| dc.description.abstract | Hereditary angioedema (HAE) is rare; it is usually a
monogenic, genetic disease with autosomal dominant
transmission, resulting in C1-inhibitor deficiency (C1InH).
Renal involvement associated with HAE has been known for
nearly thirty years. We are reporting on two observations of
a father and his daughter presenting a glomerulonephritis
evolving towards to CKD class V. Renal needle biopsy had
evidenced acute vascular lesions associated with segmental
and focal hyalinosis. Since these lesions were not described,
we discussed the possible causal links with HAE. The
accumulation of bradykinin caused by mutation in C1-Inh
could have been misleading, considering all the beneficial
vascular and renal effects widely described in the literature,
if we stick to that fact, in the study of vascular and
glomerular lesions. On the other hand, taken as a whole,
HAE appears to pave the way for the development of a
glomerular and vascular disease: dysimmunity, circulating
immune complexes, and vascular hyperpermeability favor
deposition of immune complexes, overexpression and
activation of B1 receptors | en_US |
