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Home › Dataset Library › Serum Follistatin-Like Protein 1 Is a Biomarker for Macrophage Activation Syndrome and May Predict Disease Course in Systemic Juvenile...

Dataset: Serum Follistatin-Like Protein 1 Is a Biomarker for Macrophage Activation Syndrome and May Predict Disease Course in Systemic Juvenile Idiopathic Arthritis

Objective. Follistatin-like protein 1 (FSTL-1) is a secreted glycoprotein that is over-expressed in certain inflammatory diseases. Our...

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Objective. Follistatin-like protein 1 (FSTL-1) is a secreted glycoprotein that is over-expressed in certain inflammatory diseases. Our objective in this study was to correlate FSTL-1 levels with measures of clinical disease activity in systemic juvenile idiopathic arthritis (sJIA), including macrophage activation syndrome (MAS). Methods. FSTL-1 serum levels were measured by ELISA in 28 patients with sJIA, including 7 patients who developed MAS, as well as in 30 normal controls. Levels were correlated with erythrocyte sedimentation rate (ESR), ferritin and sIL-2Rα expression. Differential gene expression based on FSTL-1 levels was analyzed in peripheral blood mononuclear cells (PBMCs). Results. FSTL-1 serum levels were elevated at time of presentation (pre-treatment) of sJIA (mean 200.7 ng/ml) and decreased to normal (mean 133.7 ng/ml) over 24 months (p<0.01). FSTL-1 levels were markedly elevated during acute MAS (mean 279.8 ng/ml) and decreased to normal (mean 120.3 ng/ml) following treatment (p<0.001). FSTL-1 levels correlated with serum markers of inflammation, including sIL-2Rα and ferritin, in patients with MAS. PBMCs from patients with FSTL-1 levels >200 ng/ml showed an increase in expression of genes related to innate immunity and erythropoiesis, and decrease in NK cell function. Patients with the highest FSTL-1 levels at disease onset (>300 ng/ml) ultimately required cyclosporine treatment. Conclusion. In patients with sJIA, serum FSTL-1 is a biomarker for MAS and FSTL-1 levels at presentation may predict subsequent disease course. Patients with FSTL-1 levels >200 ng/ml had altered gene expression patterns suggestive of subclinical MAS and may represent a subgroup of sJIA with more severe disease. FSTL-1 serum levels were measured by ELISA in 28 patients with sJIA, including 7 patients who developed MAS, as well as in 30 normal controls. Levels were correlated with erythrocyte sedimentation rate (ESR), ferritin and sIL-2Rα expression. Differential gene expression based on FSTL-1 levels was analyzed in peripheral blood mononuclear cells (PBMCs). Only 11 patients were run on microarray.

Species:
human

Samples:
11

Source:
E-GEOD-38849

Updated:
Dec.12, 2014

Registered:
Jul.11, 2014


Factors: (via ArrayExpress)
Sample FSTL-1 NG/ML AGE_AT_ONSET RACE TTF SEX
GSM340378 242 LT6 White 65min male
GSM340376 248 GTE6 White 70min male
GSM340375 218 LT6 White 120min female
GSM340372 311 GTE6 White 140min female
GSM34037 176 LT6 White 70min female
GSM340370 222 LT6 Black/African American 110min male
GSM340363 118 GTE6 White 100min male
GSM340362 159 LT6 White 120min male
GSM34036 176 GTE6 Black/African American 135min male
GSM340360 187 LT6 White 80min female
GSM340358 94 GTE6 White 125min female

Tags

  • arthritis
  • cell
  • disease
  • erythrocyte
  • glycoprotein
  • juvenile idiopathic arthritis
  • macrophage
  • peripheral
  • protein
  • serum
  • syndrome

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