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Cartilage oligomeric matrix protein-induced complement activation in systemic sclerosis.

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    Cartilage oligomeric matrix protein (COMP)-C3b complexes are elevated in the serum of systemic sclerosis (SSc) patients. These complexes form in circulation, not skin, suggesting COMP does not initiate complement activation locally in SSc skin.

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    Area of Science:

    • Immunology
    • Rheumatology
    • Dermatology

    Background:

    • Cartilage oligomeric matrix protein (COMP) and complement activation product C3b complexes are observed in rheumatoid arthritis and systemic lupus erythematosus.
    • Systemic sclerosis (SSc) shows increased COMP expression and circulating levels, particularly in lesional skin.

    Purpose of the Study:

    • To investigate the presence of COMP-C3b complexes in serum and skin biopsies of SSc patients.
    • To correlate COMP-C3b levels with clinical parameters and assess longitudinal changes.

    Main Methods:

    • ELISA was used to measure COMP-C3b complexes in serum from 80 SSc patients (40 limited cutaneous SSc, 40 diffuse cutaneous SSc) and 97 controls.
    • Serum samples were analyzed at baseline and after 3-5 years; skin biopsies from 7 dcSSc patients and 3 controls were examined for COMP, C3b, and IgG deposition.

    Main Results:

    • Serum COMP-C3b levels were significantly elevated in both limited and diffuse cutaneous SSc patients compared to healthy controls.
    • COMP-C3b levels decreased in patients receiving immunosuppressive therapy.
    • No co-localization of COMP and C3b was observed in SSc skin biopsies.

    Conclusions:

    • COMP-C3b complexes are present in the circulation of SSc patients.
    • The absence of COMP and C3b co-localization in skin indicates that COMP-C3b complex formation occurs in the bloodstream, not within the skin.
    • These findings suggest COMP does not directly drive complement activation in SSc skin lesions.