Juvenile systemic lupus erythematosus in Turkey: demographic, clinical and laboratory features with disease activity and outcome

Abstract

Objectives This paper aims to assess in a retrospective fashion the clinical and laboratory features, severity and outcome of juvenile systemic lupus erythematosus (jSLE) from a referral center in Turkey. Methods We have included all jSLE patients ( n = 92) diagnosed according to the revised American College of Rheumatology 1997 criteria between January 2004 and January 2017. Results The most prevalent clinical feature in our cohort was mucocutaneous manifestations (97.8%), followed by constitutional (81.5%), hematological (59.8%) and musculoskeletal manifestations (56.5%). Renal involvement was observed in 38% ( n = 35) of the patients, whereas biopsy-proven lupus nephritis was detected in 29.3% ( n = 27) of the cohort. Neurologic involvement was seen in 15 (16.3%) individuals. Among the patients positive for anticardiolipin IgM and/or IgG ( n = 11, 12%), only three developed antiphospholipid antibody syndrome. The mean SLEDAI-2K scores at disease onset (10.5 ± 4.8) showed a substantial decrease at last visit (4.3 ± 4.6). One-quarter of the patients (26.1%, n = 24) had damage according to the PedSDI criteria with a mean score of 0.45 ± 1.0 (range 0-7). When the PedSDI damage items were evaluated individually, growth failure was the most frequent damage criterion ( n = 6), followed by seizure ( n = 5). Two patients died during the designated study period of end-stage renal disease. The five-year and 10-year survival rate of our cohort was 100% and 94.4%, respectively. Conclusions Given the lower frequency of nephritis and central nervous system disease and lower basal disease activity and damage scores, we could conclude that children with jSLE in Turkey have a more favorable course compared to Asian and African American children, as expected from Caucasian ethnicity.

Pyoderma Gangrenosum Mimicking Antiphospholipid Antibody Syndrome in a Child With Juvenile Systemic Lupus Erythematosus

“…It reported in adult studies of cases of SLE that it had noted leg ulcers in 5-8 % of them [4,6]. In a study in Turkey, although mucocutaneous lesions were 97,8 % in children with SLE, the skin ulcers were not reported [1]. PG is associated with a systemic disease, most commonly infammatory bowel disease, arthritis, and hematologic malignancy, in at least half of the cases [7] .…”

“…Children with SLE may have a severe multisystem disease, including cutaneous lesions. One of the most common clinical features was mucocutaneous manifestations, followed by constitutional, hematological and musculoskeletal manifestations [1]. In recent years, antiphospholipid antibody syndrome (APS) has been increasingly recognised in various pediatric autoimmune [2,3] .…”

Çocukluk çağı başlangıçlı sistemik lupus eritematozus hastalarında avasküler nekrozis sıklığı

“…Avascular necrosis is a debilitating disorder that characterized by interruption of the blood supply which leads to death of the bone marrow and trabecular bone, presenting with joint pain, bone destruction, and loss of function 8 . It is a well-known complication of SLE since its first determination in 1960 and has been reported in 1.4-40% of jSLE patients [1][2][3][4][9][10][11][12][13][14] . The most common affected sites are femoral head, femoral condyles, proximal tibia, and the bones of foot and ankle 8 .…”

Screening for Fabry Disease in Patients With Juvenile Systemic Lupus Erythematosus

“…1,2 Besides cutaneous and musculoskeletal manifestations, renal and neurologic involvement could be seen in the course of both diseases. 1,[2][3][4][5][6][7][8][9][10][11][12] Deficient activity of lysosomal alpha-galactosidase enzyme that is caused by mutations in alphagalactosidase A (GLA) gene is responsible for clinical manifestations. The lack of enzyme activity results in the progressive accumulation of neutral glycosphingolipids, primarily globotriaosylceramide (Gb3), within lysosomes in a variety of cell types, including endothelial, renal, cardiac and nerve cells.…”