Background/Purpose : Systemic sclerosis (SSc) is a rare disease, with a mean incidence 3.8 cases, prevalence 99 per million in Sweden. The two most important blood group systems for hemagglutination are called AB0 and RhD. The AB0 system consists of two antigens, A and B which are oligosaccharide (Os). On the B antigen the terminal monosaccharide (Ms) always is a galactose (Gal), while on the A antigen, the terminal Ms is an acetylgalactosamine (GalNAc). This study examines the prevalence of different AB0 and RhD blood group antigens in SSc patients in comparison with the general Swedish population.

Methods : 150 patients, fulfilling the 2013 ACR/EULAR criteria for SSc, were included in the study. All patients were tested for the blood group antigens AB0 and Rhesus factor (Rh) and antinuclear SSc-associated antibodies (anticentromer; ACA, antitopoisomerase 1; ATA or anti RNA polymerase 3; ARA) were analysed.

According to our hypothesis, antigen present on the erythrocyte’s surface has a significant role in the formation of the SSc disease.

Results: In this studied group, the frequency of B antibodies was significantly lower (21%) compared to the average among the Swedish population (36%).

Two blood groups, B RhD negativ and AB RhD negativ, were not found among the 150 patients in the study group; compared to the national expected average of 2% and 1% respectively.

The two main findings included 0 RhD positive cases with centromer antibodies (ACA) being 61% and A RhD positive with ACA being 57%.

Conclusion: In conclusion, the natural antibodies present on the surface of the erythrocytes are most likely to influence the emergence of SSc. Therefore, the presence of Gal most likely has a significant defensive roll against the development of the SSc disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/ssc-and-the-significance-of-blood-group-antigens/