Male microchimerism and HLA compatibility in French women with sclerodema: a different profile in limited and diffuse subset

• Published in: Rheumatology (Oxford, England) England), 2009-04, Vol.48 (4), p.363-366
• Main Authors: Rak, Justyna M., Pagni, Philippe P., Tiev, Kiet, Allanore, Yannick, Farge, Dominique, Harlé, Jean-Robert, Launay, David, Hachulla, Eric, Didelot, Rémi, Cabane, Jean, Kahan, André, Martin, Marielle, Granel, Brigitte, Roudier, Jean, Lambert, Nathalie C.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Biological and medical sciences; Blood; Case-Control Studies; Child; Chimerism; Diffuse cutaneous scleroderma; Diseases of the osteoarticular system; Female; Histocompatibility; Histocompatibility Testing; HLA-DR Antigens; HLA-DRB1 Chains; Humans; Limited cutaneous scleroderma; Male; Medical sciences; Microchimerism; Middle Aged; Mothers; Peripheral blood mononuclear cells; Pregnancy - genetics; Pregnancy - immunology; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Scleroderma, Diffuse - genetics; Scleroderma, Limited - genetics; Scleroderma, Systemic - genetics; Scleroderma, Systemic - immunology; Y-chromosome; Young Adult
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/ken505

Objectives. Male microchimerism (Mc) persisting from pregnancy has been found at greater frequencies and/or higher quantities in women with scleroderma (SSc) compared with controls, suggesting a possible role in disease development. Moreover, women with an HLA-compatible child have a higher risk to develop SSc. We tested the hypothesis, on our French SSc cohort, that women with lcSSc and dcSSc, two distinct clinical subsets, have a different profile in terms of Mc and HLA compatibility in families. Methods. We studied 98 women (52 lcSSc and 46 dcSSc) for male Mc, by real-time PCR, in their whole blood and/or peripheral blood mononuclear cells (PBMC). Similarly, 91 matched healthy women were analysed. Complete HLA-DRB1 typing was obtained for 58 SSc and 68 control families (proband/children). Results. Women with lcSSc (N = 50) had male Mc more often in their whole blood than women with dcSSc (N = 40, 20 vs 5%, P = 0.038), but not significantly more than controls. By contrast, women with dcSSc (N = 36) hold Mc more often in PBMC (25 vs 9%), but not significantly and have greater quantities than controls (N = 82, P = 0.048). This contrast is also visible in feto-maternal HLA-DRB1 compatibility, which was increased only among women with lcSSc (N = 33) compared with controls (N = 68, P = 0.003). Conclusion. For the first time, we showed that women with lcSSc and dcSSc hold male Mc in different blood compartments. Furthermore, a distinct pattern between the two SSc subtypes is observed for feto-maternal HLA-DRB1 compatibility. These results suggest a different mechanism behind each type of disease.