Familial juvenile systemic lupus erythematosus in Arab children

Abstract

A ten-year retrospective analysis of the clinical features and survival of 60 Saudi children with systemic lupus erythematosus (SLE) was made. All the patients fulfilled the 1982 American College of Rheumatology's revised criteria for SLE and had had the disease at or before the age of 16 years. The female to male ratio was 5:1, the mean age of onset was 12.1 years (range 1.6-16 years), and the mean duration of follow-up was 4.7 years (range 2.2-11). Thirty-eight patients (63%) were diagnosed correctly before referral to KFSH&RC or KKUH. The mode of presentation was as follows: 55 patients had musculoskeletal involvement (91.6%), 49 patients had skin involvement (81.6%), 40 patients had hematological abnormalities (66.6%), 39 patients had renal disease (65%), 10 patients had pulmonary involvement (16%), 23 patients had cardiovascular disease (38%) and 18 patients had central nervous system involvement. During the study period four patients died (6.6%)-two of renal failure, one from meningitis and one from severe sepsis. This is the largest collection of childhood systemic lupus erythematosus from the Middle East and it shows that SLE is more common in Saudis than was hitherto believed, and that it has a high rate of organ involvement.

Early onset left ventricular remodeling in juvenile systemic lupus erythematosus; Insight from 3-dimensional speckle tracking echocardiography

“…The age at disease onset of our cohort was similar to most worldwide studies in the last 10 years. 19,20 In our study, females were affected more often than males with a ratio of 2.7:1, this is consistent with previous publication. 19 This observation could be interpreted by hormonal effect on SLE occurrence.…”

“…In our cohort, the most common initial presenting symptom was arthritis/arthralgia and malar rash and this is in harmony with published data. 20,21 Left ventricular echocardiographic study by conventional method…”

Evaluating the performance of ACR, SLICC and EULAR/ACR classification criteria in childhood onset systemic lupus erythematosus

“…Furthermore, the cohort included had a higher degree of familial SLE than previous studies due to higher degree of consanguineous marriage in the region. We have been able to identify a genetic mutation in a subgroup of these patients with familial SLE in the region (DNASE1L3) [23,26,27]. Both these epidemiological factors should be taken into consideration prior to making a generalization on a wider cSLE cohort from other ethnicity.…”

“…While, the EULAR/ACR criteria was met in 81, 88 and 89% of cSLE patients, while median weight scores were 19(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26), 21 (15-28) and 23(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28) at first visit, first year and last assessment respectively as shown in Table2.…”

Juvenile systemic lupus erythematosus: onset patterns and short-term outcome in Egyptian children, a single-center experience

“…In a cohort of 50 Arab children with familial SLE belonging to 18 families, the frequency of familial jSLE was 20.8%, indicating that familial jSLE is not uncommon in our societies. 40…”