Type I Interferon Score and Interferon Induced Mediators CXCL10 and Neopterin Are Correlated with Disease Activity in Juvenile Dermatomyositis

Rebecca Nicolai¹, Ivan Caiello ², Rava' Lucilla ³, Silvia Rosina ⁴, Francesco Licciardi ⁵, Luisa Bracci Laudiero ², Angelo Ravelli ⁶, Fabrizio De Benedetti ⁷ and Gian Marco Moneta ², ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Lazio, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ³Clinical Epidemiology Unit, Ospedale Pediatrico Bambino Gesù, Roma, Italy, Rome, Italy, ⁴Giannina Gaslini Institute, IRCCS Clinica Pediatrica e Reumatologia, Genoa, Italy, Genoa, Italy, ⁵Division of Pediatric Immunology and Rheumatology, Regina Margherita Children Hospital,Turin, Italy, Turin, Italy, ⁶IRCCS Istituto Giannina Gaslini, Università degli Studi di Genova, Genova, Italy, ⁷Bambino Gesù Children’s Hospital, Rome, Italy

Meeting: ACR/ARP Annual Meeting 2019

Keywords: chemokines and biomarkers, interferons, juvenile dermatomyositis

Session Information

Date: Sunday, November 10, 2019

Title: Pediatric Rheumatology – ePoster I: Basic Science, Biomarkers, & Sclerodermic Fever

Session Type: Poster Session (Sunday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Interferons (IFNs) seem to play an important role in the pathogenesis of juvenile dermatomyositis (JDM). Our group previously reported that expression of both type I and type II IFN related genes is increased in muscle biopsies of JDM patients and correlates with histological and clinical features of the disease. The aim of this study was to investigate expression of interferon regulated genes (IRGs), as well as serum levels of two type I and type II IFN induced chemokines (CXCL9, CXCL10) and neopterin in peripheral blood of JDM patients and to assess their correlations with clinical and laboratory findings.

Methods: We collected 189 blood samples from 39 JDM patients at different time points during follow-up. In 11 patients we obtained the first blood sample at time of muscle biopsy. We measured expression of type I IRGs (IFI27, IFI44L, IFIT1, ISG15, RSAD2, SIGLEC1), IFNg and type II IRGs (CXCL9, CIITA, IDO1) by quantitative PCR (qPCR) and calculated a type I and type II IFN score for muscle and blood samples; serum levels of CXCL9, CXCL10 and neopterin were analyzed by ELISA. Ten healthy subjects were used as controls (HC). At each visit, the following clinical data were recorded: physician’s global assessment (PGA) of disease activity VAS (Visual Analogue Scale), cutaneous VAS, Cutaneous Assessment Tool (CAT) activity score, Childhood Myositis Assessment Score (CMAS), serum levels of creatine phosphokinase (CK, IU/l), presence of myositis specific or myositis associated antibodies (MSA/MAA), prednisone (or equivalent) dose (mg/kg/daily), ongoing immunosuppressive medications.

Results: Serum levels of CXCL9 where significantly correlated with muscle expression of IFNg and type II IFN score. The correlation of CXCL10 levels with muscle type I and type II IFN score was weaker. Muscle expression of CXCL9 and CXCL10 correlated with serum levels of these chemokines. Type I IFN score in blood of JDM patients was increased compared to HC and significantly correlated with PGA, cutaneous VAS, CAT activity score. Serum levels of CXCL9 and CXCL10 were significantly higher in JDM patients compared to HC. MSA positive JDM patients showed higher levels of CXCL9 and CXCL10 compared to MSA negative patients. CXCL10 levels were correlated with PGA and CMAS, but not with cutaneous disease activity. CXCL9 showed no significant association with the evaluated clinical features. Neopterin levels were significantly correlated with PGA, cutaneous VAS, CAT activity score and CMAS.

Conclusion: Our findings indicate that expression of IRGs, measured as type I IFN score, and serum levels of CXCL10 and neopterin reflect specific features of disease activity in JDM, supporting their role as valuable disease biomarkers.

Disclosure: R. Nicolai, None; I. Caiello, None; R. Lucilla, None; S. Rosina, None; F. Licciardi, None; L. Bracci Laudiero, None; A. Ravelli, Angelini, AbbVie, Bristol-Myers Squibb, Johnson & Johnson, Novartis, Pfizer, Reckitt Benkiser, and Roche, 2, 5, 8; F. De Benedetti, AbbVie, 2, BMS, 2, Novartis, 2, Novartis, Novimmune, Hoffmann- La Roche, SOBI, AbbVie, Pfizer, 2, Novimmune, 2, Pfizer, 2, Roche, 2, Sanofi, 2, Sobi, 2, Swedish Orphan Biovitrum, 2, UCB, 2; G. Moneta, None.

To cite this abstract in AMA style:

Nicolai R, Caiello I, Lucilla R, Rosina S, Licciardi F, Bracci Laudiero L, Ravelli A, De Benedetti F, Moneta G. Type I Interferon Score and Interferon Induced Mediators CXCL10 and Neopterin Are Correlated with Disease Activity in Juvenile Dermatomyositis [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/type-i-interferon-score-and-interferon-induced-mediators-cxcl10-and-neopterin-are-correlated-with-disease-activity-in-juvenile-dermatomyositis/. Accessed .

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