CORONAVIRUS AND THE ANTIPHOSPHOLIPID (HUGHES’) SYNDROME (APS)

INFORMATION FOR PATIENTS

Written by Dr Arvind Kaul,
Consultant Rheumatologist and Expert Advisor to GHIC

Last updated: June 2020

The last 4 months have been difficult for everyone around the world. A pandemic which few could have foreseen causing global personal and economic catastrophe was far off everyone’s mind as New Year’s resolutions were made (and quite probably broken by January 2nd!).

There will be concerns from many of our readers about their diagnosis of Antiphospholipid (Hughes’) Syndrome (APS), other diagnoses which they also have and the risk and effects of Coronavirus infection. The purpose of this article is to summarise what is known and to frame that in the context of what patients with APS should be doing. This is especially important as severe coronavirus infection has been associated with thrombosis (blood clots), especially in areas of inflamed tissue such as the lungs (Pulmonary Embolus)

What is Coronavirus?

Novel Coronavirus (Severe Acute Respiratory Syndrome virus or SARS-2) is a micro-organism which is highly infectious and causes covid-19 (Coronavirus Disease-2019). It is related to a virus, SARS-1 which caused an outbreak of respiratory illness between 2003-4. Interestingly, no other cases of SARS-1 have been reported since 2004. It is thought these organisms are transmitted from other animals including Palm Civets and bats, to humans.

What are the symptoms?

It is important to remember that the vast majority of people who catch the virus, perhaps 80%, will not even need to see a doctor.  Mild symptoms start between 2-14 days after infection and range from temperatures, loss of smell or taste, sore throat, aches, to diarrhoea and vomiting. If you have these symptoms, you should self-isolate for 14 days and arrange for others in your household to do the same.

What if my symptoms progress?

If your symptoms are not mild, then you may have high fevers, shivering, cough and shortness of breath. You should call 111 if this happens. In general, although anyone can catch and transmit the virus, severe covid-19 infection is more likely if you are >70, male, have heart disease or diabetes. Patients from ethnic minority backgrounds may also be in this higher risk category.

What is the treatment for coronavirus infection?

There is no treatment which is known to cure covid-19 infection as yet. The vast majority of people will need no treatment apart from measures such as paracetamol, fluids and nutritional support and will recover without any issues. Medications have been used to treat more serious cases where the virus causes the body to release high levels of pro-inflammatory chemicals (cytokines). It is thought these chemicals may encourage damage to organs such as the lungs and kidneys. Although medications could reduce the levels of these cytokines and promote recovery in severe covid-19 infection, which one’s work if any, and which are best is still far from clear.

One medication which has been tested is the antimalarial drug Hydroxychloroquine, used by many patients with APS and SLE (Lupus). Hydroxychloroquine may inhibit virus reproduction in laboratories and early reports from China suggested it may improve the outcome from severe coronavirus infection in patients. More recent reports using very high doses (800mg) in the UK based RECOVERY study contradict these findings and suggest that unfortunately Hydroxychloroquine does not appear to be protective in patients with severe coronavirus infection. It is therefore important that this drug is not started or taken specifically for coronavirus. It must be continued if you take it for other conditions especially lupus where Hydroxychloroquine 200-400mg daily is protective against flares and may reduce the liklihood of strokes and heart disease.

What should I do about my medications?

For all patients with APS, you must remain on all your medications at the same level unless your doctor or specialist advise otherwise. Warfarin or other similar drugs such as acenocoumarin, heparin, and newer blood thinning agents such as Rivaroxaban, Apixiban or Dabigatran, should continue as before. If you are unfortunate enough to contract coronavirus, speak with your local Rheumatologist or Haematologist who will be best placed to advise you but currently, these anticoagulants have no known effect on the rate or severity of coronavirus infection and should not be stopped without good reason, most commonly bleeding.

What if I am hospitalised with coronavirus infection?

You must let your hospital team know about all of your medications. These may need to be continued or changed depending on your condition. In general, hospital inpatients are given prophylaxis against thrombosis with heparin, so if you are on oral anticoagulation already, this will continue or may be changed to heparin injections and will be closely monitored according to the drug you are on to reduce the risk of thrombosis.

