Churg Strauss Syndrome Heart Involvement: Churg Strauss Syndrome Forum Question & Responses

Churg Strauss CSS Heart Involvement

For those with heart involvement.

Bradford Lawrence:  Context; My main issues with Churg Strauss Syndrome were the tingling in the legs (ankles and feet ) coupled with nerve pain & some muscle spasms. Hot feeling in the shins too…. I also have nasal polyps and mild asthma. Also, a general feeling of not being well, loss of smell and taste.

Scenario now: It seems my tingling and nerve pain have stopped. For at least 4 days I don’t even notice it anymore.
But I feel a weird feeling in my chest now. Dead center of my chest feels “uneasy”… It’s hard to explain. I feel like I can maybe skip a beat or flutter once every couple of hours. 

When I try to meditate and control my breathing it just feels different in that area. My mind is telling me that this thing has moved to my heart.
I went to the Drs for it and they didn’t see anything on the ekg. Or stress test. Ultra sound was okay.

For those with Churg Strauss Syndrome heart issues is this the way it starts?

What do I need to look for?

What are the symptoms that you had ?

Any feedback would be greatly appreciated…

My cardiologist that I saw has no experience with this and said he really has know way of knowing until damage presents itself.

 

Cardiac involvement in Churg-Strauss syndrome

Brucato A, et al. G Ital Cardiol (Rome). 2015.
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Abstract

Churg-Strauss syndrome, recently renamed eosinophilic granulomatosis with polyangiitis (EGPA), is a rare form of systemic vasculitis, characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia. EGPA is classified as a small and medium-sized vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) and the hypereosinophilic syndrome. Typical clinical features include asthma, sinusitis, transient pulmonary infiltrates and neuropathy. Blood eosinophils are often >1500/µl or more than 10% on the differential leukocyte count. Blood eosinophils should always be tested in unexplained cardiac disorders, and may normalize even after low doses of corticosteroids. ANCA are positive in 40-60% of cases, mainly anti-myeloperoxidase. Heart involvement occurs in approximately 15-60% of EGPA patients, especially those who are ANCA negative. Any cardiac structure can be involved, and patients present with myocarditis, heart failure, pericarditis, arrhythmia, coronary arteritis, valvulopathy, intracavitary cardiac thrombosis. Although cardiovascular involvement is usually an early manifestation, it can also occur later in the course of the disease. A significant proportion of patients with cardiac involvement is asymptomatic. In the absence of symptoms and major ECG abnormalities, cardiac involvement may be detected in nearly 40% of the patients. All patients with EGPA should be studied not only with a detailed history of cardiac symptoms and ECG, but also with echocardiography; if abnormalities are detected, a cardiac magnetic resonance study should be performed.

Coronary angiography and endomyocardial biopsy should be reserved to selected cases. Heart involvement carries a poor prognosis and causes 50% of the deaths of these patients. It is often insidious and underestimated. Optimal therapy is therefore important and based on high-dose corticosteroids plus immunosuppressive agents, particularly cyclophosphamide in case of myocardial inflammation. Thus, early diagnosis of cardiac involvement and subsequent therapy may prevent progression of cardiac disease. At present, the role of troponin and brain natriuretic peptide in monitoring and therapy remains unclear. Orthotopic heart transplantation is feasible in case of severe disease, even if the experience is limited in -EGPA, and optimal post-transplantation immunosuppressive strategy has yet to be defined.

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