Anti-factor H antibody associated hemolytic uremic syndrome following SARS-CoV-2 infection.
Pediatr Nephrol
; 37(9): 2151-2156, 2022 09.
Article
in English
| MEDLINE | ID: covidwho-1653488
ABSTRACT
BACKGROUND:
The pathogenesis of autoantibody generation in anti-factor H (FH) antibody associated atypical hemolytic uremic syndrome (aHUS) is unknown and is perhaps triggered by an infectious or environmental agent. We observed an unusual increase of patients with anti-FH antibody associated aHUS coinciding with the second pandemic wave in New Delhi and suspected that SARS-CoV-2 infection might be a potential trigger.METHODS:
We screened for SARS-CoV-2 infection using reverse transcriptase polymerase chain reaction (RT-PCR) and serology in 13 consecutive patients with anti-FH antibody associated aHUS during the past year in New Delhi.RESULTS:
We report 5 patients, 4-13 years old, who presented with a febrile illness without respiratory symptoms during the second pandemic wave. Of these, 3 patients presented with a relapse 25-85 months following the initial episode of aHUS. SARS-CoV-2 was detected by RT-PCR in 1 patient and by serology in 4 patients (median titer 47.1 cut-off index). Patients had high titers of anti-FH antibodies (median 2,300 AU/ml). Genetic studies, done in 3 of the 5 patients, showed homozygous CFHR1 deletion without other significant genetic abnormalities. Specific management comprised plasma exchanges and oral prednisolone, combined with either cyclophosphamide or mycophenolate mofetil. At median follow-up of 3.3 months, the estimated glomerular filtration rate in 4 patients ranged from 62 to 110 ml/min/1.73 m2; one patient was dialysis-dependent.CONCLUSION:
Increased vigilance is required during the pandemic, especially in patients with anti-FH associated aHUS, who might relapse despite quiescent disease for a prolonged period. A higher resolution version of the Graphical abstract is available as Supplementary information.Keywords
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Atypical Hemolytic Uremic Syndrome
/
COVID-19
Type of study:
Case report
/
Cohort study
/
Diagnostic study
/
Prognostic study
Topics:
Long Covid
Limits:
Adolescent
/
Child
/
Child, preschool
/
Humans
Language:
English
Journal:
Pediatr Nephrol
Journal subject:
Nephrology
/
Pediatrics
Year:
2022
Document Type:
Article
Affiliation country:
S00467-021-05390-4
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