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Early onset systemic lupus erythematosus: differential diagnoses, clinical presentation, and treatment options

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Abstract

Juvenile systemic lupus erythematosus is a rare multisystemic autoimmune disease with variable clinical manifestations, and disease onset before 16 years of age. Patients younger than 5 years are rarely affected and the age of onset may contribute to the course of disease in terms of clinical presentation, organ involvement, and serological findings. Here, we report two exemplary early-onset SLE patients, a 28-month-old patient with WHO class IIB kidney disease, arthritis, and a typical antibody constellation and an 11-month-old infant that presented with microcytic anemia, leukocytosis, arthritis, fasciitis, fatty liver disease, protein losing enteropathy, edema, and minimal change glomerulonephritis. Epidemiologic and clinical features of early-onset SLE compared to other forms of SLE are given and differential diagnoses and treatment options are discussed.

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Acknowledgments

The authors thank PD Dr. med. D. Aust, Department of Pathology, University Medical Center Carl Gustav Carus, TU Dresden, Germany, for providing us with high quality pictures for this manuscript. We thank Dr. med. Karsten Conrad, Department of Immunology, University Medical Center Carl Gustav Carus, TU Dresden, Germany, for helpful discussion and thoughtful suggestions on differential diagnoses.

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Authors and Affiliations

  1. Pediatric Rheumatology and Immunology Section, University Children’s Hospital Dresden, University Medical Center Garl Gustav Carus, Technical University Dresden, Fetscherstr. 74, 01307, Dresden, Germany

    Christian Michael Hedrich & Manfred Gahr

  2. University Children’s Hospital Göttingen, Georg August University, Robert-Koch-Str. 40, 37075, Göttingen, Germany

    Hildegard Zappel

  3. Pediatric Gastroenterology Section, University Children’s Hospital Dresden, University Medical Center Garl Gustav Carus, Technical University Dresden, Dresden, Germany

    Simon Straub & Martin W. Laass

  4. Department of Pathology, University Children’s Hospital Dresden, University Medical Center Garl Gustav Carus, Technical University Dresden, Dresden, Germany

    Kathrin Wieczorek

  5. Department of Radiology, University Children’s Hospital Dresden, University Medical Center Garl Gustav Carus, Technical University Dresden, Dresden, Germany

    Gabriele Hahn

  6. Children’s Hospital Freital, Weisseritztalkliniken, Buergerstr. 7, 01705, Freital, Germany

    Georg Heubner

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  1. Christian Michael Hedrich
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  2. Hildegard Zappel
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  8. Manfred Gahr
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Correspondence to Christian Michael Hedrich.

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Hedrich, C.M., Zappel, H., Straub, S. et al. Early onset systemic lupus erythematosus: differential diagnoses, clinical presentation, and treatment options. Clin Rheumatol 30, 275–283 (2011). https://doi.org/10.1007/s10067-010-1576-2

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  • DOI: https://doi.org/10.1007/s10067-010-1576-2

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