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

Tekin Z. E., Yener G. O., YÜKSEL S.

Rheumatology International, vol.38, no.8, pp.1577-1584, 2018 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Review
  • Volume: 38 Issue: 8
  • Publication Date: 2018
  • Doi Number: 10.1007/s00296-018-4088-z
  • Journal Name: Rheumatology International
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.1577-1584
  • Keywords: Acquired angioedema, Autoimmunity, C1 esterase inhibitor protein, C1 inhibitor, Systemic lupus erythematosus
  • Çanakkale Onsekiz Mart University Affiliated: No


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.