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Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK.

Adrian M ShieldsAriharan AnantharachaganGururaj ArumugakaniKenneth BakerSameer BahalHelen BaxendaleWilliam BerminghamMalini BholeEvon BoulesPhilip BrightCharu ChopraLucy CliffeBetsy CleaveJohn DempsterLisa DevlinFatima DhallaLavanya DiwakarElizabeth DreweChristopher DuncanMagdalena DziadzioSuzanne ElcombeShuayb ElkhalifaAndrew GenneryHarichandrana GhantaSarah GoddardSofia GrigoriadouScott HackettGrant HaymanRichard HerriotArchana HerwadkarAarnoud HuissoonRashmi JainStephen JollesSarah JohnstonSujoy KhanJames LaffanPeter LaneLucy LeemanDavid M LoweShanti MahabirDylan James Mac LochlainnElizabeth McDermottSiraj MisbahFiona MoghaddasHadeil MorsiSai MurngSadia NooraniRachael O'BrienSmita PatelArthur PriceTasneem RahmanSuranjith SeneviratneAnna ShrimptonCatherine StroudMoira ThomasKatie TownsendPrashantha VaitlaNisha VermaAnthony WilliamsSiobhan O BurnsSinisa SavicAlex G Richter
Published in: Clinical and experimental immunology (2022)
In March 2020, the United Kingdom Primary Immunodeficiency Network (UKPIN) established a registry of cases to collate the outcomes of individuals with PID and SID following SARS-CoV-2 infection and treatment. A total of 310 cases of SARS-CoV-2 infection in individuals with PID or SID have now been reported in the UK. The overall mortality within the cohort was 17.7% (n = 55/310). Individuals with CVID demonstrated an infection fatality rate (IFR) of 18.3% (n = 17/93), individuals with PID receiving IgRT had an IFR of 16.3% (n = 26/159) and individuals with SID, an IFR of 27.2% (n = 25/92). Individuals with PID and SID had higher inpatient mortality and died at a younger age than the general population. Increasing age, low pre-SARS-CoV-2 infection lymphocyte count and the presence of common co-morbidities increased the risk of mortality in PID. Access to specific COVID-19 treatments in this cohort was limited: only 22.9% (n = 33/144) of patients admitted to the hospital received dexamethasone, remdesivir, an anti-SARS-CoV-2 antibody-based therapeutic (e.g. REGN-COV2 or convalescent plasma) or tocilizumab as a monotherapy or in combination. Dexamethasone, remdesivir, and anti-SARS-CoV-2 antibody-based therapeutics appeared efficacious in PID and SID. Compared to the general population, individuals with PID or SID are at high risk of mortality following SARS-CoV-2 infection. Increasing age, low baseline lymphocyte count, and the presence of co-morbidities are additional risk factors for poor outcome in this cohort.
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