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2017 ; 6
(1
): 91-97
Nephropedia Template TP
gab.com Text
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English Wikipedia
Two cases of atypical hemolytic uremic syndrome (aHUS) and eosinophilic
granulomatosis with polyangiitis (EGPA): a possible relationship
#MMPMID28509134
Cao M
; Ferreiro T
; Leite BN
; Pita F
; Bolaños L
; Valdés F
; Alonso A
; Vázquez E
; Mosquera J
; Trigás M
; Rodríguez S
CEN Case Rep
2017[May]; 6
(1
): 91-97
PMID28509134
show ga
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by
hemolysis, thrombocytopenia, and renal failure. It is related to genetic
mutations of the alternative complement pathway and is difficult to differentiate
from other prothrombotic microangiopathies. Eosinophilic granulomatosis with
polyangiitis (EGPA) (Churg-Strauss syndrome, CSS) is a systemic ANCA-associated
vasculitis and a hypereosinophilic disorder where eosinophils seem to induce cell
apoptosis and necrosis and therefore, vasculitis. Here, we report the case of two
CSS patients with a genetic complement disorder consistent with aHUS diagnosis.
Both patients showed histologic features that supported the diagnosis of CSS, and
a genetic complement study confirmed the suspected aHUS diagnosis. In the case
where eculizumab was administered, the global response was excellent. There is
very limited understanding of the genetics and epidemiology of both, atypical HUS
and EGPA, but considering our two patients we suggest that an etiopathogenic link
exists among patients diagnosed with both entities.