Understanding Cardiac Disease in EGPA

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg–Strauss syndrome, is a rare form of systemic vasculitis that causes inflammation of small- and medium-sized blood vessels throughout the body. It typically develops in people with long-standing asthma or allergies and is marked by elevated eosinophils—white blood cells that play a role in allergic responses. While EGPA can affect many organs, its impact on the heart is among the most serious and can be life-threatening.

Cardiac involvement occurs in roughly one-third of individuals with EGPA, making it a major contributor to illness severity and mortality. Heart complications can take several forms. Pericarditis, or inflammation of the sac surrounding the heart, may lead to chest pain and fluid buildup. Cardiomyopathy can weaken the heart muscle, reducing its ability to pump effectively and potentially causing heart failure. Another particularly severe manifestation is eosinophilic myocarditis, in which eosinophils infiltrate heart tissue directly. This infiltration can trigger inflammation, scarring (fibrosis), and electrical abnormalities that disrupt normal heart rhythm. Over time, these processes compromise cardiac function and increase the risk of long-term damage.

Because heart involvement dramatically worsens prognosis, early detection is essential. Modern diagnostic tools offer valuable insights into cardiac health in EGPA. Echocardiography can identify structural abnormalities and reduced heart function, while cardiac MRI provides detailed images that reveal inflammation and scarring. In some cases, coronary angiography is used to rule out blockages or assess blood vessel inflammation. Detecting changes early allows clinicians to intervene before damage becomes irreversible.

Treatment typically centers on rapidly reducing inflammation. High-dose corticosteroids remain the cornerstone of therapy, often combined with immunosuppressive medications such as cyclophosphamide or azathioprine for more severe disease. Newer biologic therapies that target specific components of the immune system may also benefit certain patients, offering more precise control of inflammation with fewer long-term side effects. Effective management usually requires collaboration among specialists, including rheumatologists, cardiologists, and pulmonologists.

In summary, the potential for serious heart complications makes cardiac monitoring a critical component of EGPA care. Timely diagnosis, appropriate imaging, and early, aggressive treatment can significantly improve outcomes. A coordinated, multidisciplinary approach helps ensure that patients receive comprehensive care tailored to the complex nature of this rare condition.
Understanding Cardiac Disease in EGPA