Abstract

Increased levels of serum pro-fibrotic cytokines have been reported in patients with systemic sclerosis (SSc). Some of these cytokines also play an important role in the differentiation and migration of eosinophils. The aim of this study was to determine whether eosinophilic inflammation is caused in SSc. We retrospectively reviewed the peripheral blood eosinophil counts in 70 untreated patients with SSc and compared them with those in patients with other major collagen diseases. We additionally evaluated a possible association with disease severity. Eosinophil counts were significantly higher levels in patients with SSc than in those with other collagen diseases, whereas total leukocyte counts were not. Eosinophil counts correlated positively with both severe interstitial lung disease (ILD; r = 0.255, p = 0.033) and modified Rodnan total skin thickness score (m-Rodnan TSS) in SSc (r = 0.347, p = 0.003), but did not correlate with ILD severity in other collagen diseases. In conclusion, peripheral eosinophil counts were higher in patients with SSc than in those with other collagen diseases and were correlated with increased disease severity. Our data suggest that eosinophilic inflammation is involved in the pathogenesis and progression of SSc.

Highlights

  • Systemic sclerosis is an autoimmune connective tissue disorder characterized by microvascular injury, excessive fibrosis of the skin, and distinctive visceral changes involving the lungs, heart, kidneys, and gastrointestinal tract (Steen et al 1994)

  • We identified 126 with rheumatoid arthritis (RA), 10 with polymyositis/dermatomyositis (PM/DM), with primary Sjögren syndrome, with systemic lupus erythematosus (SLE), and eight with mixed connective tissue disease (MCTD) according to a protocol similar to that used for systemic sclerosis (SSc)

  • Half of the patients with Interstitial lung disease (ILD) had grade 2 or more severe ILD, whereas ILD could not be detected by chest X-ray in seven patients with grade 1 ILD

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Summary

Introduction

Systemic sclerosis (scleroderma, SSc) is an autoimmune connective tissue disorder characterized by microvascular injury, excessive fibrosis of the skin, and distinctive visceral changes involving the lungs, heart, kidneys, and gastrointestinal tract (Steen et al 1994). These forms are generally classified into two major types on the basis of the extent of cutaneous fibrosis: (1) limited cutaneous SSc and (2) diffuse cutaneous SSc (LeRoy et al 1988). Interstitial lung disease (ILD) and pulmonary hypertension (PH) are the most serious complications and common causes of premature death (Altman et al 1991; Chang et al 2003). Several substances have been evaluated as biomarkers to assess the disease activity and its complications. Hasegawa et al previously reported that serum

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