Eosinophilic myocarditis as a first presentation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Bluett, Ronan; McDonnell, David; O’Dowling, Claire; Vaughan, Carl J.

doi:10.1136/bcr-2017-221227

Cited by 12 publications

(7 citation statements)

References 13 publications

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“…Primary results have shown a similar molecular basis of HES and EGPA which may actually represent the same disease [5]. Cardiac involvement from EGPA may resemble eosinophilic endomyocarditis and is generally accompanied by high eosinophil counts and negative ANCA [6]. Therefore, and while clonal eosinophilia and most causes of reactive eosinophilia seemed to be ruled-out in our patient, some vasculitis/EGPA components were still present.…”

Section: Discussionsupporting

confidence: 57%

An Almost Fatal Case of the Hypereosinophilic Syndrome: A Dilemma Between Vasculitis or Clonal Disorder

Kessel¹,

Paz²,

Solopov³

et al. 2019

JCMCR

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Hypereosinophilic syndrome (HES) is a rare and potentially life-threatening disorder. While many conditions can cause eosinophilia, it can also be provoked by rarer diseases such as vasculitis or myeloproliferative disorders. Here we present an almost fatal presentation of HES characterized with both vasculitis and clonal features. Treatment with combined chemo-immunotherapy allowed reversal of most of the complications. There is still much uncertainty in the diagnosis and management of HES, and research should be done to improve knowledge on pathophysiology and cytogenetics of this disease in order to improve treatment and prevent life-threatening complications.

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Section: Discussionsupporting

confidence: 57%

An Almost Fatal Case of the Hypereosinophilic Syndrome: A Dilemma Between Vasculitis or Clonal Disorder

Kessel¹,

Paz²,

Solopov³

et al. 2019

JCMCR

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“…Myocarditis is another form of cardiac manifestations of CSS, presenting as chest pain, arrhythmias, or cardiogenic shock. Some reports suggest an increased risk of myocarditis in young adults between the ages of 20 to 30 years [11]. There is a marked increase in eosinophil counts in patients with myocarditis, according to some reports [12].…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) Mimicking a Stroke and Acute Coronary Syndrome: A Case Report

Ishaq

Malik

Baig

et al. 2020

Cureus

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Churg-Strauss syndrome (CSS) is a rare disease of multiple organ involvement attributed to asthma, eosinophilia, and vasculitis as a diagnostic criterion. Here we report a case of CSS presenting with left leg weakness and chest pain with a diagnosis of myocarditis and neuropathy. Eosinophilia, history of asthma, peripheral neurological damage led to the diagnosis of CSS. Transthoracic echo showed a full-sized segmental wall motion abnormality with normal CT angiography. He responded well to steroid therapy.

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“… 3 Regarding anti-neutrophil cytoplasmic antibody (ANCA) status, CSS can be divided into two major subsets: the ANCA-positive patients, who demonstrate clinical and histopathologic features of vasculitis, and the ANCA-negative patients, who exhibit tissue eosinophilic infiltration. 4 Eosinophilic myocarditis 5 , neuroendocrine carcinoma 6 , eosinophilic vasculitic neuropathy 7 , multiple oral ulcerations 8 , and inflammatory pseudotumor of the anterior orbit 9 have been reported as initial presentations of CSS in the literature. Herein, we describe a patient with undiagnosed CSS who developed simultaneous bilateral CRAO.…”

Section: Introductionmentioning

confidence: 99%

ANCA-Negative Churg-Strauss Syndrome Presenting as Bilateral Central Retinal Artery Occlusion: A Case Report

Nikandish¹,

Saremi²

2021

tjo

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A 42-year-old man with undiagnosed Churg-Strauss syndrome (CSS) developed bilateral central retinal artery occlusion (CRAO). His medical history included bronchial asthma and irregular prednisolone usage but no atherosclerotic risk factors. At presentation, visual acuity (VA) was hand motion in the right eye and counting fingers in left eye. On fundoscopy, retinal whitening and a cherry red spot were observed in the right eye, while the fundus was normal in the left eye. After eyeball massage and systemic intraocular pressure lowering agents, his VA improved. On day 5 of treatment, he experienced right limb weakness and purpura on his right foot, and electromyography revealed mononeuritis multiplex. Laboratory tests indicated eosinophilia (52%). Based on the presence of hypereosinophilia, bronchial asthma, mononeuritis multiplex, vasculitis purpura, and sinusitis that was detected during etiological investigations, the patient was diagnosed as having CSS according to the American College of Rheumatology diagnostic criteria. Intravenous methylprednisolone 1 g/day was administrated for 3 consecutive days and 1 g cyclophosphamide was started and continued monthly for 6 months. Foot drop and vasculitic purpura improved after 7 days, but there was no further improvement in visual acuity. In conclusion, in the presence of bilateral CRAO and lack of atherosclerotic risk factors, CSS should be considered as a predisposing factor and investigations should be conducted accordingly.

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Eosinophilic myocarditis as a first presentation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Cited by 12 publications

References 13 publications

An Almost Fatal Case of the Hypereosinophilic Syndrome: A Dilemma Between Vasculitis or Clonal Disorder

An Almost Fatal Case of the Hypereosinophilic Syndrome: A Dilemma Between Vasculitis or Clonal Disorder

Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) Mimicking a Stroke and Acute Coronary Syndrome: A Case Report

ANCA-Negative Churg-Strauss Syndrome Presenting as Bilateral Central Retinal Artery Occlusion: A Case Report

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