Distinct immune profiles characterize patients with diffuse or limited systemic sclerosis

Francesca Ingegnoli, Daria Trabattoni, Marina Saresella, Flavio Fantini, Mario Clerici

Research output: Contribution to journalArticlepeer-review


Mitogen-stimulated IL-2, IFN-γ, TNF-α (type 1 cytokines), and IL-10 (type 2 cytokine) production by peripheral blood mononuclear cells, as well as expression of surface markers on immune cells, was evaluated in systemic sclerosis (SSc) patients. Fifty-four SSc patients with either diffuse (dSSc) or limited (lSSc) disease and 20 age- and sex-matched healthy controls (HCs) were examined. Fourteen patients were treated with prednisone and 9 patients with prednisone and cyclophosphamide pulses. Results showed that (1) IL-2 production is significantly decreased, whereas IL-10 is higher in untreated patients compared to HCs; IL-10, IFN-γ, and TNF-α production is higher in lSSc compared to dSSc patients; (2) CD4+25+ (IL-2R), CD8+, and CD8+45RA-28+57- (memory) lymphocytes are reduced in patients compared to HCs; (3) CD95-expressing CD4+ and CD8+ cells are significantly higher in dSSc patients; and (4) steroids are more effective alone than in combination with cyclophosphamide in reducing IL-10 and IFN-γ production in these patients. These results confirm that a complex imbalance in cytokine production is present in SSc patients and suggest that peculiar phenotypic populations are underrepresented in these patients. Overexpression of Fas in dSSc could results from the attempt of the immune system to induce apoptosis of autoreactive T-cell clones.

Original languageEnglish
Pages (from-to)21-28
Number of pages8
JournalClinical Immunology
Issue number1
Publication statusPublished - Jul 1 2003


  • Cell surface markers
  • Cytokines
  • Immunosuppressive therapy
  • Systemic sclerosis

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy


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