Does Coronavirus cause Antiphospholipid (Hughes’) Syndrome?

While the risk of thrombosis from mild covid-19 infection is not known as most people are not tested to confirm the diagnosis, severe covid-19 infection is associated with a high rate of blood clots. Although 28% of non-covid patients may have thrombosis in ITU settings, once heparin prophylaxis is given, this rate halves to around 14%.

Estimates suggest between 20-49% of severe covid-19 patients in intensive care settings have thrombosis despite prophylactic heparin so the rate seems to be much higher than expected. Covid-19 thrombosis occurs especially in the lungs, where CT scans show Pulmonary Embolus associated with severe inflammation but can occur in other areas including the brain (strokes) and heart (heart attacks).

What causes these blood clots? Severe covid-19 infection is associated with very high levels of inflammatory cytokines, leading to higher levels of fibrinogen, a pro-sticky blood component. This leads to a higher risk for thrombosis with the pattern of thrombosis similar to a condition called Disseminated Intravascular Coagulation (DIC). DIC can occur in many other infections but also with cancer, childbirth and surgery.

The pattern of thrombosis in DIC can sometimes be similar to Antiphospholipid (Hughes’) Syndrome which relies on thrombosis and one or more of three routine blood tests (Lupus Anticoagulant, Anticardiolipin antibodies and b2-Glycoprotein-1) being positive. There have been reports of Antiphospholipid antibodies being present in patients with severe covid-19 infection. One study in China found Anticardiolipin and b2-Glycoprotein-1 antibodies were present in 3 patients who had blood clots. Lupus Anticoagulant was not found. However, another study found Lupus Anticoagulant in 45% of 56 patients with coronavirus infection and Anticardiolipin or b2-Glycoprotein-1 antibodies in 10% but no mention was made of whether any of these patients even had thrombosis. Another study found about 8% of patients with confirmed thrombosis had positive but low levels of one of the antiphospholipid antibody tests but none had more than one antibody.

This following points are important in interpreting any data and should provide reassurance to patients with APS concerned about covid-19 infection.

  1. Thrombosis generally occurs only in very severe covid-19 in ITU which comprise a very small minority of all coronavirus infections.
  2. Studies on antiphospholipid antibodies thus far have too few patients for their data to be considered reliable and these antibodies are likely to be an innocuous side effect.
  3. Many infections can provoke antiphospholipid antibodies and most never cause thrombosis.
  4. Some of the patients in these studies may have had antiphospholipid antibodies even before coronavirus infection. Perhaps 5% of people have these antibodies anyway, their levels can rise with age and reassuringly the vast majority do not develop any thrombosis.
  5. Treatment with anticoagulation in APS patients will continue in hospital and may potentially reduce the risk of thrombosis.

In summary, currently, there is no good evidence that covid-19 infection causes APS. Antibodies may occur, much as they do in other infections as an innocuous side effect rather than a cause of thrombosis. Our advice is to continue taking your regular medication, to ensure you socially distance and wear a face mask outdoors to reduce any risk of infection.

Does having APS and previous DVTS make you high risk if you contract Covid-19? 

No, there is no evidence that patients with previous APS or previous DVT’s are at higher risk of contracting Covid-19.

Some people with APS have received shielding letters, some haven’t. I have APS, should I be shielding?

If you look on the British Society for Rheumatology website, you will be able to score yourself as to whether shielding is needed. The BSR provide a scoring grid which accommodates medical conditions and immune suppressing drugs. In general there is no data on whether aps patients are at higher risk of Covid-19, (and it is unlikely to be the case that they are unless they have other risks such as heart disease, are >70, male, have diabetes) but there is some data suggesting that patients on immune suppressing drugs are not at higher risk of hospitalisation if they catch coronavirus. The exceptions are steroids taken long term >10mg daily. As with everyone, it is best to practice safe social distancing and wear a facemask, avoid crowded places such as shops as far as is possible.

Do you have a question about Covid-19 and APS?