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Active NET formatio...
Active NET formation in Libman–Sacks endocarditis without antiphospholipid antibodies : A dramatic onset of systemic lupus erythematosus
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- Appelgren, Daniel (author)
- Linköpings universitet,Linköping University,Avdelningen för läkemedelsforskning,Medicinska fakulteten
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- Dahle, Charlotte (author)
- Linköpings universitet,Linköping University,Avdelningen för neuro- och inflammationsvetenskap,Medicinska fakulteten,Region Östergötland, Klinisk immunologi och transfusionsmedicin
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- Knopf, Jasmin (author)
- University Hospital Erlangen,Friedrich Alexander Univ Erlangen Nurnberg FAU, Germany
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- Bilyy, Rostyslav (author)
- University Hospital Erlangen,Friedrich Alexander Univ Erlangen Nurnberg FAU, Germany; Danylo Halytsky Lviv Natl Med Univ, Ukraine
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- Vovk, Volodymyr (author)
- Danylo Halytsky Lviv National Medical University,Danylo Halytsky Lviv Natl Med Univ, Ukraine
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- Sundgren, Pia C. (author)
- Lund University,Lunds universitet,Diagnostisk radiologi, Lund,Sektion V,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Diagnostic Radiology, (Lund),Section V,Department of Clinical Sciences, Lund,Faculty of Medicine,Skåne University Hospital,Lund Univ, Sweden
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- Bengtsson, Anders A. (author)
- Lund University,Lunds universitet,Reumatologi och molekylär skelettbiologi,Sektion III,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Rheumatology,Section III,Department of Clinical Sciences, Lund,Faculty of Medicine,Lund Univ, Sweden
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- Wetterö, Jonas (author)
- Linköpings universitet,Linköping University,Avdelningen för neuro- och inflammationsvetenskap,Medicinska fakulteten
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- Muñoz, Luis E. (author)
- University Hospital Erlangen,Friedrich Alexander Univ Erlangen Nurnberg FAU, Germany
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- Herrmann, Martin (author)
- University Hospital Erlangen,Friedrich Alexander Univ Erlangen Nurnberg FAU, Germany
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- Höög, Anders (author)
- Linköpings universitet,Karolinska Institutet,Linköping University,Karolinska University Hospital,Avdelning för neurobiologi,Medicinska fakulteten,Region Östergötland, Klinisk patologi,Karolinska Inst, Sweden
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- Sjöwall, Christopher (author)
- Linköpings universitet,Linköping University,Avdelningen för neuro- och inflammationsvetenskap,Medicinska fakulteten,Region Östergötland, Reumatologiska kliniken i Östergötland
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(creator_code:org_t)
- 2018-10-28
- 2018
- English.
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In: Autoimmunity. - : Informa UK Limited. - 0891-6934 .- 1607-842X. ; 51:6, s. 310-318
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Abstract
Subject headings
Close
- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Reumatologi och inflammation (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Rheumatology and Autoimmunity (hsv//eng)
Keyword
- Anti-dsDNA antibodies
- elastase activity
- Libman–Sacks endocarditis
- NET remnants
- neuropsychiatric lupus
- neutrophil extracellular traps
- systemic lupus erythematosus
- Anti-dsDNA antibodies; elastase activity; Libman-Sacks endocarditis; neuropsychiatric lupus; neutrophil extracellular traps; NET remnants; systemic lupus erythematosus
Publication and Content Type
- art (subject category)
- ref (subject category)
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To the university's database
- By the author/editor
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Appelgren, Danie ...
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Dahle, Charlotte
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Knopf, Jasmin
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Bilyy, Rostyslav
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Vovk, Volodymyr
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Sundgren, Pia C.
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show more...
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Bengtsson, Ander ...
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Wetterö, Jonas
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Muñoz, Luis E.
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Herrmann, Martin
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Höög, Anders
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Sjöwall, Christo ...
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Rheumatology and ...
- Articles in the publication
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Autoimmunity
- By the university
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Lund University
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Karolinska Institutet
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Linköping University