Rheumatology International

, Volume 38, Issue 8, pp 1577–1584 | Cite as

Acquired angioedema in juvenile systemic lupus erythematosus: case-based review

  • Zahide Ekici Tekin
  • Gülçin Otar Yener
  • Selçuk YükselEmail author
Cases with a Message


An acquired form of angioedema that is clinically similar but scarcer than the hereditary form may be caused, even more rarely, by the presence of an underlying autoimmune disease. We report a previously healthy 16-year-old girl with an acquired angioedema as a rare and initial presentation of systemic lupus erythematosus. The patient had no previous angioedema attack and no family history. She did not have any chronic diseases and did not use any medicine regularly. The patient was diagnosed with systemic lupus erythematosus with the presence of polyarthralgia, angioedema, leucopenia, and positivity of immunologic criteria. Her edema resolved with high-dose methylprednisolone and hydroxychloroquine slowly. In conclusion, new-onset angioedema in adolescent girls should be investigated to evaluate autoimmunity and the possibility of systemic lupus erythematosus. The related literature on acquired angioedema associated with systemic lupus erythematosus is also reviewed.


Acquired angioedema Autoimmunity Systemic lupus erythematosus C1 esterase inhibitor protein C1 inhibitor 



This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Author contributions

The case was diagnosed and followed up by SY. ZET conceived and planned the presented case report. ZET and SY wrote the manuscript with support from GOY. Literature data were provided by SY, ZET, and GOY. The manuscript was edited for proper English language by Papercheck Editing Services (1521793396-e).


This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

Written informed consent was obtained from the patient and his parents for publication of this case report and an accompanying image.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Pediatric of RheumatologyPamukkale University School of MedicinePamukkaleTurkey

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