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Clinical and serological features of juvenile systemic lupus erythematosus in an italian tertiary centre of pediatric rheumatology

  • 1,
  • 2,
  • 1,
  • 2,
  • 2,
  • 2 and
  • 1
Pediatric Rheumatology201412 (Suppl 1) :P325

https://doi.org/10.1186/1546-0096-12-S1-P325

  • Published:

Keywords

  • Membranous Nephropathy
  • Tendon Rupture
  • Pediatric Rheumatology
  • Proliferative Glomerulonephritis
  • Malar Rash

Introduction

Juvenile systemic lupus erythematosus (jSLE) is a multisystem autoimmune disorder that is characterized by widely variable clinical presentations and uncertain course.

Objectives

to evaluate the clinical and serological features of jSLE in a long lasting observation of a single italian centre.

Methods

104 patients affected by jSLE (< 18 years) were enrolled in thirty one years (from 1982 to 2013). 11 patients were male. The mean age of onset was 13 (SD 3.18); 41 were 12 years old or less. The mean disease duration was 163 months (SD 118).

Results

Fever and fatigue were frequent symptoms at onset, occurring respetively in 47 (45.1%) and 39 (37.5%) patients. The most common organ involvement was skeletal, affecting 89 (85.5%) of patients, 87 (97.7%) of whom were affected by non erosive arthritis. Jaccoud's arthropathy was observed in 7 (6.7%) patients. Malar rash, leukopenia and non scarring alopecia were common findings. They occurred respectively in 60 (57.6%), 59 (56.7%) and 52 (50%) patients. Clinical evidence of renal involvement occurred in 42 (40.3%) patients. 31 patients had 1 or more renal biopsies: 22 (70.9%) resulted affected by diffuse proliferative glomerulonephritis, 6 (19,3%) by focal proliferative glomerulonephritis and 6 (19.3%) by membranous nephropathy. Cutaneous vasculitic lesions, observed in 38 (36.5%) patients, were an important cause of morbidity. Oral ulcers and serositis were found in 27 (25.9%) and 25 (24%) patients respectively. Neurologic involmentent occurred in 20 (19.2%) patients. Seizures were observed in 8 (7.6%) patients. Infections remain a major problem in morbidity: serious infective manifestations occurred in 21 (20.1%) patients. Avascular necrosis of bone occurred in 7 (6.7%) patients, 3 of whom suffered more than 1 episode. Six (5.7%) patients developed steroid induced cataract. Tendon ruptures were observed in 4 (3.8%) patients. Growth failure and established osteoporosis, resulting from prolonged corticosteroid treatment in chronically active disease, were unfrequent but severe complications in our jSLE series occurring respectively in 8 (7.6%) and 5 (4.8%) patients. Cushingoid features and striae rubrae were frequent problems observed in 17 (16.3%) patients, due to disease itself and/or its treatment, that caused increased psychological distress, particularly in adolescents. Finally there were 4 deaths. Two due to infection complications and two due to myocardial infarction.

Conclusion

these data show that jSLE is not necessarily associated with poor prognosis; survival has improved, but morbidity due to disease itself and complications of therapy remain a significant problem.

Disclosure of interest

None declared

Authors’ Affiliations

(1)
Department of Rheumatology, Unit of Pediatric Rheumatology, Chair of Rheumatology, University of Milan, Italy
(2)
Department of Rheumatology, Chair of Rheumatology, University of Milan, G. Pini Institute, Milan, Italy

Copyright

© Pontikaki et al; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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