Angioedema and systemic lupus erythematosus--a complementary association?

Manjari Lahiri, Anita Y N Lim
2007 Annals of the Academy of Medicine, Singapore  
We report angioedema as a rare presentation leading to a diagnosis of systemic lupus erythematosus (SLE). A 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. Management proved challenging
more » ... oved challenging and included high dose steroids and immunosuppressants. The patient responded to treatment and remains in remission without recurrence of the angioedema. AAE 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.
pmid:17364082 fatcat:fq7uninzrjdrzn6a2om47woyba