vEDS and COVID-19

Updated: January 5, 2022

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Certain health conditions increase the risk of complications from covid-19. Some of these may be specific concerns in people with vEDS and others who have an ‘aortopathy’ (aortic dilation/aneurysm, dissection/past rupture), heart valve disorders, or restrictive breathing. These are:

In vEDS, this might include heart valve disease (regurgitation, moderate-severe prolapse, or stenosis), aneurysms/dissections (VASCERN, Vascular Disease Network, March 2020), and heart failure from rib cage deformities that are affecting the heart and/or lungs such as a severe pectus deformity.

There are many causes of lung disease that are not known to be related to having EDS or HSD. However, there are lung conditions that arise in EDS that should be considered:

  • One concern may be poor lung volumes that can occur if a person has severe kyphosis/scoliosis, or emphysema, that might increase the risk of pneumonia.
  • In addition, persistent dry cough and sudden/increasing shortness of breath could arise from a pneumothorax (sudden partial or complete collapse of a lung). A pneumothorax can be spontaneous or may arise in a person known to have cystic (bullous) lung disease. Forceful coughing from a respiratory infection may cause a pneumothorax but there is no way to know if this is a particular risk in an individual unless they have had a pneumothorax before.
  • Also, some individuals with EDS and HSD have apnea (breathing stops and starts, especially when sleeping) (Sedky K et al. J Clin Sleep Med. 2019 Feb 15;15(2):293-299. Meta-analysis). If you have this, it should be raised with an attending doctor if you are experiencing worsening breathing symptoms.
  • Symptoms of wheeze and cough are common in EDS – affecting on average 15-20% of hEDS, cEDS and vEDS individuals in a recent large survey (Sheehan TP et al. Respiratory Symptoms Among Patients with Ehlers-Danlos Syndromes (eds). ATS Conferences. 2017; C37). With regard to COVID-19, and any other respiratory infections, the change in the severity of these symptoms should be alerted to an attending doctor if a person is concerned that they are developing a respiratory infection.

Some people may be taking medications for autoimmune diseases such as steroids or disease-modifying drugs like Azathioprine or Methotrexate.

Antibiotics for Bacterial Pneumonia in vEDS and others with an ‘aortopathy’

Antibiotics do not kill viruses, but a person with a viral infection may develop an added bacterial infection. First-line antibiotics for bacterial pneumonia include: Penicillin (e.g. Amoxicillin); a Macrolide (e.g. Clarithromycin); and Tetracyclines (e.g. Doxycycline). Second line might include Fluoroquinolones (e.g. Ciprofloxacin / Levofloxacin / Norfloxacin).

The FDA, EMA, and other international medicines regulator guidance indicate that patients who have an aortic aneurysm or are at risk of an aortic aneurysm should not be prescribed fluoroquinolones unless no other suitable treatment is available.

In the Ehlers-Danlos syndromes this risk applies primarily to vascular Ehlers-Danlos syndrome [vEDS]. Sometimes individuals with other rare types of EDS may also have aortic aneurysms, or any person may have an aneurysm for another reason – the guidance applies to these people too. 

Most people with EDS (with the exception of the groups listed above) are not at increased risk of an aneurysm from their EDS.

In addition, the FDA, MHRA, and other international medicines regulators have published that, rarely, fluoroquinolones can cause serious and potentially irreversible complications that affect tendons, muscles, joints, nerves, and the central nervous system. There have been cases, for example, where tendon inflammation and tendon rupture has occurred. Notably (but not exclusively) this has occurred at the Achilles tendon. The increased risks of tendon rupture appear to be aged 60 years and over and taking corticosteroids at the same time as taking the fluoroquinolone. Research suggests that ofloxacin and norfloxacin have the greatest risk for tendon rupture (Alves et al Eur J Clin Pharmacol 2019;75:1431-1443 Meta-analysis). 

The specific risk of tendon rupture and other non-vascular side effects in EDS or HSD, compared to the general population, is not known. 

To reduce risk in the general population the guidance states that fluoroquinolones should not be used for uncomplicated infections (e.g. mild-moderate sinusitis, bronchitis, or cystitis) unless there is no other suitable class of antibiotic. 

A fluoroquinolone may be the only suitable antibiotic to fight an infection because, for example, bacteria are resistant to other types of antibiotic, or the infection is causing serious complications such as sepsis. In these situations, doctors with their patients have to determine whether the benefits of taking a fluoroquinolone outweigh the risks.

Non-Steroidal Anti-inflammatories (NSAIDs)

There has been advice in the media very recently that Ibuprofen (an NSAID) should be avoided for symptom relief from covid-19. Studies suggest ibuprofen can dampen down the immune system and slow recovery from infection. When taken during an infection Ibuprofen may cause more severe illness and complications. The advice is to use paracetamol instead.

