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High levels of BAFF and APRIL seen in sera from patients with JDM, rapidly progressive ILD
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Patients with juvenile dermatomyositis complicated with the rapidly progressive interstitial lung disease subtype had elevated levels of B-cell activating factor and a proliferation-inducing ligand compared to patients with other subtypes of interstitial lung disease, according to a study.
Researchers studied clinical records from 24 patients with juvenile dermatomyositis (JDM), including eight patients with rapidly progressive interstitial lung disease (RP-ILD), 15 patients with chronic ILD (C-ILD), and 17 JDM patients without ILD from 12 centers in Japan and compared them with 10 children matched for age and sex. Patients were diagnosed according to Bohan and Peter or Gerami, et al criteria.
Serum levels of B-cell activating factor (BAFF) and a proliferation-inducing ligand (APRIL), autoantibodies associated with JDM, ferritin and enzymes, including aspartate aminotransferase (AST) and creatine kinase (CK) were analyzed.
Analysis showed the serum levels of BAFF and APRIL were significantly elevated in patients with RP-ILD and C-ILD and tended to be higher in the group without ILD compared to the control group. Significantly elevated serum levels of APRIL were observed in patients with RP-ILD compared to patients in the C-ILD, non-ILD and control groups. A significant correlation was observed between BAFF, APRIL, AST and aldolase, but not with CK. Serum BAFF and APRIL correlated with KL-6, and ferritin correlated with BAFF and not APRIL.
“In spite of the limited cohort size, our results indicate that sustained abnormal levels of BAFF and APRIL may contribute to a poor response to treatment and that analysis of these cytokine levels after treatment may be useful for evaluating treatment progress in patients with ILD-JDM,” the researchers wrote. “Our findings on BAFF and APRIL levels in ILD-JDM support the notion that these cytokines may be disease markers of RP-ILD that can be used to assess treatment response in ILD associated with JDM.” – by Shirley Pulawski
Disclosure: The researchers report no relevant financial disclosures.
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