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Case Reports
. 2018 Mar 28:2018:bcr2018224222.
doi: 10.1136/bcr-2018-224222.

Angioedema as the presenting feature of systemic lupus erythematosus

Naveen Kumar  1 , Deepanjali Surendran  1 , Chanaveerappa Bammigatti  1
Affiliations
Case Reports

Angioedema as the presenting feature of systemic lupus erythematosus

Naveen Kumar et al. BMJ Case Rep. .

Abstract

A young female in her early 20s presented with low-grade fever for 1 month, puffiness of face and abdominal distension for 15 days. Evaluation revealed pancytopenia with normocellular marrow, hypocomplementaemia and Coomb's positive haemolytic anaemia. She had angioedema with laryngospasm and worsening facial oedema which had dramatic response to steroids. Further workup lead to a diagnosis of systemic lupus erythematosus with neuropsychiatric involvement. Treatment with pulse steroids and cyclophosphamide resulted in dramatic improvement of her symptoms with no recurrence of angioedema during her follow-up visits.

Keywords: immunology; systemic lupus erythematosus.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Facial puffiness and lip swelling at presentation and after treatment.
Figure 2
Figure 2
MRI of brain showing swollen and hyperintense putamen and head of caudate nucleus bilaterally and focal hyperintensity of subcortical white matter of right middle frontal gyrus and posterior periventricular region.
See this image and copyright information in PMC

References

    1. Petri M, Orbai AM, Alarcón GS, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012;64:2677–86. 10.1002/art.34473 - DOI - PMC - PubMed
    1. Agostoni A, Aygören-Pürsün E, Binkley KE, et al. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol 2004;114(3 Suppl):S51–131. 10.1016/j.jaci.2004.06.047 - DOI - PMC - PubMed
    1. Bork K, Barnstedt S-E, Koch P, et al. Hereditary angioedema with normal C1-inhibitor activity in women. The Lancet 2000;356:213–7. 10.1016/S0140-6736(00)02483-1 - DOI - PubMed
    1. Cicardi M, Zingale LC, Pappalardo E, et al. Autoantibodies and lymphoproliferative diseases in acquired C1-inhibitor deficiencies. Medicine 2003;82:274–81. 10.1097/01.md.0000085055.63483.09 - DOI - PubMed
    1. Cacoub P, Frémeaux-Bacchi V, De Lacroix I, et al. A new type of acquired C1 inhibitor deficiency associated with systemic lupus erythematosus. Arthritis Rheum 2001;44:1836–40. 10.1002/1529-0131(200108)44:8<1836::AID-ART321>3.0.CO;2-Y - DOI - PubMed

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