Juvenile systemic lupus erythematosus in multicase families from Saudi Arabia: comparison of clinical and laboratory variables with sporadic cases

Abstract

The object of this study was to compare patients with familial versus sporadic systemic lupus erythematosus (SLE) with respect to clinical, laboratory variables and outcome. The familial SLE group comprised 12 patients while the comparative group comprised 24 patients selected by systemic sampling from our pediatric rheumatology clinic database. Those patients are listed according to the date of referral, which represents a sampling frame. The first patient was chosen randomly and subsequent patients were chosen at intervals of three. The two groups were compared with respect to: demographic information, age of onset of SLE, disease and follow up duration, clinical and laboratory variables and outcome. The patients from the familial group were younger and had an earlier age of onset of disease (P = 0.03, 0.001 respectively). Seven patients with familial SLE were from the eastern region of Saudi Arabia (P = 0.006). The two groups were comparable with respect to gender, disease duration and follow-up. At diagnosis, the discoid rash was more frequent in the familial group (P = 0.03) while other clinical and laboratory variables including disease activity as measured by SLEDAI did not show significant differences. The mean dose of steroid and use of other immunosuppressive therapy were similar in both groups. Three patients from the familial group died; two of them had unusual complications (one patient had transverse myelitis and pancreatic pseudocyst and the other one had extensive pyoderma gangrenosum). All patients from the sporadic group are alive in stable condition but one patient had severe central nervous system disease. Familial SLE patients tend to be younger and more likely to have discoid rash, in addition a marked difference in the origin of patients was noted. These differences may be helpful in identifying SLE patients with a stronger genetic predisposition. The mortality among familial SLE patients is more frequent which may reflect the disease severity.

Familial juvenile systemic lupus erythematosus in Arab children

“…These patients originated from a relatively 2 Arab highly consanguineous homogenous population, indicating that our cohort is likely to be representative of Arab countries. The frequency of familial SLE in our cohort (20.8%) is higher than that of the previous reports [16]. The referral bias is not a potential contributor to the increased prevalence of FJSLE in our clinics.…”

“…The rates of clotting disorders, seizures, and myocardial infarction were similar between the two groups [11,12]. In spite of the similarities of the clinical and serological manifestations between familial and non-familial SLE in adults SLE [13][14][15], it is not clear whether clinical and laboratory features of the FJSLE differ from those of sporadic cases; however, we found a marked difference in the origin of patients and the age of disease onset between the familial and sporadic SLE groups [16]. This study analyzed the clinical and laboratory features of 50 patients with FJSLE from 18 unrelated families seen in 3 tertiary health care centers in Saudi Arabia and Oman.…”

Clinicopathological characteristics of familial SLE patients with lupus nephritis

“…[4][5][6] To the best of our knowledge, however, no large clinicopathological study of familial SLE patients with LN has previously been reported. It is not clear whether the clinicopathological features of familial SLE patients with LN are similar to those of sporadic cases.…”

“…Although some clinical and laboratory features (e.g., photosensitivity, oral ulcers, malar rash and anti-Sm antibody) have been reported to occur more frequently in patients with familial SLE, familial and sporadic SLE are assumed to represent the same clinical entity. [4][5][6] To the best of our knowledge, however, no large clinicopathological study of familial SLE patients with LN has previously been reported. It is not clear whether the clinicopathological features of familial SLE patients with LN are similar to those of sporadic cases.…”

“…[1][2][3] Previous studies have suggested that familial and sporadic SLE share the same clinical entity although they differ in the subtypes of disease. [4][5][6] To the best of our knowledge, however, there is no available information regarding the clinicopathological nature of familial SLE patients with LN. It is not clear whether the clinicopathological features of familial SLE patients with LN are similar to or different from those of sporadic cases.…”