[A CASE OF CHILDHOOD-ONSET EOSINOPHILIC GRANULOMATOUS POLYANGIITIS WITH ASYMPTOMATIC MYOCARDIAL INVOLVEMENT WHO WAS

Erika Takigami1, Masashi Zuiki1, Shinji Akioka1

  • 1Department of Pediatrics, University Hospital, Kyoto Prefectural University of Medicine.

Arerugi = [Allergy]
|July 17, 2019
PubMed

Insights

Eosinophilic granulomatosis with polyangiitis (EGPA) can develop in children with allergic conditions like asthma and atopic dermatitis. Early diagnosis is crucial for better outcomes, especially due to potential cardiac involvement in pediatric cases.

Area of Science:

  • Pediatric Allergy and Immunology
  • Rheumatology
  • Vascular Medicine

Background:

  • Atopic dermatitis and bronchial asthma are common pediatric allergic conditions.
  • Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis often preceded by allergic disorders.

Observation:

  • A case of a young girl with pre-existing atopic dermatitis and asthma who developed EGPA.
  • The patient presented with atypical skin eruptions, hypereosinophilia, sinusitis, and pulmonary nodules.
  • Asymptomatic myocardial involvement was detected via scintigraphy.

Findings:

  • Diagnosis of anti-neutrophil cytoplasmic antibody-negative EGPA was confirmed at age 10.
  • Eosinophil-infiltrating granulomatous vasculitis was identified.
  • Treatment with glucocorticoid and immunosuppressive drugs led to improvement.

Implications:

  • Pediatric-onset EGPA carries a poorer prognosis than adult-onset EGPA, primarily due to cardiac complications.
  • Accurate and timely diagnosis of EGPA is critical for improving patient prognosis.
  • Consider EGPA in children with allergic diseases presenting with atypical or refractory symptoms.

Related Concept Videos

Asthma-I: Introduction01:29

Asthma-I: Introduction

Asthma is a chronic respiratory ailment that requires careful management due to its varying symptoms and influencing factors. It is characterized by airway inflammation, bronchial hyperresponsiveness, and reversible airflow obstruction, leading to symptoms like wheezing, shortness of breath, chest tightness, and coughing. The symptom frequency and intensity may vary considerably over time. It is also linked to immune system responses to allergens and irritants, highlighting the complex...
3.4K
The Bronchial Tree01:23

The Bronchial Tree

The human bronchi and bronchial tree play a crucial role in the respiratory system, facilitating the exchange of oxygen and carbon dioxide. Let's delve into the intricate structure and functions of these respiratory components.
The trachea, commonly known as the windpipe, is a tube that connects the larynx (voice box) to the bronchi. At a point called the carina, it bifurcates into two primary bronchi. The right primary bronchus is wider, shorter, and more vertical than the left primary...
6.4K
Diagnosing Acidosis and Alkalosis01:24

Diagnosing Acidosis and Alkalosis

Diagnosing acid-base imbalances involves systematically analyzing arterial blood samples, focusing on three key measurements: pH, bicarbonate (HCO3−) concentration, and carbon dioxide partial pressure (PCO2). This analysis follows a four-step process that helps identify the imbalance's underlying cause and nature.
First, the pH level is assessed to determine whether the blood pH is normal (7.35–7.45), low (acidosis), or high (alkalosis).
Next, the PCO2  and...
1.1K
Asthma-II: Pathophysiology and Classification01:26

Asthma-II: Pathophysiology and Classification

Asthma is a prevalent chronic respiratory condition marked by inflammation and hyperresponsiveness of the airways. Its pathophysiology involves complex interactions among inflammatory pathways, immune responses, and neural mechanisms.
Additionally, environmental and genetic factors play crucial roles in determining an individual's susceptibility to asthma and the severity of their condition.
Critical processes in asthma pathophysiology include:
4.2K
Asthma: Pathogenesis and Management01:20

Asthma: Pathogenesis and Management

Asthma is a chronic pulmonary condition involving inflammation of the airways, hyper-reactivity, and reversible obstruction of the airways. This condition can significantly impact a person's quality of life, making breathing difficult and leading to distressing symptoms.
Asthma is classified as allergic and non-allergic. Allergens such as dust mites, pollen, and pet dander trigger allergic asthma, while factors like cold air, intense emotions, or exercise can induce non-allergic asthma.
1.3K
Asthma-III: Symptoms and Complications01:24

Asthma-III: Symptoms and Complications

Asthma, a common chronic respiratory condition, is classified considering the frequency and severity of symptoms alongside lung function impairment. Understanding this classification is essential for appropriate treatment and management. Here's a detailed look at the classification of asthma and its clinical features and complications:
Classification of Asthma
3.3K