Flare of Antiphospholipid Syndrome in the Course of COVID-19

Abstract

We report the case of a 48-year-old man followed since 2013 for primary antiphospholipid syndrome (APLS) revealed by venous thromboembolism in the presence of antiphospholipid antibodies (APL-Abs, anticardiolipin and anti-β-2-glycoprotein-1), who decompensated in the course of coronavirus disease (COVID-19). Despite efficient anticoagulation, he suffered bilateral adrenal glands hemorrhage and limb arterial ischemia. The tropism of severe acute respiratory syndrome coronavirus-2 for endothelium can lead to microangiopathy and increased risk for thrombosis. If APL-Abs positivity can be an epiphenomenon under inflammatory and prothrombotic conditions, COVID-19 was herein responsible for disseminated thrombosis and a threat of catastrophic APLS, despite efficient anticoagulation.

Keywords

COVID-19 - infection - thrombosis - antiphospholipid syndrome - hydroxychloroquine

Authors' Contributions

Each author contributed to the patient care. A.T.J.M. and P.G. wrote the first draft, and all the authors proofread it. The same was accepted in its last version.

Publikationsverlauf

Eingereicht: 03. Mai 2020

Angenommen: 12. August 2020

Artikel online veröffentlicht:
11. September 2020

• References

• 1 Willis R, Pierangeli SS. Pathophysiology of the antiphospholipid antibody syndrome. Auto Immun Highlights 2011; 2 (02) 35-52
• 2 Zhang Y, Xiao M, Zhang S. , et al. Coagulopathy and antiphospholipid antibodies in patients with Covid-19. N Engl J Med 2020; 382 (17) e38
• 3 Connors JM, Levy JH. Thromboinflammation and the hypercoagulability of COVID-19. J Thromb Haemost 2020; 18 (07) 1559-1561
• 4 Bangalore S, Sharma A, Slotwiner A. , et al. ST-segment elevation in patients with Covid-19 - a case series. N Engl J Med 2020; 382 (25) 2478-2480
• 5 Cui S, Chen S, Li X, Liu S, Wang F. Prevalence of venous thromboembolism in patients with severe novel coronavirus pneumonia. J Thromb Haemost 2020; 18 (06) 1421-1424
• 6 Lillicrap D. Disseminated intravascular coagulation in patients with 2019-nCoV pneumonia. J Thromb Haemost 2020; 18 (04) 786-787
• 7 Tang N, Li D, Wang X, Sun Z. Abnormal coagulation parameters are associated with poor prognosis in patients with novel coronavirus pneumonia. J Thromb Haemost 2020; 18 (04) 844-847
• 8 Thachil J, Tang N, Gando S. , et al. ISTH interim guidance on recognition and management of coagulopathy in COVID-19. J Thromb Haemost 2020; 18 (05) 1023-1026
• 9 Barton LM, Duval EJ, Stroberg E, Ghosh S, Mukhopadhyay S. COVID-19 autopsies, Oklahoma, USA. Am J Clin Pathol 2020; 153 (06) 725-733
• 10 Gianotti R, Veraldi S, Recalcati S, Cusini M, Ghislanzoni M, Boggio F, Fox LP. Cutaneous clinico-pathological findings in three COVID-19-positive patients observed in the metropolitan area of Milan, Italy. Acta Derm Venereol 2020; 100 (08) adv00124
• 11 Magro C, Mulvey JJ, Berlin D. , et al. Complement associated microvascular injury and thrombosis in the pathogenesis of severe COVID-19 infection: a report of five cases. Transl Res 2020; 220: 1-13
• 12 Gao T, Hu M, Zhang X. , et al. Highly pathogenic coronavirus N protein aggravates lung injury by MASP-2-mediated complement over-activation. Infectious Diseases (except HIV AIDS) 2020 (preprint). Available at: https://www.medrxiv.org/content/10.1101/2020.03.29.20041962v2.full.pdf. Accessed September 2, 2020
• 13 Cugno M, Meroni PL, Gualtierotti R. , et al. Complement activation in patients with COVID-19: a novel therapeutic target. J Allergy Clin Immunol 2020; 146 (01) 215-217
• 14 Chauhan AJ, Wiffen LJ, Brown TP. COVID-19: a collision of complement, coagulation and inflammatory pathways. J Thromb Haemost 2020
• 15 Risitano AM, Mastellos DC, Huber-Lang M. , et al. Complement as a target in COVID-19?. Nat Rev Immunol 2020; 20 (06) 343-344
• 16 Diurno F, Numis FG, Porta G. , et al. Eculizumab treatment in patients with COVID-19: preliminary results from real life ASL Napoli 2 Nord experience. Eur Rev Med Pharmacol Sci 2020; 24 (07) 4040-4047
• 17 Smith K, Pace A, Ortiz S, Kazani S, Rottinghaus S. A phase 3 open-label, randomized, controlled study to evaluate the efficacy and safety of intravenously administered ravulizumab compared with best supportive care in patients with COVID-19 severe pneumonia, acute lung injury, or acute respiratory distress syndrome: a structured summary of a study protocol for a randomised controlled trial. Trials 2020; 21 (01) 639
• 18 Miyakis S, Lockshin MD, Atsumi T. , et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006; 4 (02) 295-306
• 19 Sène D, Piette J-C, Cacoub P. Antiphospholipid antibodies, antiphospholipid syndrome and infections. Autoimmun Rev 2008; 7 (04) 272-277
• 20 Harzallah I, Debliquis A, Drénou B. Lupus anticoagulant is frequent in patients with Covid-19. J Thromb Haemost 2020; 18 (08) 2064-2065
• 21 Devreese KMJ, Linskens EA, Benoit D, Peperstraete H. Antiphospholipid antibodies in patients with COVID-19: a relevant observation?. J Thromb Haemost 2020;
• 22 Pineton de Chambrun M, Frere C, Miyara M. , et al. High frequency of antiphospholipid antibodies in critically ill COVID-19 patients: a link with hypercoagulability?. J Intern Med 2020;
• 23 Siguret V, Voicu S, Neuwirth M. , et al. Are antiphospholipid antibodies associated with thrombotic complications in critically ill COVID-19 patients?. Thromb Res 2020; 195: 74-76
• 24 Xiao M, Zhang Y, Zhang S. , et al. Brief report: anti-phospholipid antibodies in critically ill patients with coronavirus disease 2019 (COVID-19). Arthritis Rheumatol 2020;
• 25 Monteil V, Kwon H, Prado P. , et al. Inhibition of SARS-CoV-2 infections in engineered human tissues using clinical-grade soluble human ACE2. Cell 2020; 181 (04) 905-913.e7
• 26 Varga Z, Flammer AJ, Steiger P. , et al. Endothelial cell infection and endotheliitis in COVID-19. Lancet 2020; 395 (10234): 1417-1418