndal, Sweden; Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital, Mölndal, Sweden.
⁷ Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, the Sahlgrenska Academy at the University of Gothenburg, Mölndal, Sweden; Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital, Mölndal, Sweden; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, United Kingdom; UK Dementia Research Institute at UCL, London, United Kingdom.
⁸ Department of Infectious Diseases, Institute of Biomedicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Clinical Microbiology, Sahlgrenska University Hospital, Gothenburg, Sweden.
⁹ Department of Rheumatology and Inflammation Research, Institute of Medicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
¹⁰ Centre for Molecular Medicine, Department of Medicine (Solna), Karolinska Institute, Stockholm, Sweden; Department of Medical Biochemistry and Microbiology, Uppsala University, Uppsala, Sweden.
¹¹ Centre for Hematology and Regenerative Medicine, Department of Medicine, Karolinska Institute, Stockholm, Sweden; Division of Clinical Immunology and Transfusion Medicine, Karolinska University Hospital, Stockholm, Sweden; Broegelmann Research Laboratory, Department of Clinical Sciences, University of Bergen, Bergen, Norway.
¹² Department of Pediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Pediatrics, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden.
¹³ Department of Pediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Rheumatology and Inflammation Research, Institute of Medicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. Electronic address: olov.ekwall@gu.se.

PMID: 33892926
PMCID: PMC8051851
DOI: 10.1016/j.jaci.2021.03.034

No abstract available

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Figures

**Fig 1**
A, Timeline showing interventions in our patient. B, Chest x-radiographs before start of plasmapheresis and after treatment. C, Trajectories for serum aAbs, inflammatory markers, and SARS-CoV-2 Ct values throughout the disease course. Gray bars indicate plasmapheresis. D, IFN aAbs in sera from the patient and healthy controls assessed using a bead-based array. The patient is positive for all IFN aAbs tested except for aAbs against IFN-γ and IFN-β1. E, Heatmap illustrating that IFN aAb concentrations decrease during plasmapheresis (day 12) and return to high levels after discontinuation of plasmapheresis (day 20). F, Neutralization assay for IFN-α. High-level IFN-α control and serum from a patient with COVID-19 with 0.1% serum from our patient before and after plasmapheresis. G, Effects of patient sera diluted 1:10, on the phosphorylation of STAT1 in monocytes after stimulation with either IFN-α or IFN-γ. Phosphorylation after IFN-α stimulation in presence of 0.4% patient serum during treatment. H, Proliferation of CD4⁺ and CD8⁺ T cells stimulated, separately, with SARS-CoV-2 nucleocapsid (N) and spike (S) peptides and PHA. I, T-cell proliferation and production of cytokines, after peptide N and peptide S stimulation of PBMCs, and serum concentrations of specific IgG and IgA anti–SARS-CoV-2 antibodies on days 7 and 28. *CRP*, C-reactive protein; HC, healthy control; *HFO*, high-flow oxygen; iv, intravenous, *MFI*, mean fluorescence intensity; po, *per os* (by mouth); *STAT1*, signal transducer and activator of transcription 1.

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References

1. Bastard P., Rosen L.B., Zhang Q., Michailidis E., Hoffmann H.-H., Zhang Y. Autoantibodies against type I IFNs in patients with life-threatening COVID-19. Science. 2020;370 - PMC - PubMed
1. Zhang Q., Bastard P., Liu Z., Le Pen J., Moncada-Velez M., Chen J. Inborn errors of type I IFN immunity in patients with life-threatening COVID-19. Science. 2020;370 - PMC - PubMed
1. Hadjadj J., Yatim N., Barnabei L., Corneau A., Boussier J., Smith N. Impaired type I interferon activity and inflammatory responses in severe COVID-19 patients. Science. 2020;369:718–724. - PMC - PubMed
1. Fernández-Zarzoso M., Gómez-Seguí I., de la Rubia J. Therapeutic plasma exchange: review of current indications. Transfus Apher Sci. 2019;58:247–253. - PubMed
1. Meager A., Visvalingam K., Peterson P., Möll K., Murumägi A., Krohn K. Anti-interferon autoantibodies in autoimmune polyendocrinopathy syndrome type 1. PLoS Med. 2006;3:e289. - PMC - PubMed

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[1] Bastard P., Rosen L.B., Zhang Q., Michailidis E., Hoffmann H.-H., Zhang Y. Autoantibodies against type I IFNs in patients with life-threatening COVID-19. Science. 2020;370 - PMC - PubMed

[2] Bastard P., Rosen L.B., Zhang Q., Michailidis E., Hoffmann H.-H., Zhang Y. Autoantibodies against type I IFNs in patients with life-threatening COVID-19. Science. 2020;370 - PMC - PubMed

[3] Zhang Q., Bastard P., Liu Z., Le Pen J., Moncada-Velez M., Chen J. Inborn errors of type I IFN immunity in patients with life-threatening COVID-19. Science. 2020;370 - PMC - PubMed

[4] Zhang Q., Bastard P., Liu Z., Le Pen J., Moncada-Velez M., Chen J. Inborn errors of type I IFN immunity in patients with life-threatening COVID-19. Science. 2020;370 - PMC - PubMed

[5] Hadjadj J., Yatim N., Barnabei L., Corneau A., Boussier J., Smith N. Impaired type I interferon activity and inflammatory responses in severe COVID-19 patients. Science. 2020;369:718–724. - PMC - PubMed

[6] Hadjadj J., Yatim N., Barnabei L., Corneau A., Boussier J., Smith N. Impaired type I interferon activity and inflammatory responses in severe COVID-19 patients. Science. 2020;369:718–724. - PMC - PubMed

[7] Fernández-Zarzoso M., Gómez-Seguí I., de la Rubia J. Therapeutic plasma exchange: review of current indications. Transfus Apher Sci. 2019;58:247–253. - PubMed

[8] Fernández-Zarzoso M., Gómez-Seguí I., de la Rubia J. Therapeutic plasma exchange: review of current indications. Transfus Apher Sci. 2019;58:247–253. - PubMed

[9] Meager A., Visvalingam K., Peterson P., Möll K., Murumägi A., Krohn K. Anti-interferon autoantibodies in autoimmune polyendocrinopathy syndrome type 1. PLoS Med. 2006;3:e289. - PMC - PubMed

[10] Meager A., Visvalingam K., Peterson P., Möll K., Murumägi A., Krohn K. Anti-interferon autoantibodies in autoimmune polyendocrinopathy syndrome type 1. PLoS Med. 2006;3:e289. - PMC - PubMed

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Severe COVID-19 in an APS1 patient with interferon autoantibodies treated with plasmapheresis

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Severe COVID-19 in an APS1 patient with interferon autoantibodies treated with plasmapheresis

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