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Arthritis Research & Therapy
Published in: Arthritis Research & Therapy 1/2015

Open Access 01-12-2015 | Research article

Serum progranulin levels are elevated in dermatomyositis patients with acute interstitial lung disease, predicting prognosis

Authors: Atsushi Tanaka, Hiroshi Tsukamoto, Hiroki Mitoma, Chikako Kiyohara, Naoyasu Ueda, Masahiro Ayano, Shun-ichiro Ohta, Yasutaka Kimoto, Mitsuteru Akahoshi, Yojiro Arinobu, Hiroaki Niiro, Yoshifumi Tada, Takahiko Horiuchi, Koichi Akashi

Published in: Arthritis Research & Therapy | Issue 1/2015

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Abstract

Introduction

Progranulin (PGRN), a pleiotropic growth factor, has emerged as an immunoregulatory molecule. Because the roles of PGRN in dermatomyositis (DM) are still unknown, we investigated whether serum PGRN levels are associated with disease activity and prognosis in DM patients, particularly in those with DM complicated with interstitial lung disease (ILD).

Methods

The serum levels of PGRN were measured by enzyme-linked immunosorbent assay in patients with DM (n =57; acute/subacute interstitial pneumonia (A/SIP): n =17, chronic interstitial pneumonia (CIP): n =24, without ILD: n =16), polymyositis (PM, n =21; including 6 with ILD) and normal healthy controls (NHCs, n =60). We assessed the correlation between the serum PGRN levels and the activity indexes of ILD or prognosis in DM patients with ILD.

Results

Serum PGRN levels were significantly higher in DM patients than in PM patients (P =0.0025) and in NHCs (P <0.0001). In DM patients, the levels were significantly higher in patients with A/SIP than in those with CIP (P <0.0001) or without ILD (P =0.0003). The serum PGRN levels in DM patients with ILD significantly correlated with serum ferritin (r S =0.77, P <0.0001), lactate dehydrogenase (r S =0.54, P =0.0003) and C-reactive protein (r S =0.48, P =0.0015) levels. Moreover, in DM patients with ILD, the cumulative survival rate for 6 months was significantly lower in the group with serum PGRN levels ≥200 ng/ml (67%) than in the group with serum PGRN levels <200 ng/ml (100%) (P =0.0009).

Conclusions

Serum PGRN is associated with disease activity and prognosis of DM with ILD. PGRN may play a role in the pathogenesis of DM and could be a useful biomarker.
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Metadata
Title
Serum progranulin levels are elevated in dermatomyositis patients with acute interstitial lung disease, predicting prognosis
Authors
Atsushi Tanaka
Hiroshi Tsukamoto
Hiroki Mitoma
Chikako Kiyohara
Naoyasu Ueda
Masahiro Ayano
Shun-ichiro Ohta
Yasutaka Kimoto
Mitsuteru Akahoshi
Yojiro Arinobu
Hiroaki Niiro
Yoshifumi Tada
Takahiko Horiuchi
Koichi Akashi
Publication date
01-12-2015
Publisher
BioMed Central
Published in
Arthritis Research & Therapy / Issue 1/2015
Electronic ISSN: 1478-6362
DOI
https://doi.org/10.1186/s13075-015-0547-z

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