TY - JOUR
T1 - Acquired C1 esterase inhibitor deficiency
AU - Markovic, Svetomir N.
AU - Inwards, David J.
AU - Frigas, Evangelos A.
AU - Phyliky, Robert P.
PY - 2000/1/18
Y1 - 2000/1/18
N2 - Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorders. Adults or elderly patients are most commonly affected. The diagnosis is suspected when patients present with recurrent angioedema and low serum levels of C4 with normal levels of C3. Low levels of C1q and low C1 esterase inhibitor activity confirm the diagnosis. In this paper, we summarize experience with 22 cases of acquired C1 esterase inhibitor deficiency in the context of a review of the published literature on diagnosis and treatment of this condition.
AB - Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorders. Adults or elderly patients are most commonly affected. The diagnosis is suspected when patients present with recurrent angioedema and low serum levels of C4 with normal levels of C3. Low levels of C1q and low C1 esterase inhibitor activity confirm the diagnosis. In this paper, we summarize experience with 22 cases of acquired C1 esterase inhibitor deficiency in the context of a review of the published literature on diagnosis and treatment of this condition.
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U2 - 10.7326/0003-4819-132-2-200001180-00009
DO - 10.7326/0003-4819-132-2-200001180-00009
M3 - Review article
C2 - 10644276
AN - SCOPUS:0033974103
SN - 0003-4819
VL - 132
SP - 144
EP - 150
JO - Annals of internal medicine
JF - Annals of internal medicine
IS - 2
ER -