Annals of the Academy of Medicine, Singapore  2007;36(2):142-145

Angioedema and systemic lupus erythematosus--a complementary association?

Manjari LAHIRI 1 ; Anita Y N LIM

Affiliations

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Country

Singapore

Language

English

MeSH

Abstract

INTRODUCTIONWe report angioedema as a rare presentation leading to a diagnosis of systemic lupus erythematosus (SLE).

CLINICAL PICTUREA diagnosis of angioedema was delayed in a patient presenting with limb and facial swelling until she developed acute upper airway compromise. After excluding allergic and hereditary angioedema, acquired angioedema (AAE) was suspected, possibly precipitated by respiratory tract infection. Associated clinical and laboratory features led to a diagnosis of SLE.

TREATMENTManagement proved challenging and included high dose steroids and immunosuppressants.

OUTCOMEThe patient responded to treatment and remains in remission without recurrence of the angioedema.

CONCLUSIONAAE occurs due to the acquired deficiency of inhibitor of C1 component of complement (C1 INH). Lymphoproliferative disorders and anti-C1 INH antibodies are well-described associations. However, one should also consider the possibility of SLE.