368 Angioedema in patients with hypogonadism or under anti-androgen treatment

“…Es gibt jedoch auch Berichte über das Auftreten menstruationsabhängiger nicht-hereditärer Angioödeme, die nach Einnahme von oralen Kontrazeptiva, in der Schwangerschaft und unter Therapie mit Tranexamsäure verschwanden [25]. Der aggravierende Einfluß östro-genhaltiger Kontrazeptiva geht aus unserem Patientengut hervor und bestä-tigt die Erkenntnisse anderer Untersucher [6,11,34,39]. Die Erstmanifestationen des HAE nach Einnahme östrogenhaltiger Kontrazeptiva sollte Veranlassung sein, nach einem C1-INHDefekt zu suchen und auf andere Mög-lichkeiten der Kontrazeption zu orientieren.…”

Section: Manifestationsalterunclassified

“…Die Erstmanifestationen des HAE nach Einnahme östrogenhaltiger Kontrazeptiva sollte Veranlassung sein, nach einem C1-INHDefekt zu suchen und auf andere Mög-lichkeiten der Kontrazeption zu orientieren. Der biochemische Hintergrund von Angioödemen in Verbindung mit Verschiebung des Sexualhormongleichgewichts bei Frauen ohne C1-INH-Defekte ist unbekannt [24,34,41].…”

Section: Manifestationsalterunclassified

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Untersuchungen zum hereditären Angioödem im deutschsprachigen Raum

Göring

Bork

Späth³

et al. 1998

Der Hautarzt

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A multicentre, retrospective study of hereditary deficiency of C1-esterase inhibitor (C1-INH) function, a deficiency which clinically manifests as hereditary angioedema (HAE), was performed in six centres in Germany, Austria and Switzerland. 242 individuals were registered with proven functional or quantitative deficiency of C1-INH who belonged to kindered with disease manifestation in 2 to 6 generations. Considering the total population in the three countries and the number of registered individuals, a frequency of the deficiency of 0.02 x 10(-4) was calculated. As this epidemiological study involved only 6 centres, a 10 to 100 times higher frequency of C1-INH deficiency is estimated to be a more realistic value. Out of the 242 registered individuals 110 were evaluated for type and location of clinical manifestation of the deficiency, the laboratory data and the therapy outcome. 86 (78.2%) of the patients belonged to the "common type" and 24 (21.8%) to the "variant type" of HAE. In 53.9% of the cases first manifestation of the disease was before the age of 20 years. In only 3.9% of the patient population did the disease begin after 40 years of age. A mean time lag of 5,3 years was observed, between the first manifestation and correct diagnosis. Initial diagnosis was correct in only 31.8% of the cases of which dermatologists provided 51.7%. False diagnoses include urticaria (41.3%), allergy (20%), acute abdomen (18.7%), angina (8%), rheumatoid disease (5.3%) and intracranial haemorrhage, CNS tumour, epilepsy, migraine (5.3%). The distribution pattern of HAE resembled that of intolerance reactions and pseudoallergies. Urticarial lesions were not associated with C1-INH deficiency. 24% of the patients had at least one episode of laryngeal edema. 40% of patients were unable to identify a trigger of edema formation. The others indicated as triggers trauma, hormonal changes, mental stress, insect stings and in a few cases food and drugs. Menstruation and oral contraceptives aggravated or made disease manifestations more frequent. In contrast, during pregnancy in many cases clinical manifestations improved and delivery posed no problems. The possibility of HAO is very much suggested by the tailure of edema to respond to classical anti-allergic therapy. Therapy of choice of acute attacks is C1-INH concentrate. No side reactions, antibody formation or virus transmission have been observed. For long term prophylaxis danazol, an attenuated androgen, or tranexamic acid, a protease inhibitor, was chosen. The daily dose of danazol should be kept as low as possible because of its anabolic, anti-estrogenic, anti-gestagenic, and anti-gonadotropic effects. Indeed, adverse reactions were observed in 41.7% of patients receiving danazol. Frequencies of adverse reactions were twice as common in women as in men. Adverse reactions were dose dependent and reversible except for one woman with irreversible deepening of her voice. Measuring C1r is a effective way to assess C1-INH function and monitor therapy.

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“…The levels of C1/C4 and the C1-esterase inhibitor functional activity usually are normal in cases of visceral angioedema induced by ACE inhibitors [2,8,9]. The following mechanisms are possible explanations: 1) bradykinin and substance P buildup secondary to the affect of ACE inhibitor may lead to the inflammatory response, therefore increasing permeability of the vascular compartment; 2) deficiency of complement and enzymes carboxypeptidase N/alpha 1 antitrypsin; 3) antibody-antigen reaction [10]; 4) effect of hormones such as estrogen and progesterone due to high prevalence of angioedema in female gender [11]; 5) contrast media used for the imaging [12]; 6) genetic predisposition; 7) inflammation due to acute phase proteins; 8) C1 inhibitor deficiency or dysfunction and many other theories which are actively being explored [11,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29]. The increase in bradykinin levels and its metabolites remains to be the most plausible explanation [19].…”

mentioning

confidence: 99%