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SAT0479 Increased Serum Light Levels Correlate with Disease Progression and Severity of Interstitial Pneumonia in Patients with Dermatomyositis
  1. T. Kotani1,
  2. T. Takeuchi1,
  3. T. Ishida1,
  4. R. Masutani2,
  5. K. Isoda1,
  6. K. Hata1,
  7. S. Makino1,
  8. T. Hanafusa1
  1. 1Department of Internal Medicine (I)
  2. 2Department of Central Laboratory, Osaka Medical College, Takatsuki, Japan


Background Dermatomyositis (DM) is frequently complicated with interstitial pneumonia (IP), causing increased morbidity and mortality. It is important to identify a useful serum marker associated with the progression, severity, and prognosis of DM-IP patients. Activated CD8+ T cells play an important role in the pathogenesis of DM-IP. LIGHT (the name of which is derived from ‘homologous to lymphotoxins, exhibits inducible expression, competes with herpes simplex virus glycoprotein D for herpes simplex virus entry mediator [HVEM], and expressed by T lymphocytes’) is a member of the TNF superfamily and activates CD4+ and CD8+ T cells, monocytes and macrophages, natural killer cells, immature dendritic cells, and platelets. LIGHT mainly binds to the HVEM receptor on T cells and transmits co-stimulatory signals. HVEM signals stimulated by LIGHT more strongly induce CD8+ T cells than CD4+ T cells. LIGHT has been reported as a biomarker of inflammatory diseases, such as rheumatid arthritis, ankylopoietic spondylarthritis, inflammatory bowel disease, and atopic dermatitis, but its association with the pathology of DM-IP has not been clarified.

Objectives Serum CD8+ T-cell activator, LIGHT, and Th1/Th2/Th17 cytokines were measured in DM-IP patients and compared with clinical parameters to investigate their usefulness.

Methods The correlations between the clinical findings and serum LIGHT and Th1/Th2/Th17 cytokine levels were investigated in 21 patients with DM-IP (14 with rapidly progressive IP [RPIP] and 7 with chronic IP [CIP], including 4 fatal cases of IP).

Results The median serum LIGHT level was 119 (16-335.4) pg/mL, which was higher than that in healthy control subjects and DM patients without IP. The median serum IL-6 level was 14.7 (2.4-154.5) pg/mL (n=13). The other cytokines were detected in only a few patients. The median serum LIGHT level in DM-RPIP patients (156 [49.6-335.4] pg/mL) was significantly higher than that in DM-CIP patients (94.3 [16-164.2] pg/mL) (P =0.02). The serum IL-6 level did not correlate with either progression or outcome of DM-IP. ROC curve analysis determined a serum LIGHT level of ≥120 pg/mL to be the cut-off value for the rapid progression of DM-IP. Serum LIGHT levels correlated significantly with %DLco (R =0.55, P =0.04) and total ground-glass opacity scores (R =0.72, P =0.0002). The serum LIGHT level significantly decreased to 100.5 (12.4-259.3) pg/mL 4 weeks after treatment initiation (P =0.04).

Conclusions The serum LIGHT level may be a promising marker of disease progression and severity in patients with DM-IP.

Disclosure of Interest None declared

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