Home Featured posts Nine year follow-up of a rare case of angioedema due to acquired C1-inhibitor deficiency with late onset and good response to attenuated androgen.

Nine year follow-up of a rare case of angioedema due to acquired C1-inhibitor deficiency with late onset and good response to attenuated androgen.

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Angioedema due to acquired deficiency of C1-inhibitor (C1-INH-AAE) is a rare disease sharing some clinical and laboratory similarities with hereditary angioedema, but with late onset and no positive family history.

It is reported a case of rare angioedema due to acquired deficiency of C1-inhibitor, which has no clear cause after long follow-up, but good response to attenuated androgen which is found to a 75 year old woman suffering from recurrent episodes of angioedema since the age of 66. First she was on a daily prophylactic treatment with tranexamic acid, with no amelioration after 3 months. Then the treatment was switched to attenuated androgen danazol. She completed 3 years of continuous treatment with low daily maintenance dose of danazol (ongoing), with no angioedema attack.

It is concluded that the awareness of angioedema due to C1-INH deficiency should be increased within medical community and therapeutic options should be more clearly indicated and available for all diagnosed cases. More information regarding this rare case you can find here.

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