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Increased Serum Levels of the IL ‐33 Neutralizing sST 2 in Limited Cutaneous Systemic Sclerosis
Author(s) -
Wagner A.,
Köhm M.,
Nordin A.,
Svenungsson E.,
Pfeilschifter J.M.,
Radeke H. H.
Publication year - 2015
Publication title -
scandinavian journal of immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.934
H-Index - 88
eISSN - 1365-3083
pISSN - 0300-9475
DOI - 10.1111/sji.12317
Subject(s) - fibrosis , medicine , pathogenesis , iloprost , connective tissue disease , pathophysiology , biomarker , scleroderma (fungus) , pathological , pathology , autoimmune disease , immunology , disease , prostacyclin , biology , biochemistry , inoculation
The pathophysiology of both limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous SSc (dcSSc), representing two subtypes of an autoimmune disease of the connective tissue, is still enigmatic. Life‐limiting, progressive fibrotic changes as a consequence of vasculopathy and autoimmunity are characteristic in varying extent for lc SS c and dc SS c. Previously, an increased IL ‐33 serum concentration in early phase SS c patients and an elevated tissue expression of its receptor, ST 2L, on endothelial cells ( EC ) were described. While suggested as a biomarker for fibrotic diseases, for example liver fibrosis, the role of soluble ST 2 ( sST 2) in the pathological processes and its contribution to vascular fibrosis in SS c has not been investigated. Here, we showed that sST 2 is elevated in late phase limited cutaneous SS c (lc SS c) as compared to patients with shorter disease duration or with the diffuse subtype of SS c. We demonstrated that sST 2, not IL ‐33, is significantly increased in serum of lc SS c patients with disease duration over 9 years. Soluble ST 2 was not elevated in healthy controls or in SS c patients with early skin involvement or disease duration shorter than 9 years. Furthermore, we observed that sST 2 serum levels were lowered by iloprost (prostacyclin) treatment. After 5 days of iloprost infusion, sST 2 serum levels fell in 6 of 7 patients. Therefore, we not only like to propose sST 2 as a biomarker for progressive vascular fibrosis, but moreover, suggest that the involvement of sST 2 in the pathogenesis of lc SS c may be exploited therapeutically.