Long‑term follow‑up of a case of recurrent angioedema due to acquired C1‑inhibitor deficiency – clinical and therapeutic aspects

Abstract

Angioedema (AE) is a frequent pathology in medical practice, raising significant diagnostic and therapeutic problems. Angioedema due to acquired C1-inhibitor esterase (C1-INH) deficiency is a rare form of AE without urticaria, with life-threatening potential outcome, often associated with lymphoproliferative or autoimmune diseases. We report the long-term outcome of an 80-year-old female patient, diagnosed in 2014 with AE due to acquired C1-INH deficiency, with good response to attenuated androgens, who has been constantly monitored in our clinic for 12 years. The onset of AE attacks was six years before diagnosis confirmation, and was considered first induced by angiotensin-converting enzyme inhibitors (ACEIs) therapy, but she continued to have AE attacks after the discontinuation of ACEI. No underlying disease could be identified, despite extended medical evaluation. AE episodes stopped after the initiation of treatment with danazol in September 2014 and the C1-INH and C4 plasma levels became normal after one month. We have closely monitored the possible side effects of danazol and the occurrence of other clinical and laboratory manifestations. Danazol therapy was well tolerated and we could not find any causal disease for the C1-INH deficiency. During the last three years, moderate blood hypereosinophilia was recorded, with no clinical manifestations and clear cause, still under investigation.