Eosinophilic cardiomyopathy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Eosinophillic Cardiomyopathy also known as Loeffler's Syndrome is a type of restrictive cardiomyopathy caused by eosinophillic infiltration of the endomyocardium.[1] Eosinophills enter the tissue and undergo degranulation, releasing cytotoxic proteins, increasing production of reactive oxygen species, enzymes, growth factor, and cytokines. This process leads to tissue damage and dysfunction, eventually leading to fibrosis and restrictive cardiomyopathy. There are many causes of Eosinophillic Cardiomyopathy, these can be classified as:

  • Reactive[2]
  • Eosinophillic Granulomatosis Polyangitis
    • Churg Strauss Disease
    • Parasitic Infection
    • Autoimmune disorder
    • Medication related
  • Clonal Myeloid Disorder[3]
  • Idiopathic Hypereosinophillic Syndrome [4]

Historical Perspective

  • Eosinophillic Cardiomypoathy was first discovered by Wilhem Loeffler, a Swiss Physician, in 1936 during/following [event].[5] [6]
  • In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

  • Eosinophilic Cardiomyopathy may be classified according to cause into three subtypes/groups:
  • Reactive
  • Clonal myeloid disorder
  • Idiopathic hypereosinophillic syndrome
  • Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

  • The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
  • The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
  • On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Clinical Features

Differentiating [disease name] from other Diseases

  • [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
  • [Differential dx1]
  • [Differential dx2]
  • [Differential dx3]

Epidemiology and Demographics

  • The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
  • In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Patients of all age groups may develop [disease name].
  • [Disease name] is more commonly observed among patients aged [age range] years old.
  • [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

  • [Disease name] affects men and women equally.
  • [Gender 1] are more commonly affected with [disease name] than [gender 2].
  • The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

  • There is no racial predilection for [disease name].
  • [Disease name] usually affects individuals of the [race 1] race.
  • [Race 2] individuals are less likely to develop [disease name].

Risk Factors

  • Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

  • The majority of patients with [disease name] remain asymptomatic for [duration/years].
  • Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
  • If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

  • The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • [Disease name] is usually asymptomatic.
  • Symptoms of [disease name] may include the following:
  • [symptom 1]
  • [symptom 2]
  • [symptom 3]
  • [symptom 4]
  • [symptom 5]
  • [symptom 6]

Physical Examination

  • Patients with [disease name] usually appear [general appearance].
  • Physical examination may be remarkable for:
  • [finding 1]
  • [finding 2]
  • [finding 3]
  • [finding 4]
  • [finding 5]
  • [finding 6]

Laboratory Findings

  • There are no specific laboratory findings associated with [disease name].
  • A [positive/negative] [test name] is diagnostic of [disease name].
  • An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
  • Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

  • There are no [imaging study] findings associated with [disease name].
  • [Imaging study 1] is the imaging modality of choice for [disease name].
  • On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
  • [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

  • [Disease name] may also be diagnosed using [diagnostic study name].
  • Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

  • There is no treatment for [disease name]; the mainstay of therapy is supportive care.
  • The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
  • [Medical therapy 1] acts by [mechanism of action 1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

  • Surgery is the mainstay of therapy for [disease name].
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
  • [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

  • There are no primary preventive measures available for [disease name].
  • Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
  • Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

References

  1. Christopher C. Cheung, Maggie Constantine, Amir Ahmadi, Carolyn Shiau & Luke Y. C. Chen (2017). "Eosinophilic Myocarditis". The American journal of the medical sciences. 354 (5): 486–492. doi:10.1016/j.amjms.2017.04.002. PMID 29173361. Unknown parameter |month= ignored (help)
  2. Christopher C. Cheung, Maggie Constantine, Amir Ahmadi, Carolyn Shiau & Luke Y. C. Chen (2017). "Eosinophilic Myocarditis". The American journal of the medical sciences. 354 (5): 486–492. doi:10.1016/j.amjms.2017.04.002. PMID 29173361. Unknown parameter |month= ignored (help)
  3. Christopher C. Cheung, Maggie Constantine, Amir Ahmadi, Carolyn Shiau & Luke Y. C. Chen (2017). "Eosinophilic Myocarditis". The American journal of the medical sciences. 354 (5): 486–492. doi:10.1016/j.amjms.2017.04.002. PMID 29173361. Unknown parameter |month= ignored (help)
  4. Christopher C. Cheung, Maggie Constantine, Amir Ahmadi, Carolyn Shiau & Luke Y. C. Chen (2017). "Eosinophilic Myocarditis". The American journal of the medical sciences. 354 (5): 486–492. doi:10.1016/j.amjms.2017.04.002. PMID 29173361. Unknown parameter |month= ignored (help)
  5. Christopher C. Cheung, Maggie Constantine, Amir Ahmadi, Carolyn Shiau & Luke Y. C. Chen (2017). "Eosinophilic Myocarditis". The American journal of the medical sciences. 354 (5): 486–492. doi:10.1016/j.amjms.2017.04.002. PMID 29173361. Unknown parameter |month= ignored (help)
  6. Amit Alam, Shankar Thampi, Shahryar G. Saba & Rita Jermyn (2017). "Loeffler Endocarditis: A Unique Presentation of Right-Sided Heart Failure Due to Eosinophil-Induced Endomyocardial Fibrosis". Clinical medicine insights. Case reports. 10: 1179547617723643. doi:10.1177/1179547617723643. PMID 28890659.

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