Systemic lupus erythematosus: Current state of the genetic hypothesis

The occurrence of SLE in more than one member of a family has been frequently reported.“* In fact, 7%12% of patients with SLE will have a first or second degree relative with the same disease.‘,* In keeping with the female predilection for SLE, the majority are mother-daughter and sister combinations.’ There are, however, reports of mother-son, father-daughter, brother, and even father-son pairs.‘,’ In the aggregate, the proportion of men with familial SLE approximates the 10% reported in series of patients with “sporadic” disease. Moreover, the clinical and serologic expression of familial SLE does not differ from that of nonfamilial SLE.’ In addition, other diseases of immune dysfunction have been frequently described in such families, as well as serologic abnormalities in apparently healthy family members.‘.‘-’