Angioedema and systemic lupus erythematosus--a complementary association?
BACKGROUND
We report angioedema as a rare presentation leading to a diagnosis of systemic lupus erythematosus (SLE).


METHODS
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.


METHODS
Management proved challenging and included high dose steroids and immunosuppressants.


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


CONCLUSIONS
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.
Lahiri
Manjari
M
Department of Medicine, Division of General Medicine and Rheumatology, National University Hospital, Singapore. manjari_lahiri@nuh.com.sg
Lim
Anita Y N
AY
D000799:Angioedema / Q000097:blood / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D016736:Antiphospholipid Syndrome / Q000175:diagnosis / Q000209:etiology; D001921:Brain / Q000473:pathology; D003174:Complement C1 Inactivator Proteins / Q000032:analysis / Q000172:deficiency; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis* / Q000209:etiology*; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D012121:Respiration, Artificial; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy
D003174:Complement C1 Inactivator Proteins