Juvenile systemic lupus erythematosus: a single-center experience from southern Turkey

Objectives This study was conducted to analyze clinical characteristics, laboratory data, disease activity, and outcome of juvenile systemic lupus erythematosus (jSLE) patients from southern Turkey. Methods Fifty-three patients with jSLE diagnosed according to the revised American College of Rheumat...

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Bibliographic Details
Published in:Clinical rheumatology 2019-05, Vol.38 (5), p.1459-1468
Main Authors: Balci, Sibel, Ekinci, Rabia Miray Kisla, Bayazit, Aysun Karabay, Melek, Engin, Dogruel, Dilek, Altintas, Derya Ufuk, Yilmaz, Mustafa
Format: Article
Language:English
Subjects:
Adolescent
Adrenal Cortex Hormones - therapeutic use
Antibodies, Monoclonal, Humanized - therapeutic use
Biopsy
Child
Child, Preschool
Children
Corticosteroids
Cyclophosphamide
Cyclophosphamide - therapeutic use
Diagnosis
Disease Progression
Female
Hemorrhage
Humans
Immunosuppressive agents
Kidney - pathology
Kidneys
Longitudinal Studies
Lupus
Lupus Erythematosus, Systemic - drug therapy
Lupus Erythematosus, Systemic - physiopathology
Lupus nephritis
Lupus Nephritis - drug therapy
Lupus Nephritis - physiopathology
Male
Medicine
Medicine & Public Health
Monoclonal antibodies
Mycophenolate mofetil
Mycophenolic acid
Mycophenolic Acid - therapeutic use
Nephritis
Original Article
Patients
Retrospective Studies
Rheumatology
Rituximab
Severity of Illness Index
Systemic lupus erythematosus
Turkey
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Summary:Objectives This study was conducted to analyze clinical characteristics, laboratory data, disease activity, and outcome of juvenile systemic lupus erythematosus (jSLE) patients from southern Turkey. Methods Fifty-three patients with jSLE diagnosed according to the revised American College of Rheumatology 1997 criteria between January 2005 and June 2018 were included in the present study. Results The median age at the diagnosis was 12.8 (range, 5.1–17.7) years. The female to male ratio was 9.6:1. The most prevalent clinical features were mucocutaneous involvement (96.2%) and constitutional manifestations (94.3%). Renal manifestations, hematological manifestations, and neuropsychiatric involvement were detected in 40 (75%), in 38 (71.7%), and in 13 (24.5%) patients, respectively. Renal biopsy was performed to 49 patients (92.5%). Class IV lupus nephritis (LN) (34%) and class II LN (20.4%) were the most common findings. Mycophenolate mofetil, cyclophosphamide with corticosteroid were the main treatment options. Eighteen patients received rituximab and one tocilizumab. The mean SLE Disease Activity Index (SLEDAI) score at the time of diagnosis was 22.47 ± 8.8 (range = 3–49), and 1.34 ± 1.85 (range = 0–7) at last visit. Twenty-one patients (39.6%) had damage in agreement with Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (PedSDI; mean = 0.60 ± 0.94; range = 0–5) criteria. Growth failure was the most prevalent cause of damage ( n  = 13, 26%). One patient deceased due to severe pulmonary hemorrhage and multiple cerebral thromboses. Conclusion jSLE patients in this cohort have severe disease in view of the higher frequency of renal and neurologic involvement. Nevertheless, multicenter studies are needed to make a conclusion for all Turkish children with jSLE.
ISSN:0770-3198
1434-9949
DOI:10.1007/s10067-019-04433-4