ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2079

Serum Interferon α, β and Interleukin-18 Are Associated With Disease Activity Of Rapidly Progressive Interstitial Lung Disease In Patients With Clinical Amyopathic Dermatomyositis

Shinji Sato1, Noriko Sasaki1, Shinichi Nogi1, Naofumi Chinen2, Kiri Honda1, Eiko Saito1, Chiho Yamada2 and Yasuo Suzuki2, 1Rheumatology, Tokai University School of Medicine, Isehara, Japan, 2Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Japan

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: ,

Session Information

Title: Muscle Biology, Myositis and Myopathies: Advances in the Epidemiology, Immunology and Therapy of Myositis

Session Type: Abstract Submissions (ACR)

Background/Purpose: It is well known that rapidly progressive interstitial lung disease (RP-ILD) in patients with dermatomyositis (DM) is associated with poor outcome. Previous findings suggested that interferon alfa (IFNα), interferon beta (IFNβ) and interleukin-18 (IL-18) might play an important role in the pathogenesis of DM (including the DM subtype clinically amyopathic dermatomyositis, CADM) and RP-ILD. The aim of this study was to determine whether levels of IFNα, IFNβ, IL-18 and other cytokines were associated with disease activity in CADM and RP-ILD.

Methods: Serum samples and clinical data were collected from 28 patients diagnosed as having DM with or without RP-ILD (14 with RP-ILD, 9 with chronic ILD and 5 without ILD), 4 other connective tissue diseases (CTD) and 4 normal healthy controls (NHC). Serum IFNα, IFNβ, IL-18 and other proinflammatory cytokines were quantified by commercially-available enzyme-linked immunosorbent assay (ELISA) kits over the disease course and related to disease activity.

Results: IFNα was detected in 7 of 28 (25%) DM serum samples before treatment. All 7 patients who had high IFNα concentration were diagnosed as CADM and RP-ILD. IFNα decreaced significantly and was no longer detectable after treatment which reduced symptoms (33.8 pg/ml vs. 0.0 pg/ml, P=0.019). In contrast, no IFNα was detected in any sera from DM without RP-ILD, other CTD or NHC. Sera from 14 of 28 (50%) patients with DM had high IFNβ in contrast to only 1 of 8 (13%) with other CTD and NHC. Nine of 14 (64%) sera with IFNβ before treatment were from CADM with RP-ILD patients. As with IFNα, IFNβ levels of 7 CADM and RP-ILD patients who responded to therapy and survived decreased significantly after improvement of pulmonary symptoms (108.9 pg/ml vs. 38.3 pg/ml, P=0.027). IL-18 was detected in all sera from DM with or without RP-ILD as well as other CTD and NHC before treatment. Serum IL-18 levels in CADM with RP-ILD, as well as in DM without RP-ILD, decreased significantly, parelleling symptom improvement (70.2 pg/ml vs. 25.0 pg/ml, P=0.017). Serum IL-17A, IL-23 and BAFF were not detected in any DM patient or NHC.

Conclusion: These results suggest that serum IFNα, IFNβ and IL-18 levels might be involved in the pathogenesis of RP-ILD and CADM, and might represent a useful biomarker of disease activity.

Disclosure:

S. Sato,
None;

N. Sasaki,
None;

S. Nogi,
None;

N. Chinen,
None;

K. Honda,
None;

E. Saito,
None;

C. Yamada,
None;

Y. Suzuki,
None.

« Back to 2013 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/serum-interferon-%ce%b1-%ce%b2-and-interleukin-18-are-associated-with-disease-activity-of-rapidly-progressive-interstitial-lung-disease-in-patients-with-clinical-amyopathic-dermatomyositis/