Advertisement

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 Yüksel
Cases with a Message
  • 102 Downloads

Abstract

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.

Keywords

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

Notes

Acknowledgements

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).

Funding

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.

References

  1. 1.
    Caldwell JR, Ruddy S, Schur PH, Austen KF (1972) Acquired C1 inhibitor deficiency in lymphosarcoma. Clin Immunol Immunopathol 1:39–52.  https://doi.org/10.1016/0090-1229(72)90006-2 CrossRefGoogle Scholar
  2. 2.
    Pappalardo E, Zingale LC, Terlizzi A, Zanichelli A, Falcioni A, Cicardi M (2002) Mechanisms of C1-inhibitor deficiency. Immunobiology 205:542–551CrossRefPubMedGoogle Scholar
  3. 3.
    Nettis E, Colanardi MC, Loria MP, Vacca A (2005) Acquired C1-inhibitor deficiency in a patient with systemic lupus erythematosus: a case report and review of the literature. Eur J Clin Invest 35:781–784.  https://doi.org/10.1111/j.1365-2362.2005.01571.x CrossRefPubMedGoogle Scholar
  4. 4.
    Markovic SN, Inwards DJ, Frigas EA, Phyliky RP (2000) Acquired C1 esterase inhibitor deficiency. Ann Intern Med 132:144–150.  https://doi.org/10.7326/0003-4819-132-2-200001180-00009 CrossRefPubMedGoogle Scholar
  5. 5.
    Alsenz J, Loos M (1989) The acquired C1-INH deficiencies with autoantibodies (AAE type II). Behring Inst Mitt 84:165–172Google Scholar
  6. 6.
    Cacoub P, Fremeaux-Bacchi V, De Lacroix I, Guillien F, Kahn MF, Kazatchkine D et al (2001) A new type of acquired C1 inhibitor deficiency associated with systemic lupus erythematosus. Arthritis Rheum 44:1836–1840. https://doi.org/10.1002/1529-0131(200108)44:8<1836::AID-ART321>3.0.CO;2-YGoogle Scholar
  7. 7.
    Jazwinska EC, Gatenby PA, Dunckley H, Serjeantaon SW (1993) C1 inhibitor functional deficiency in systemic lupus erythematosus (SLE). Clin Exp Immunol 92:268–273.  https://doi.org/10.1111/j.1365-2249.1993.tb03391.x CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Lahiri M, Lim AY (2007) Angioedema and systemic lupus erythematosus—a complementary association? Ann Acad Med Singapore 36:142–145PubMedGoogle Scholar
  9. 9.
    Bienstock D, Mandel L (2015) Facial Angioedema and Systemic Lupus Erythematosus. J Oral Maxillofac Surg 73:928–932.  https://doi.org/10.1016/j.joms.2014.12.007 CrossRefPubMedGoogle Scholar
  10. 10.
    Nagy L, Hannema A, Swaak A (1999) Acquired C1 inhibitor deficiency associated with systemic lupus erythematosus, secondary antiphospholipid syndrome and IgM monoclonal paraproteinaemia. Clin Rheumatol 18:56–58.  https://doi.org/10.1007/s100670050054 CrossRefPubMedGoogle Scholar
  11. 11.
    Nakamura S, Yoshinari M, Saku Y, Hirakawa K, Miishima C, Murai K et al (1991) Acquired C1 inhibitor deficiency associated with systemic lupus erythematosus affecting the central nervous system. Ann Rheum Dis 50:713–716.  https://doi.org/10.1136/ard.50.10.713 CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Ochonisky S, Intrator L, Wechsler J, Revuz J, Bagot M (1993) Acquired C1 inhibitor deficiency revealing systemic lupus erythematosus. Dermatology 186:261–263.  https://doi.org/10.1159/000247366 CrossRefPubMedGoogle Scholar
  13. 13.
    Thong BY, Thumboo J, Howe HS, Feng PH (2001) Life-threatening angioedema in systemic lupus erythematosus. Lupus 10:304–308.  https://doi.org/10.1191/096120301680417011 CrossRefPubMedGoogle Scholar
  14. 14.
    Ko CH, Ng J, Kumar S, Hurst M (2006) Life-threatening angioedema in a patient with systemic lupus. Clin Rheumatol 25:917–918.  https://doi.org/10.1007/s10067-005-0098-9 CrossRefPubMedGoogle Scholar
  15. 15.
    Furlanetto V, Kde G, Neves FDS, et al (2010) Intractable acquired autoimmune angioedema in a patient with systemic lupus erythematosus. Rev Bras Reumatol 50:102.  https://doi.org/10.1590/S0482-50042010000100011 CrossRefPubMedGoogle Scholar
  16. 16.
    Ali SI, Qayser Y, Roohi R (2013) Subtle angioedema presented systemic lupus erythematosus: a case report. IJCRI 4:631–634.  https://doi.org/10.5348/ijcri-2013-11-397-CR-11 CrossRefGoogle Scholar
  17. 17.
    Habibagahi Z, Ruzbeh J, Yarmohammadi V, Kamali V, Rastegar MH (2015) Refractory angioedema in a patient with systemic lupus erythematosus. Iran J Med Sci 40:372–375PubMedPubMedCentralGoogle Scholar
  18. 18.
    Meganathan A, Anu S (2017) Lupus presenting as angioedema- a case report. J Evol Res Gen Med 3:63–68Google Scholar
  19. 19.
    Tran JP, McCracken JL, Morsy A, Gonzalez EB (2017) Systemic lupus erythematous presenting as acquired angioedema: a case report and review of the literature. J Autoimmune Syst Dis 1:100002Google Scholar
  20. 20.
    Michailidou D, Kribis M, Kataria R, Sedaliu K, Dumitrescu M (2017) Facial and limb angioedema with parotitis and Kikuchi-like necrotizing lymphadenitis preceding neuropsychiatric systemic lupus erythematosus in a young African American male. Lupus 27:676–680.  https://doi.org/10.1177/0961203317726379 CrossRefPubMedGoogle Scholar

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

Personalised recommendations