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Serum progranulin associated with dermatomyositis, interstitial lung disease and disease activity
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Elevated serum progranulin levels in patients with dermatomyositis and interstitial lung disease were associated with disease activity and could be a biomarker for the disease, according to researchers in Japan.
The researchers enrolled 57 patients with dermatomyositis (DM), 21 patients with polymyositis (PM) and 60 healthy control individuals into a cross-sectional study of patients treated at Kyushu University Hospital, Saga University Hospital and Japanese Red Cross Fukuoka Hospital between 2002 and 2013. All patients met Bohan and Peter criteria for DM, PM or clinically amyopathic DM. Forty-nine of the patients with DM, 16 of the patients with PM and 51 of the healthy controls were women, and median age was 53 years. Patients with other autoimmune and infectious diseases were excluded.
In addition to blood sampling, demographic data, disease characteristics and medical history data were collected. Serological analysis included white blood cell counts, levels of hemoglobin, liver enzymes, progranulin and other markers, including antibodies associated with inflammation. Interstitial lung disease (ILD) was confirmed by chest radiography and CT.
Compared with healthy individuals and patients with PM, serum progranulin levels among patients with DM were significantly higher. Median levels of progranulin in patients with DM were 109 ng/mL, compared with 60 ng/mL in patients with PM and the range of 35 ng/mL to 70 ng/mL with normal distribution in healthy individuals.
In patients with ILD, those with acute or subacute interstitial pneumonia had significantly higher levels of progranulin than patients with chronic interstitial pneumonia and healthy participants, according to the researchers. No significant differences were seen when adjusted for age or sex.
Disease activity was positively correlated with elevated levels of progranulin. Serum progranulin elevation was associated with elevated lactate dehydrogenase, ferritin and C-reactive protein in patients with DM and acute or subacute interstitial pneumonia compared with patients without acute or subacute interstitial pneumonia.
Five patients in remission from ILD showed decreased levels of serum progranulin, suggesting that progranulin levels may be reflective of disease activity, according to the researchers. – by Shirley Pulawski
Disclosure: The researchers report no relative financial disclosures.
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