In an NHS England statement, March 17, 2020 the advice given is that those currently on NSAIDs for other medical reasons (e.g. arthritis) should not stop them. The WHO also states (March 19, 2020) that they are not aware of any reports that ibuprofen causes the illness from covid-19 infection to be more severe.

The European Medicines Agency (EMA, 18 March 2020) has said “There is currently no scientific evidence establishing a link between ibuprofen and worsening of COVID‑19”. This has been further affirmed by the MHRA (14 April 2020).

Renin-Angiotensin Aldosterone System (RAAS) Inhibitor Treatment for Blood Pressure Control

The Renin-Angiotensin Aldosterone System (RAAS) inhibitors include the following medications:

  • ACE inhibitors – Enalapril, lisinopril, ramipril, captopril, benazepril
  • ARBs – Valsartan, candesartan, losartan, irbesartan
  • Direct renin inhibitors – Aliskiren

Researchers have concluded that there is no increased risk of complications from COVID-19 from taking a RAAS Inhibitor medication. A scientific review (Tignanelli and colleagues, March 2020) in The Lancet Respiratory Medicine, and in a study in the New England Journal of Medicine (NEJM) Reynolds and colleagues (May 2020) has looked at these concerns. A lay summary of the findings by Reynolds is discussed in a New York Times article. In addition, in the NEJM Mehra and colleagues report similar conclusions to Reynolds. Also, later in May 2020 Ackermann and colleagues report similar observations regarding vascular concerns in the lungs. These findings suggest that statins (lipid lowering drugs) and RAAS medications may protect against the vascular damage seen in severe COVID-19 disease. Clinical trials are needed to confirm this.


Some with vEDS may have additional concerns related to being pregnant. The Royal College of Obstetrics and Gynaecology, UK has given this opinion that :

“Generally, pregnant women do not appear to be more likely to be seriously unwell than other healthy adults if they develop the new coronavirus. It is expected the large majority of pregnant women will experience only mild or moderate cold/flu like symptoms” (RCOG, 9 April 2020).

An article in the New Scientist (March 16 2020)  said: 

“One reason to worry about covid-19 in pregnancy is that people are more likely to become severely ill with flu when they are pregnant…However, initial reports suggest that covid-19 might not hit pregnant women or their newborn babies too heavily.”

However, in the UK the Government has today (17th March 2020) included pregnancy as a risk and advised that people self-isolate for 12 weeks like others at risk. Information and links are on the BBC website.

The Ehlers-Danlos Society advises that you speak with your Obstetrician about how to maintain the level of monitoring you may need based on your particular case.

Gastrointestinal (GI) symptoms and risk of spreading COVID-19

The following is taken from The American College of Gastroenterology, March 16, 2020. It applies to everyone but some people with vEDS have issues with bowel function and should maintain good hygiene. 

  • There is evidence suggesting that Coronavirus could be spread from feces. 
  • Cough, fever, fatigue, or sore throat are the most common symptoms in adults.
  • But GI symptoms including nausea and / or diarrhea may also occur in some cases. 
  • The virus may be present in GI mucous secretions and feces. 
  • Gastrointestinal infection and the potential to pass on infection from fecal contamination / poor hand hygiene must be considered like any other diarrheal illness.

European Reference Networks Priorities and Contra-indications for COVD-19 Vaccines

As European Reference Networks (ERNs) are formed by experts in rare diseases, all ERNs provided their opinions on the priorities and contraindications for patients with a rare disease within their network. The results are summarized in this document and were discussed during the internal ERN-Coordinator group meeting of January 27, 2021.

Other General Advice from The Ehlers-Danlos Society

Countries and regions or states around the world are in different stages of the management of the pandemic. Individuals should follow the local official advice both with regard to emergency service and social and medical isolation policies.

The signs and symptoms to look out for in particular are fever (37.8 centigrade/100 fahrenheit or above), persistent cough, and increasing difficulty breathing. Nausea and diarrhea may also be a concern. We recommend everyone make themselves familiar with these signs, and that they also are familiar with their local emergency services recommendations as to when and how to seek help.

The ways to reduce the risk of exposure to the virus are the same for everyone, and we recommend everyone follow these protective measures from The World Health Organization.

The statement above is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.

Alan Hakim, MB BChir, MA, FRCP, Chief Medical Officer, The Ehlers-Danlos Society

Fransiska Malfait, MD, PhD, Chief Scientific Officer, The Ehlers-Danlos Society

Clair Francomano, MD, Medical and Scientific Board Chair, The Ehlers-Danlos Society

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