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

  • Sibel BalciEmail author
  • Rabia Miray Kisla Ekinci
  • Aysun Karabay Bayazit
  • Engin Melek
  • Dilek Dogruel
  • Derya Ufuk Altintas
  • Mustafa Yilmaz
Original Article
  • 82 Downloads

Abstract

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.

Keywords

Juvenile systemic lupus erythematosus Lupus nephritis Outcome Tocilizumab 

Notes

Authors’ contributions

Dr. Balci and Dr. Yilmaz conceptualized and designed the study, drafted the initial manuscript, and reviewed and revised the manuscript. Dr. Kişla Ekinci, Dr.Karabay Bayazit, Dr. Melek, and Dr. Dogruel collected data, carried out the initial analyses, and reviewed and revised the manuscript. Dr. Altintas and Dr. Dogruel designed the data collection instruments, coordinated and supervised data collection, and critically reviewed the manuscript. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Funding

No funding was received.

Compliance with ethical standards

Disclosures

None.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Approval of the institutional review board of the local medical school was obtained.

Informed consent

Written informed consent was obtained from all patients or their legal guardians according to legal ethics regulations.

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Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  1. 1.Department of Pediatric Rheumatology, Faculty of MedicineCukurova UniversityAdanaTurkey
  2. 2.Department of Pediatric Nephrology, Faculty of MedicineCukurova UniversityAdanaTurkey
  3. 3.Department of Pediatric Allergy and Immunology, Faculty of MedicineCukurova UniversityAdanaTurkey

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