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Open Access Highly Accessed Case report

Systemic lupus erythematosus and granulomatous lymphadenopathy

Devendra Shrestha1*, Ajaya Kumar Dhakal1, Shiva Raj KC2, Arati Shakya1, Subhash Chandra Shah1 and Henish Shakya1

Author Affiliations

1 Department of Pediatrics, KIST Medical College Teaching Hospital, Imadol, Lalitpur, Nepal

2 Department of Pathology, KIST Medical College Teaching Hospital, Imadol, Lalitpur, Nepal

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BMC Pediatrics 2013, 13:179  doi:10.1186/1471-2431-13-179

Published: 5 November 2013

Abstract

Background

Systemic lupus erythematosus (SLE) is known to present with a wide variety of clinical manifestations. Lymphadenopathy is frequently observed in children with SLE and may occasionally be the presenting feature. SLE presenting with granulomatous changes in lymph node biopsy is rare. These features may also cause diagnostic confusion with other causes of granulomatous lymphadenopathy.

Case presentation

We report 12 year-old female who presented with generalized lymphadenopathy associated with intermittent fever as well as weight loss for three years. She also had developed anasarca two years prior to presentation. On presentation, she had growth failure and delayed puberty. Lymph node biopsy revealed granulomatous features. She developed a malar rash, arthritis and positive ANA antibodies over the course of next two months and showed WHO class II lupus nephritis on renal biopsy, which confirmed the final diagnosis of SLE. She was started on oral prednisolone and hydroxychloroquine with which her clinical condition improved, and she is currently much better under regular follow up.

Conclusion

Generalized lymphadenopathy may be the presenting feature of SLE and it may preceed the other symptoms of SLE by many years as illustrated by this patient. Granulomatous changes may rarely be seen in lupus lymphadenitis. Although uncommon, in children who present with generalized lymphadenopathy along with prolonged fever and constitutional symptoms, non-infectious causes like SLE should also be considered as a diagnostic possibility.

Keywords:
Granulomatous; Lymphadenopathy; SLE