Pulmonary intravascular coagulopathy in COVID-19 pneumonia
Authors: Hisyovi Cárdenas Suri
Journal: The Lancet Rheumatology (2020)

I read with interest the Viewpoint by Dennis McGonagle and colleagues,1McGonagle D O'Donnell JS Sharif K Emery P Bridgewood C Immune mechanisms of pulmonary intravascular coagulopathy in COVID-19 pneumonia.Lancet Rheumatol. 2020; (published online May 7.)https://doi.org/10.1016/S2665-9913(20)30121-1Summary Full Text Full Text PDF Scopus (582) Google Scholar in which they associate the changes that occur in the lungs of patients with COVID-19 with macrophage activation syndrome (MAS) and emphasise the possible occurrence of pre-terminal disseminated intravascular coagulation. Coagulation disorders in patients with COVID-19 were initially thought to be due to systemic disseminated intravascular coagulopathy but evidence from autopsies instead shows that these disorders are due to a procoagulant event together with a severe inflammatory state.2Panigada M Bottino N Tagliabue P et al.Hypercoagulability of COVID-19 patients in intensive care unit. A report of thromboelastography findings and other parameters of hemostasis.J Thromb Haemost. 2020; (published online Apr 17.)DOI:10.1111/jth.14850Crossref PubMed Scopus (965) Google Scholar Serum ferritin is elevated in 71% of patients with catastrophic antiphospholipid antibody syndrome. Indeed, four well recognised clinical conditions exist that might be associated with high ferritin concentrations: MAS, adult-onset Still's disease, catastrophic antiphospholipid antibody syndrome, and septic shock.3Betancur JF Navarro EP Echeverry A Moncada PA Cañas CA Tobón GJ Hyperferritinemic syndrome: Still's disease and catastrophic antiphospholipid syndrome triggered by fulminant Chikungunya infection: a case report of two patients.Clin Rheumatol. 2015; 34: 1989-1992Crossref PubMed Scopus (22) Google Scholar Adult-onset Still's disease has diagnostic criteria that do not correspond to the clinical presentation and disease course of critically ill patients with COVID-19. MAS is closely related to juvenile idiopathic arthritis, although it has been described in other autoimmune diseases such as lupus, and is seen more frequently among children and adolescents than in adults. Bleeding, however, occurs more frequently in MAS than thrombosis does.3Betancur JF Navarro EP Echeverry A Moncada PA Cañas CA Tobón GJ Hyperferritinemic syndrome: Still's disease and catastrophic antiphospholipid syndrome triggered by fulminant Chikungunya infection: a case report of two patients.Clin Rheumatol. 2015; 34: 1989-1992Crossref PubMed Scopus (22) Google Scholar, 4Ravelli A Grom AA Behrens EM Cron RQ Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment.Genes Immun. 2012; 13: 289-298Crossref PubMed Scopus (295) Google Scholar Antiphospholipid antibodies have been reported in some patients with COVID-19, and one study found a high concentration of lupus anticoagulant antibody in 50 (88%) of 57 tested individuals.5Zhang Y Xiao M Zhang S et al.Coagulopathy and antiphospholipid antibodies in patients with COVID-19.N Engl J Med. 2020; 382: e38Crossref PubMed Scopus (1624) Google Scholar When reviewing the diagnostic criteria, causes, and clinical forms of catastrophic antiphospholipid antibody syndrome, and the autopsy findings from patients with COVID-19, this syndrome is very likely to be the cause of exaggerated inflammatory response and thrombosis in most patients with severe COVID-19, even in children with severe multisystemic inflammatory response who have had an initial negative test for SARS-CoV-2. The medical community must address this diagnostic possibility since it could radically change the treatment and prognosis of critically ill patients with COVID-19. I declare no competing interests. Pulmonary intravascular coagulopathy in COVID-19 pneumoniaWe read with pleasure the thoughtful Viewpoint by Dennis McGonagle and colleagues1 on lung immunothrombosis during infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). However, some of the key pathogenetic events were not highlighted by the authors. Full-Text PDF Pulmonary intravascular coagulopathy in COVID-19 pneumoniaWe read with interest the Viewpoint by Dennis McGonagle and colleagues.1 To account for unusual clinicopathological features of COVID-19 disease, particularly coagulopathy, the authors point to dysregulated immunity and systemic inflammation reminiscent of a cytokine storm or macrophage activation syndrome (MAS). Although the authors' contribution comes down firmly on the immunological side of the debate over whether COVID-19 coagulopathy is due principally to immune or endothelial dysfunction,2 the common background assumption to both sides is that modelling COVID-19 using previously described clinical syndromes and traditional pharmacological and physiological mechanisms will lead to deeper insights into the disease. Full-Text PDF Pulmonary intravascular coagulopathy in COVID-19 pneumonia – Authors' replyWe thank Gianfranco Ferraccioli and colleagues, Brandon Reines and colleagues, and Hisyovi Cárdenas Suri for their comments on our Viewpoint1 discussing the diffuse, alveolar-centred inflammation that triggers immunothrombosis in the lung microvasculature of patients with COVID-19 pneumonia. Full-Text PDF Immune mechanisms of pulmonary intravascular coagulopathy in COVID-19 pneumoniaThe lung pathology seen in patients with coronavirus disease 2019 (COVID-19) shows marked microvascular thrombosis and haemorrhage linked to extensive alveolar and interstitial inflammation that shares features with macrophage activation syndrome (MAS). We have termed the lung-restricted vascular immunopathology associated with COVID-19 as diffuse pulmonary intravascular coagulopathy, which in its early stages is distinct from disseminated intravascular coagulation. Increased circulating D-dimer concentrations (reflecting pulmonary vascular bed thrombosis with fibrinolysis) and elevated cardiac enzyme concentrations (reflecting emergent ventricular stress induced by pulmonary hypertension) in the face of normal fibrinogen and platelet levels are key early features of severe pulmonary intravascular coagulopathy related to COVID-19. Full-Text PDF