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Active NET formation in Libman-Sacks endocarditis without antiphospholipid antibodies: A dramatic onset of systemic lupus erythematosus
Linköpings universitet, Institutionen för medicin och hälsa, Avdelningen för läkemedelsforskning. Linköpings universitet, Medicinska fakulteten.
Linköpings universitet, Institutionen för klinisk och experimentell medicin, Avdelningen för neuro- och inflammationsvetenskap. Linköpings universitet, Medicinska fakulteten. Region Östergötland, Diagnostikcentrum, Klinisk immunologi och transfusionsmedicin.
Friedrich Alexander Univ Erlangen Nurnberg FAU, Germany.
Friedrich Alexander Univ Erlangen Nurnberg FAU, Germany; Danylo Halytsky Lviv Natl Med Univ, Ukraine.
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2018 (Engelska)Ingår i: Autoimmunity, ISSN 0891-6934, E-ISSN 1607-842X, Vol. 51, nr 6, s. 310-318Artikel i tidskrift (Refereegranskat) 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.

Ort, förlag, år, upplaga, sidor
TAYLOR & FRANCIS LTD , 2018. Vol. 51, nr 6, s. 310-318
Nyckelord [en]
Anti-dsDNA antibodies; elastase activity; Libman-Sacks endocarditis; neuropsychiatric lupus; neutrophil extracellular traps; NET remnants; systemic lupus erythematosus
Nationell ämneskategori
Reumatologi och inflammation
Identifikatorer
URN: urn:nbn:se:liu:diva-153719DOI: 10.1080/08916934.2018.1514496ISI: 000452838700007PubMedID: 30369267OAI: oai:DiVA.org:liu-153719DiVA, id: diva2:1276175
Anmärkning

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]

Tillgänglig från: 2019-01-07 Skapad: 2019-01-07 Senast uppdaterad: 2019-03-22

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Publikationen är tillgänglig i fulltext från 2019-10-28 14:40
Tillgänglig från 2019-10-28 14:40

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Avdelningen för läkemedelsforskningMedicinska fakultetenAvdelningen för neuro- och inflammationsvetenskapKlinisk immunologi och transfusionsmedicinAvdelning för neurobiologiKlinisk patologiReumatologiska kliniken i Östergötland
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