COVID-19 and Catastrophic Antiphospholipid Syndrome

*Hisyovi Cardenas Suri, David Jimomila Bening and Benjamín Demah Nuertey*

### **Abstract**

One year after the beginning of the epidemic, mortality continues to be high despite several different protocols being tried. Critical patients with Covid 19 in some degree of organ failure and thrombotic events meet the diagnostic criteria of a complete or incomplete catastrophic antiphospholipid syndrome (CAPS) or at least we may need to consider a partial form of it. The findings of autopsies and the involvement of different organs and systems are similar to those of CAPS. Currently the only therapy that has been shown to reduce mortality include steroids, anticoagulation and an antinuclear antibody. The same therapy has been shown to be effective for CAPS.

**Keywords:** COVID-19, multiorgan failure, thrombosis, antiphospholipid antibodies, catastrophic antiphospholipid antibodies syndrome

#### **1. Introduction**

After the COVID-19 outbreak in December 2019, a clinical picture was identified in most critical patients that was diagnosed as a cytokine storm associated with high mortality. Hypoxemia was also justified in those patients with severe ARDS who did not respond to the usual ventilation maneuvers such as a reflex of pulmonary hypoxic vasoconstriction, which subsequently lost value or there were no more reports in this regard when thrombosis of pulmonary microvasculature was demonstrated.

After more than a year the medical community has been fighting COVID-19 and having published a number of articles perhaps like never before talking about the same subject in such a short time. We continue with almost the same mortality and with many unanswered questions about the fatal presentation with other systemic manifestations of the disease in patients who develop a serious clinical picture [1].

Obesity, hypertension, diabetes mellitus, cancer, and other chronic diseases have been clearly identified as risk factors for developing severe disease with complications. Just as after large studies it has been shown that the use of steroids, anticoagulation and antinuclear antibodies considerably reduce mortality, being the only recommended treatment in critical patients. Antiviral treatment has been shown to help in 'converting' SAR-COV-2 virus positive patient into having a negative PCR result but has not been shown to modify mortality from the disease [2].

Severe and critical cases of COVID-19 generally present with multi-organ failure, evidence of thrombosis, marked elevation of ferritin, cytokine storm, some patients with DIC but this is generally not a frequent event in the final stages of the disease.

Since March 2020, upon seeing this clinical presentation that appeared suddenly with high mortality, we began to raise the possibility initially in The Lancet rheumatologic, later in Expert Review of Respiratory Medicine about the possibility of taking into account the diagnosis of Catastrophic Antiphospholipid Antibody Syndrome (CAPS). However despite several studies having been carried out, especially relating to the presence or absence of antinuclear antibodies in patients with COVID-19, we have not found any that has made this diagnosis even after meeting the criteria, much less has it been treated as such. However, drugs have been used in COVID-19 that have been shown to reduce mortality in that are part of the CAPS therapeutic arsenal, even in children with multisystemic inflammatory syndrome, immunoglobulins have been used with good results [3].

The presence of antiphospholipid antibodies have been reported in patients with COVID-19 with a percentage that ranges from 9–96% depending on severity of condition and the presence of thrombosis. Overall figures are around 54%, however, in few studies its presence is associated with failure of more than three organs, thrombosis, and even elevated ferritin which meets the criteria for making a diagnosis of CAPS [4].

Catastrophic Antiphospholipid Antibody Syndrome or Asherson's syndrome to honor Ronald A. Asherson for his impressive work on this condition was named in 1992 when catastrophic was added to define an accelerated form of the antiphospholipid syndrome (APS). CAPS is a rare phenomenon, according to the CAPS registry, it occurs in around 1% of all antiphospholipid syndromes, however, since it is a little-studied entity and at the same time little known by doctors, we infer that it is underdiagnosed [5].
