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Anti-factor H antibody associated hemolytic uremic syndrome following SARS-CoV-2 infection.

Authors :
Khandelwal, Priyanka
Krishnasamy, Sudarsan
Govindarajan, Srinivasavaradan
Kumar, Manish
Marik, Binata
Sinha, Aditi
Hari, Pankaj
Bagga, Arvind
Source :
Pediatric Nephrology; Sep2022, Vol. 37 Issue 9, p2151-2156, 6p, 1 Chart, 1 Graph
Publication Year :
2022

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 m<superscript>2</superscript>; 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. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
0931041X
Volume :
37
Issue :
9
Database :
Complementary Index
Journal :
Pediatric Nephrology
Publication Type :
Academic Journal
Accession number :
158138166
Full Text :
https://doi.org/10.1007/s00467-021-05390-4