liu.seSearch for publications in DiVA
Change search
CiteExportLink to record
Permanent link

Direct link
Cite
Citation style
  • apa
  • harvard1
  • ieee
  • modern-language-association-8th-edition
  • vancouver
  • oxford
  • Other style
More styles
Language
  • de-DE
  • en-GB
  • en-US
  • fi-FI
  • nn-NO
  • nn-NB
  • sv-SE
  • Other locale
More languages
Output format
  • html
  • text
  • asciidoc
  • rtf
Active NET formation in Libman-Sacks endocarditis without antiphospholipid antibodies: A dramatic onset of systemic lupus erythematosus
Linköping University, Department of Medical and Health Sciences, Division of Drug Research. Linköping University, Faculty of Medicine and Health Sciences.
Linköping University, Department of Clinical and Experimental Medicine, Division of Neuro and Inflammation Science. Linköping University, Faculty of Medicine and Health Sciences. Region Östergötland, Center for Diagnostics, Department of Clinical Immunology and Transfusion Medicine.
Friedrich Alexander Univ Erlangen Nurnberg FAU, Germany.
Friedrich Alexander Univ Erlangen Nurnberg FAU, Germany; Danylo Halytsky Lviv Natl Med Univ, Ukraine.
Show others and affiliations
2018 (English)In: Autoimmunity, ISSN 0891-6934, E-ISSN 1607-842X, Vol. 51, no 6, p. 310-318Article in journal (Refereed) Published
Abstract [en]

Although neutrophil extracellular traps (NETs) have been highlighted in several systemic inflammatory diseases, their clinical correlates and potential pathological role remain obscure. Herein, we describe a dramatic onset of systemic lupus erythematosus (SLE) with clear-cut pathogenic implications for neutrophils and NET formation in a young woman with cardiac (Libman-Sacks endocarditis) and central nervous system (psychosis and seizures) involvement. Despite extensive search, circulating antiphospholipid autoantibodies, a hallmark of Libman-Sacks endocarditis, could not be detected. Instead, we observed active NET formation in the tissue of the mitral valve, as well as in the circulation. Levels of NET remnants were significantly higher in serially obtained sera from the patient compared with sex-matched blood donors (p=.0011), and showed a non-significant but substantial correlation with blood neutrophil counts (r=0.65, p=.16). The specific neutrophil elastase activity measured in serum seemed to be modulated by the provided immunosuppressive treatment. In addition, we found anti-Ro60/SSA antibodies in the cerebrospinal fluid of the patient but not NET remnants or increased elastase activity. This case illustrates that different disease mechanisms mediated via autoantibodies can occur simultaneously in SLE. NET formation with release of cytotoxic NET remnants is a candidate player in the pathogenesis of this non-canonical form of Libman-Sacks endocarditis occurring in the absence of traditional antiphospholipid autoantibodies. The case description includes longitudinal results with clinical follow-up data and a discussion of the potential roles of NETs in SLE.

Place, publisher, year, edition, pages
TAYLOR & FRANCIS LTD , 2018. Vol. 51, no 6, p. 310-318
Keywords [en]
Anti-dsDNA antibodies; elastase activity; Libman-Sacks endocarditis; neuropsychiatric lupus; neutrophil extracellular traps; NET remnants; systemic lupus erythematosus
National Category
Rheumatology and Autoimmunity
Identifiers
URN: urn:nbn:se:liu:diva-153719DOI: 10.1080/08916934.2018.1514496ISI: 000452838700007PubMedID: 30369267OAI: oai:DiVA.org:liu-153719DiVA, id: diva2:1276175
Note

Funding Agencies|Swedish Rheumatism Association; Swedish Society of Medicine; King Gustaf Vs 80-year Foundation; King Gustaf V and Queen Victorias Freemasons Foundation; Ingrid Asp Foundation; County Council of Ostergotland; SFFR of Ukraine; German Research Foundation (DFG) [CRC1181, GRK1660]; EU [690836 PANG]; Volkswagen-Stiftung [90361]

Available from: 2019-01-07 Created: 2019-01-07 Last updated: 2019-03-22

Open Access in DiVA

The full text will be freely available from 2019-10-28 14:40
Available from 2019-10-28 14:40

Other links

Publisher's full textPubMed

Search in DiVA

By author/editor
Appelgren, DanielDahle, CharlotteWetterö, JonasHöög, AndersSjöwall, Christopher
By organisation
Division of Drug ResearchFaculty of Medicine and Health SciencesDivision of Neuro and Inflammation ScienceDepartment of Clinical Immunology and Transfusion MedicineDivison of NeurobiologyClinical pathologyDepartment of Rheumatology
In the same journal
Autoimmunity
Rheumatology and Autoimmunity

Search outside of DiVA

GoogleGoogle Scholar

doi
pubmed
urn-nbn

Altmetric score

doi
pubmed
urn-nbn
Total: 359 hits
CiteExportLink to record
Permanent link

Direct link
Cite
Citation style
  • apa
  • harvard1
  • ieee
  • modern-language-association-8th-edition
  • vancouver
  • oxford
  • Other style
More styles
Language
  • de-DE
  • en-GB
  • en-US
  • fi-FI
  • nn-NO
  • nn-NB
  • sv-SE
  • Other locale
More languages
Output format
  • html
  • text
  • asciidoc
  • rtf