title: Serum progranulin levels are elevated in dermatomyositis patients with acute interstitial lung disease, predicting prognosis.
abstract: BACKGROUND
  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 (rS=0.77, P<0.0001), lactate dehydrogenase (rS=0.54, P=0.0003) and C-reactive protein (rS=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.
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. a-tanaka@med.kyushu-u.ac.jp.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Tanaka
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. tsukamot@intmed1.med.kyushu-u.ac.jp.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Tsukamoto
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. mitoma@intmed1.med.kyushu-u.ac.jp.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Mitoma
authors.affiliation: Department of Preventive Medicine, Kyushu University Graduate School of Medical Sciences, Fukuoka, 812-8582, Japan. chikako@phealth.med.kyushu-u.ac.jp.
authors.forename: Chikako
authors.initials: C
authors.lastname: Kiyohara
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. naoyasu@intmed1.med.kyushu-u.ac.jp.
authors.forename: Naoyasu
authors.initials: N
authors.lastname: Ueda
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. m-ayano@intmed1.med.kyushu-u.ac.jp.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Ayano
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. sota@intmed1.med.kyushu-u.ac.jp.
authors.forename: Shun-ichiro
authors.initials: S
authors.lastname: Ohta
authors.affiliation: Department of Internal Medicine, Kyushu University Beppu Hospital, Oita, 874-0838, Japan. kimot@beppu.kyushu-u.ac.jp.
authors.forename: Yasutaka
authors.initials: Y
authors.lastname: Kimoto
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. akahoshi@intmed1.med.kyushu-u.ac.jp.
authors.forename: Mitsuteru
authors.initials: M
authors.lastname: Akahoshi
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. yarinobu@cancer.med.kyushu-u.ac.jp.
authors.forename: Yojiro
authors.initials: Y
authors.lastname: Arinobu
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. hniiro@cancer.med.kyushu-u.ac.jp.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Niiro
authors.affiliation: Department of Rheumatology, Faculty of Medicine, Saga University, Saga, 849-8501, Japan. taday@cc.saga-u.ac.jp.
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Tada
authors.affiliation: Department of Internal Medicine, Kyushu University Beppu Hospital, Oita, 874-0838, Japan. horiuchi@beppu.kyushu-u.ac.jp.
authors.forename: Takahiko
authors.initials: T
authors.lastname: Horiuchi
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. akashi@med.kyushu-u.ac.jp.
authors.forename: Koichi
authors.initials: K
authors.lastname: Akashi
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers; D003430:Cross-Sectional Studies; D003882:Dermatomyositis / Q000097:blood*; D005260:Female; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins / Q000097:blood*; D017563:Lung Diseases, Interstitial / Q000097:blood*; D008297:Male; D008875:Middle Aged; D000077153:Progranulins
chemical_list: D015415:Biomarkers; C492314:GRN protein, human; D036341:Intercellular Signaling Peptides and Proteins; D000077153:Progranulins