• Vol. 27 No. 2, 279–284
  • 15 March 1998

Systemic Sclerosis in DRw52-positive Silica-exposed Males: A Case Report



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The aims of this paper were to describe clinical and laboratory details of 4 siblings, of whom 2 have systemic sclerosis and to describe this family in the context of reported cases of familial systemic sclerosis.

The proband and his affected male sibling share the tissue typing antigens: HLA11, B57, DR13, DRw52,- and DQ2,6. Both were concordant for gender, silica exposure, marital status, migration history and antinuclear factor status but discordant for systemic sclerosis subtype, age at disease onset and ENA status. One non-affected sibling had no disease despite concordance for gender, HLA status, silica exposure, marital status, and migration history.

Familial scleroderma was first documented in 1953 by Rees and Bennett who described the occurrence of a localised disease in a father and daughter. The first documentation of familial systemic disease was by Orabona and Albano who reported its occurrence in two sisters some five years later.

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