Impaired human ovarian follicular fluid complement function in hereditary angioedema

We sought and detected functionally active complement in human ovarian foll icular fluid obtained during the peri-ovulatory period. All the functional complement activities tested, including total haemolytic complement, classi cal pathway activity, alternative pathway activity and C1 inhibitor functio n were present with values within the normal serum range. Active complement in follicular fluid is relevant for the function of the enzymatic multifac torial mechanism of ovulation. The presence in hereditary angioedema patien ts of both complement (C1 inhibitor deficiency and chronically consumed com plement) and ovarian abnormalities (cystic ovaries), led us to study comple ment function in the follicular fluid of women of reproductive age affected with hereditary angioedema. In contrast to healthy women, hereditary angio edema patients showed dramatically reduced classical pathway activity and u ndetectable functional and antigenic C1 inhibitor. C4 was very low, while C 3 and B were slightly reduced or within the normal serum range. This comple ment profile was also detected in patients' sera. Since hereditary angioede ma patients often show cystic ovaries (polycystic or multifollicular), the presence of multifollicular ovaries in the two patients studied, along with complement dysfunction, may be relevant. These findings, as well as the no rmalisation of the ovaries found by us in hereditary angioedema patients an d in the patients reported here who were undergoing danazol treatment, and the increase in C1 inhibitor and the improvement of clinical symptoms, sugg est a further link between complement and ovarian function.