TY - JOUR T1 - Three Cases of Systemic Sclerosis Within One Family With Different Antibodies and Clinical Features JF - The Journal of Rheumatology JO - J Rheumatol SP - 544 LP - 546 DO - 10.3899/jrheum.211139 VL - 49 IS - 5 AU - Julia Spierings AU - Voon H. Ong AU - Christopher P. Denton Y1 - 2022/05/01 UR - http://www.jrheum.org/content/49/5/544.abstract N2 - To the Editor:Systemic sclerosis (SSc) is a heterogeneous disorder in which both genetic and environmental factors play a role in the pathogenesis.1 Familial occurrence of SSc is uncommon and accounts for < 2% of cases.2 Familial cases tend to have similar autoantibodies and disease features.3 HLA studies suggest that major histocompatibility complex (MHC) genes exert their influence primarily on antinuclear antibody (ANA) expression in SSc.4 Also, skin biology differences to anti-RNA polymerase III (ARA) and antitopoisomerase antibodies (ATA) were studied, suggesting both shared and ANA-specific molecular drivers.5 We hypothesize that the predisposition to develop SSc is hereditary, yet phenotype and antibodies can vary. Ethical approval was granted (London Fulham NHS Research Ethics Committee MREC 20/LO/0404 IRAS project 279682) and all patients provided written informed consent for publication.Here we present 3 cases within 1 family, each case with different antibodies but showing typical antibody-associated SSc phenotypes (Table 1 and Figure 1).View this table:In this windowIn a new windowTable 1. Case characteristics.Figure 1. Family tree with affected members.Case 1. A White, 68-year-old female … ER -