IMCA1 V1 [COVID-19]

• Research type

  Research Study

• Full title

  Impact of COVID-19 infection in patients with alpha-1 antitrypsin deficiency

• IRAS ID

  285484

• Contact name

  David Parr

• Contact email

  David.Parr@uhcw.nhs.uk

• Sponsor organisation

  University Hospitals Coventry and Warwickshire NHS Trust

• Duration of Study in the UK

  0 years, 6 months, 31 days

• Research summary

  Alpha-1 antitrypsin deficiency (AATD) is a rare inherited condition caused by defects in the genes that code for the protein alpha-1 antitrypsin (AAT). The normal function of AAT is to inhibit excessive inflammation. AATD leads to low blood levels of the protein. Severe deficiency of AAT is most commonly associated with inheritance of two Z genes (PiZZ) and can cause severe lung and and liver disease. The lung disease arises from reduced ability to limit inflammatory damage in the lung, such as is caused by cigarette smoke and respiratory infections, leading to greater lung damage than occurs in healthy individuals and can significantly shorten life expectancy. Treatment with human AAT as regular infusions reduces the rate of emphysema progression and is available in several EU countries but has not been approved for reimbursement in the UK and is not available to NHS patients. Treatment is, therefore, limited to symptomatic therapies and avoidance of risk factors.
  COVID-19 is a novel coronavirus that has caused nearly 400,000 deaths worldwide. The disease mechanisms leading to overwhelming infection and respiratory failure remain poorly understood. Patients with pre-existing respiratory disease are believed to be at increased risk compared to the general population but accurate calculations of risk are not yet possible. The evidence available to assess the level of risk and the outcome of infection with COVID-19 in AATD patients is extremely limited. Most patients have adhered to strict shielding practices, which has limited their exposure to COVID-19. Consequently, the incidence of infection in AATD patients seems to be low but sporadic reports suggest that the early outcomes are not as poor as initially believed. The IMCA1 study aims to collate information on experience of COVID-19 infection in patients with AATD across the EU using a pre-existing consortium of AATD researchers.

• REC name

  N/A

• REC reference

  N/A