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Published in: Arthritis Research & Therapy 1/2019

Open Access 01-12-2019 | Systemic Lupus Erythematosus | Research article

Interferon score is increased in incomplete systemic lupus erythematosus and correlates with myxovirus-resistance protein A in blood and skin

Authors: Wietske M. Lambers, Karina de Leeuw, Berber Doornbos-van der Meer, Gilles F.H. Diercks, Hendrika Bootsma, Johanna Westra

Published in: Arthritis Research & Therapy | Issue 1/2019

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Abstract

Objectives

Patients with incomplete systemic lupus erythematosus (iSLE) have lupus features, but do not meet classification criteria for SLE. Type I interferons (IFN) are important early mediators in SLE, and IFN upregulation in incomplete SLE may be associated with progression to SLE. Since many patients present with skin symptoms, the aim of this study is to investigate IFN type I expression and IFN-related mediators in the blood and skin of iSLE patients.

Methods

Twenty-nine iSLE patients (ANA titer ≥ 1:80, symptoms < 5 years, ≥ 1 objectified clinical criterion), 39 SLE patients with quiescent disease (fulfilling ACR or SLICC criteria, SLEDAI ≤4), and 22 healthy controls were included. IFN signature was measured in whole blood, based on 12 IFN-related genes, using RT-PCR, and IFN-score was calculated. IFN-related mediators myxovirus-resistance protein A (MxA), IFN-γ-induced protein 10 (IP-10), and monocyte chemoattractant protein (MCP-1) were measured using ELISA. IFN type I expression in the unaffected skin was analyzed by immunostaining with MxA.

Results

IFN-score was increased in 50% of iSLE patients and 46% of SLE patients and correlated positively with the number of autoantibodies, anti-SSA titer, ESR, and IgG and negatively with C4 in iSLE. Levels of MxA correlated strongly with IFN-score (r = 0.78, p < 0.0001). Furthermore, MxA expression was found in 29% of unaffected skin biopsies of iSLE and 31% of SLE patients and also correlated with IFN-score (r = 0.54, p < 0.0001).

Conclusions

IFN-score was increased in half of the iSLE patients, and given the correlation with complement and autoantibody diversity, this suggests a higher risk for disease progression. MxA in the blood and unaffected skin correlated strongly with the IFN-score and is possibly an easily applicable marker for IFN upregulation.
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Literature
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go back to reference Rúa-Figueroa Í, Richi P, López-Longo FJ, et al. Comprehensive description of clinical characteristics of a large systemic lupus erythematosus cohort from the spanish rheumatology society lupus registry (RELESSER) with emphasis on complete versus incomplete lupus differences. Med (United States). 2015;94(1):e267. https://doi.org/10.1097/MD.0000000000000267.CrossRef Rúa-Figueroa Í, Richi P, López-Longo FJ, et al. Comprehensive description of clinical characteristics of a large systemic lupus erythematosus cohort from the spanish rheumatology society lupus registry (RELESSER) with emphasis on complete versus incomplete lupus differences. Med (United States). 2015;94(1):e267. https://​doi.​org/​10.​1097/​MD.​0000000000000267​.CrossRef
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go back to reference Hochberg M. Updating the Anerican College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis & Rheumatism. 1997;40(9):1725–34. Hochberg M. Updating the Anerican College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis & Rheumatism. 1997;40(9):1725–34.
Metadata
Title
Interferon score is increased in incomplete systemic lupus erythematosus and correlates with myxovirus-resistance protein A in blood and skin
Authors
Wietske M. Lambers
Karina de Leeuw
Berber Doornbos-van der Meer
Gilles F.H. Diercks
Hendrika Bootsma
Johanna Westra
Publication date
01-12-2019
Publisher
BioMed Central
Published in
Arthritis Research & Therapy / Issue 1/2019
Electronic ISSN: 1478-6362
DOI
https://doi.org/10.1186/s13075-019-2034-4

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