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A case of eosinophilic polyangiitis with granulomatosis that evolved to cardiac arrest due to advanced atrioventricular block

Sakurai, Yuichiro Oshikata, Chiyako Katayama, Takaharu Takagi, Shunsuke Kaneko, Yasushi Yo, Kikuo Kaneko, Takeshi Kubota, Hiroyuki Matsubara, Takashi Tsurikisawa, Naomi 名古屋大学

2023.02

概要

Eosinophilic granulomatosis with polyangiitis (EGPA; also known as Churg–Strauss syndrome)
is a rare disease characterized by the presence of allergic granulomatosis and necrotizing vasculitis
with tissue and peripheral blood eosinophilia.1
In EGPA, mortality rate and prognosis are related to disease severity, which is assessed by
using a five-factor score (currently the 2009 Revised Five-Factor Score) where each of five
factors (ie, age ≥ 65 years, severe cardiac involvement, severe gastrointestinal tract involvement,
renal insufficiency, and absence of sinusitis) are scored as 0 for absent or 1 for present.2 Among
these five factors, severe cardiac involvement is the major cause of mortality in EGPA patients.
For example, survival rate at 6 years after diagnosis of myocardial involvement is reported to be
only one-third that in the absence of myocardial involvement.3 Also, EGPA patients with cardiac
involvement have a lower 5-year survival rate4 and poorer long-term prognosis than patients
without cardiac involvement.5
The most commonly reported the percentage of patients showing each cardiac involvement
among patients with EGPA are myocarditis (37.1%–89.5%), pericarditis (9.4%–50.4%), heart
failure (36.8%–51.6%), and arrhythmia (12.5%–27.4%).6-10 With respect to arrhythmia, there
are fewer reports in the literature describing bradycardia in EGPA patients—for example as a
result of bundle branch block,8 advanced atrioventricular block,9 or first-degree atrioventricular
block11—than those describing tachycardia.7-10,12-15
Here, we report a rare case of EGPA in which the patient presented with bradycardia due
to atrioventricular block that evolved into cardiac arrest; treatment by steroid pulse could be
survival, following the patient to withdraw from an external pacemaker. ...

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参考文献

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