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Open Access Research article

Cartilage oligomeric matrix protein-induced complement activation in systemic sclerosis

Kaisa E Otteby1, Emelie Holmquist1, Tore Saxne2, Dick Heinegård2, Roger Hesselstrand2 and Anna M Blom1*

Author Affiliations

1 Section of Medical Protein Chemistry, Department of Laboratory Medicine, Lund University, Skåne University Hospital, S-20502 Malmö, Sweden

2 Department of Clinical Sciences, Section of Rheumatology, Lund University, Skåne University Hospital, S-22185 Lund, Sweden

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Arthritis Research & Therapy 2013, 15:R215  doi:10.1186/ar4410

Published: 13 December 2013

Abstract

Introduction

Complexes between cartilage oligomeric matrix protein (COMP) and the complement activation product C3b have been found in the circulation of patients with rheumatoid arthritis and systemic lupus erythematosus. In systemic sclerosis (SSc) COMP expression in the skin is upregulated both in lesional and non-lesional skin, which is also reflected in an increased amount of circulating COMP. We investigated the presence of COMP-C3b complexes in serum and skin biopsies of patients with SSc.

Methods

The presence of COMP and COMP-C3b complexes in the serum of 80 patients with limited cutaneous SSc (lcSSc, n = 40) and diffuse cutaneous SSc (dcSSc, n = 40) and 97 healthy controls was measured by ELISA and correlated to different clinical parameters. Samples were collected both at baseline and after three to five years to assess longitudinal changes in COMP-C3b complex levels. Furthermore, skin biopsies from seven patients with dcSSc and three healthy controls were analyzed for expression of COMP and deposition of C3b and IgG.

Results

Serum levels of COMP-C3b were found to be elevated in both dcSSc and lcSSc compared to healthy controls and decreased at the second measurement in patients on immunosuppressive therapy. No co-localization of COMP and C3b was found in the skin biopsies, indicating that the COMP-C3b complexes are formed upon release of COMP into the circulation.

Conclusion

COMP-C3b complexes are found in the serum of patients with SSc. The lack of co-localization between COMP and C3b in the skin suggests that COMP does not drive complement activation in the skin in SSc.