Pathogenetic and Clinical Aspects of C1 Inhibitor Deficiency

Cicardi, Marco; Bergamaschini, Luigi; Cugno, Massimo; Beretta, Andrea; Zingale, Lorenza C.; Colombo, Monica; Agostoni, A

doi:10.1016/s0171-2985(98)80041-7

Cited by 88 publications

(70 citation statements)

References 31 publications

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“…The presence of autoantibodies of IgM and IgG class binding to Cl INH confirms the diagnosis, although we have not examined their neutralizing activity. Type II AAE is a rare disorder and the worldwide incidence of type II AAE reported to date is less than 100 cases (12)(13)(14)(15)(16)(17)(23)(24)(25)(26)(27)(28). To our knowledge, this is the first case of type II AAEin Japan, although two cases of AAEhave been reported in the literature (29,30).…”

Section: Discussionmentioning

confidence: 87%

Autoimmune Acquired Form of Angioedema that Responded to Danazol Therapy.

Higa

Hirata

et al. 2002

Intern. Med.

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A 51-year-old man with recurrent episodes of angioedema was diagnosed as having autoimmuneacquired angioedema, based on adult onset, lack of apparent family history, decreased activity of Cl esterase inhibitor (Cl INH) and CH50, decreased levels of serum C4 and Clq and the presence of autoantibodies to Cl INH. The danazol treatment relieved the symptoms of angioedemaand increased the Cl INHactivity and concentration with the normalization of CH50, Clq and C4 levels. To our knowledge, this is the first case of autoimmune acquired angioedema in Japan. (Internal Medicine 41 : 398-402, 2002)

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Section: Discussionmentioning

confidence: 87%

Autoimmune Acquired Form of Angioedema that Responded to Danazol Therapy.

Higa

Hirata

et al. 2002

Intern. Med.

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“…The end result is increased vascular permeability and massive uncontrolled oedema, but the precise chemical responsible for the oedema is still unknown. 5 …”

mentioning

confidence: 99%

Current management of hereditary angio-oedema (C'1 esterase inhibitor deficiency)

Fay¹,

Abinun²

2002

Journal of Clinical Pathology

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“…Os outros 15% têm angioedema do tipo II, na qual a C1-INH é estrutural e funcionalmente anormal, embora permaneça com níveis séricos normais ou mesmo elevados. A ansiedade e/ou o trauma podem precipitar crises de edema nestes pacientes 61 . O mecanismo de precipitação do edema ainda não está bem esclarecido.…”

Section: Complemento: Ativação Regulação E Deficiênciasunclassified

“…Acredita-se que esta cinina seja responsável pela precipitação do edema no angioedema. A bradicinina também é uma candidata 61 .…”

Section: Complemento: Ativação Regulação E Deficiênciasunclassified