Juvenile systemic lupus erythematosus in the Sultanate of Oman: clinical and immunological comparison between familial and non-familial cases.

Consanguineous marriage is quite prevalent in the Sultanate of Oman, with up to 45% of marriages being consanguineous. The aim of this study was to determine demographic, clinical and serological characteristics between familial and non-familial cases of juvenile systemic lupus erythematosus (SLE) in a highly consanguineous region such as Oman. Hospital medical records were retrospectively reviewed for 44 consecutive children with juvenile SLE seen at Sultan Qaboos University Hospital, 16 with familial SLE and 28 with non-familial SLE. All the children included in the study were Omani, diagnosed before 13 years of age, and fulfilled the 1982 revised ACR criteria. Analyses were performed using descriptive statistics. There were largely no significant differences in the clinical and serological manifestations between the two cohorts. However, the familial SLE group was associated with worse SLEDAI score at onset of diagnosis compared with the non-familial cohort (12 vs. 17; p = 0.003) suggesting a greater severity of disease in the familial group. Even though the rate of familial SLE is higher in Oman (36%) compared with the Western world (10-12%), it appears that familial and non-familial SLE cases are in fact similar disease entities in both the West and the Middle Eastern countries.