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. 2004 May;63(5):583–587. doi: 10.1136/ard.2003.011122

Serum concentrations of soluble P-selectin glycoprotein ligand-1 are increased in patients with systemic sclerosis: association with lower frequency of pulmonary fibrosis

K Yanaba 1, K Takehara 1, S Sato 1
PMCID: PMC1754985  PMID: 15082492

Abstract

Objective: To determine serum concentrations of soluble P-selectin glycoprotein ligand-1 (sPSGL-1) and its clinical associations in patients with systemic sclerosis.

Methods: Serum sPSGL-1 concentrations from 65 patients with systemic sclerosis were examined by enzyme linked immunosorbent assay. In a retrospective longitudinal study, 177 sera from 35 patients with systemic sclerosis were analysed (follow up 0.3 to 6.3 years)

Results: Serum sPSGL-1 was raised in patients with limited cutaneous systemic sclerosis (lSSc) (n = 34) and diffuse cutaneous systemic sclerosis (dSSc) (n = 31) compared with healthy controls (n = 22) and patients with systemic lupus erythematosus (n = 20) or dermatomyositis (n = 20). Patients with systemic sclerosis who had raised sPSGL-1 concentrations less often had pulmonary fibrosis and decreased vital capacity (%VC) than those with normal sPSGL-1 levels. sPSGL-1 concentrations were positively correlated with %VC in patients with systemic sclerosis. In the longitudinal study, patients with systemic sclerosis but without pulmonary fibrosis had consistently increased sPSGL-1 concentrations in the early phase, while those with pulmonary fibrosis had decreased sPSGL-1 throughout the follow up period.

Conclusions: A raised serum sPSGL-1 is associated with a lower frequency and severity of pulmonary fibrosis in systemic sclerosis. sPSGL-1 could be a protective factor against the development of pulmonary fibrosis in this disease and as such would be a possible therapeutic target.

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Figure 1 .

Figure 1

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Serum concentrations of soluble P-selectin glycoprotein ligand-1 (sPSGL-1) in patients with diffuse cutaneous systemic sclerosis (dSSc), limited cutaneous systemic sclerosis (lSSc), systemic lupus erythematosus (SLE), dermatomyositis (DM), and normal controls (CTL). Serum sPSGL-1 concentrations were determined by a specific enzyme linked immunosorbent assay. Bars indicate the mean value in each group. The broken line indicates the cut off value (mean +2 SD of the normal control samples).

Figure 2 .

Figure 2

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Correlations between serum soluble P-selectin glycoprotein ligand-1 (sPSGL-1) and per cent vital capacity (%VC) (left) and per cent diffusion capacity for carbon monoxide (%Dlco) (right) in patients with systemic sclerosis. Serum sPSGL-1 concentrations were determined with a specific enzyme linked immunosorbent assay. The broken lines indicate the cut off value.

Figure 3 .

Figure 3

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Serial changes in serum soluble P-selectin glycoprotein ligand-1 (sPSGL-1) during the follow up period in patients with systemic sclerosis plus pulmonary sclerosis (A) and without pulmonary sclerosis (B). Serum sPSGL-1 concentrations were determined with a specific enzyme linked immunosorbent assay. Empty symbols represent patients with diffuse cutaneous systemic sclerosis; filled symbols represent patients with limited cutaneous systemic sclerosis. The broken lines indicate the cut off values.

Selected References

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