Related Experiment Video
Updated: Apr 14, 2026
Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma
Published on: September 20, 2024
Serum progranulin levels are elevated in dermatomyositis patients with acute interstitial lung disease, predicting
Atsushi Tanaka1, Hiroshi Tsukamoto2, Hiroki Mitoma3
1Department 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.
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 (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.
Insights
Serum progranulin (PGRN) is elevated in dermatomyositis (DM) patients, especially those with interstitial lung disease (ILD). Higher PGRN levels correlate with disease activity and poorer prognosis in DM with ILD.
Area of Science:
- Immunology
- Rheumatology
- Pulmonology
Background:
- Progranulin (PGRN) is an immunoregulatory molecule with incompletely understood roles in autoimmune diseases.
- The specific involvement of PGRN in dermatomyositis (DM) and its association with interstitial lung disease (ILD) remain unclear.
Purpose of the Study:
- To investigate serum PGRN levels in DM patients.
- To determine the association between serum PGRN levels and disease activity in DM, particularly in patients with ILD.
- To evaluate the prognostic significance of serum PGRN in DM patients with ILD.
Main Methods:
- Serum PGRN levels were quantified using enzyme-linked immunosorbent assay (ELISA).
- Measurements were performed in patients with DM (categorized by ILD status), polymyositis (PM), and healthy controls (NHCs).
- Correlations between serum PGRN and disease activity markers (ferritin, LDH, CRP) and prognosis were assessed in DM patients with ILD.
Main Results:
- Serum PGRN levels were significantly higher in DM patients compared to PM patients and NHCs.
- DM patients with acute/subacute interstitial pneumonia (A/SIP) exhibited higher PGRN levels than those with chronic interstitial pneumonia (CIP) or without ILD.
- In DM patients with ILD, serum PGRN levels positively correlated with serum ferritin, lactate dehydrogenase (LDH), and C-reactive protein (CRP).
- A significantly lower 6-month cumulative survival rate was observed in DM patients with ILD and serum PGRN levels ≥200 ng/ml compared to those with levels <200 ng/ml.
Conclusions:
- Serum PGRN is significantly associated with disease activity and prognosis in dermatomyositis patients with interstitial lung disease.
- PGRN may play a crucial role in the pathogenesis of DM-ILD.
- Serum PGRN could serve as a valuable biomarker for DM with ILD.
