tetano
Editor, Senior Moderator
Pediatr Nephrol
. 2022 Jan 28.
doi: 10.1007/s00467-021-05390-4. Online ahead of print.
Anti-factor H antibody associated hemolytic uremic syndrome following SARS-CoV-2 infection
Priyanka Khandelwal[SUP] 1 [/SUP], Sudarsan Krishnasamy[SUP] 1 [/SUP], Srinivasavaradan Govindarajan[SUP] 1 [/SUP], Manish Kumar[SUP] 2 [/SUP], Binata Marik[SUP] 1 [/SUP], Aditi Sinha[SUP] 1 [/SUP], Pankaj Hari[SUP] 1 [/SUP], Arvind Bagga[SUP] 3 [/SUP]
Affiliations
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[SUP]2[/SUP]; 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: Alternative complement pathway; Complement factor H related protein; Coronavirus 19; Factor H; Thrombotic microangiopathy.
. 2022 Jan 28.
doi: 10.1007/s00467-021-05390-4. Online ahead of print.
Anti-factor H antibody associated hemolytic uremic syndrome following SARS-CoV-2 infection
Priyanka Khandelwal[SUP] 1 [/SUP], Sudarsan Krishnasamy[SUP] 1 [/SUP], Srinivasavaradan Govindarajan[SUP] 1 [/SUP], Manish Kumar[SUP] 2 [/SUP], Binata Marik[SUP] 1 [/SUP], Aditi Sinha[SUP] 1 [/SUP], Pankaj Hari[SUP] 1 [/SUP], Arvind Bagga[SUP] 3 [/SUP]
Affiliations
- PMID: 35089377
- DOI: 10.1007/s00467-021-05390-4
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[SUP]2[/SUP]; 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: Alternative complement pathway; Complement factor H related protein; Coronavirus 19; Factor H; Thrombotic microangiopathy.