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Heart involvement in Churg-Strauss syndrome: retrospective study in French Burgundy population in past 10 years
J Vinit1, P Bielefeld, G Muller
1Department of Internal Medicine and Systemic Diseases, General Hospital, University Hospital, 3 rue Faubourg Raines, Dijon Cedex, France. julien.vinit@chu-dijon.fr
Insights
Heart lesions are common in Churg-Strauss syndrome (CSS) and often underestimated. These cardiac issues may stem from a unique pathway independent of ANCA antibodies.
Area of Science:
- Cardiology
- Rheumatology
- Internal Medicine
Background:
- Heart manifestations in Churg-Strauss syndrome (CSS) are diverse and challenging to differentiate from other causes early on.
- Understanding the specific characteristics of cardiac involvement in CSS is crucial for accurate diagnosis and management.
Purpose of the Study:
- To compare the clinical features of patients with heart manifestations directly linked to CSS versus those with cardiac issues from other causes.
- To investigate the relationship between cardiac symptoms and CSS in a hospitalized patient cohort.
Main Methods:
- Retrospective analysis of clinical symptoms in patients hospitalized with CSS between 1998 and 2008 in Burgundy, France.
- Correlation of cardiac symptoms with CSS diagnosis and assessment of associated clinical and laboratory findings.
Main Results:
- Twenty out of 31 CSS patients exhibited heart lesions, with 75% directly attributable to CSS.
- CSS-related heart lesions were associated with younger patient age, earlier cardiac symptom presentation, and higher leukocyte and eosinophil counts.
- Patients with CSS-related heart lesions showed fewer initial digestive symptoms, lower lung infiltrates, and less frequent anti-MPO pANCA positivity.
Conclusions:
- Cardiac lesions are frequent, severe, and likely underestimated in Churg-Strauss syndrome.
- A distinct pathophysiological mechanism, potentially ANCA-independent, appears to underlie CSS-related heart lesions.
Introduction:
Heart manifestations of Churg-Strauss syndrome (CSS) are varied. In the early stages of the disease, it is difficult to distinguish between lesions that are specific to CSS and those of other etiologies. The aim of our work was to compare the characteristics of patients with heart manifestations linked or not to Churg-Strauss syndrome.
Material And Methods:
We recorded all clinical symptoms of patients with CSS hospitalized between 1998 and 2008 in Burgundy, France, and determined the possible relationships between heart symptoms and CSS.
Results:
From a cohort of 31 patients, we found 20 with heart lesions. When heart lesions were present, we noted fewer initial symptoms of digestive disorders (p<0.05), lower levels of lung infiltrates and fewer anti-MPO pANCA (p<0.05). Heart lesions were linked to CSS in 75% of cases. Their patients were thus younger than those in the other cardiac patients (p<0.05), were more likely to have clinical manifestations of heart involvement at diagnosis, were less likely to have lung infiltrates on the X-ray at diagnosis and during flare-ups and less likely to have lung abnormalities on X-rays during flare-ups (p<0.05) and higher level of leucocytes and eosinophils at diagnosis.
Conclusion:
Heart lesions directly attributable to CSS are frequent, severe and probably underestimated. A specific physiopathology that is not mediated by ANCA seems to be involved in the genesis of CSS-related heart lesions.
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