Cardiac involvement in Churg‐Strauss syndrome

Abstract

Objective. Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis. Previous studies showing cardiac involvement in CSS patients were limited in the number of patients and were often based solely on clinical manifestations. The aim of the present study was to determine in detail the incidence of cardiac involvement in a large population of ambulatory CSS patients.Methods. Thirty-two consecutive patients with CSS in remission (mean ؎ SD duration of disease between diagnosis and enrollment 6.1 ؎ 5.8 years, mean ؎ SD age 61 ؎ 10 years) who were previously unaware of cardiac involvement were compared with 32 randomly selected age-and sex-matched control subjects, using clinical evaluation, electrocardiography (EKG), echocardiography, and cardiac magnetic resonance imaging (MRI).Results. Detailed cardiac evaluation revealed a 62% prevalence of cardiac involvement in CSS patients compared with 3% in controls (P < 0.001), with clinical symptoms in 26% and 3%, respectively (P ‫؍‬ 0.009), EKG abnormalities in 66% and 3%, respectively (P < 0.001), and echocardiographic defects in 50% and 3%, respectively (P < 0.001). Cardiac MRI detected cardiac manifestations in 62% of CSS patients. In the presence of cardiac MRI abnormalities, echocardiography could detect cardiac involvement with a sensitivity of 83% and a specificity of 80%. The absence of symptoms or EKG abnormalities did not exclude cardiac involvement, because abnormalities could still be detected in 38% of these patients at the time of echocardiography or cardiac MRI.Conclusion. These results demonstrate a high incidence of cardiac involvement in CSS patients. Systematic cardiac evaluation including detailed imaging is required to properly identify CSS patients with cardiac involvement.

Does One Size Fit All?

“…Moreover, the accuracy of the assessment is dependent on the diagnostic methods used. For instance, we found that detailed cardiac evaluation revealed a 62% prevalence of cardiac involvement in our patients with eosinophilic GPA (previously Churg-Strauss), while only 26% had clinical symptoms 15 . Finally, whether a manifestation is caused by active vasculitis is not always evident nor can it always be proven.…”

“…To reduce CYC exposure, patients with early systemic disease were treated with a milder treatment regimen, i.e., MTX instead of CYC. In the NORAM (Nonrenal Wegener's Granulomatosis treated Alternatively with MTX) trial, MTX was not found to be inferior compared to CYC to induce remission 15 . However, results from the longterm followup are disappointing 16 .…”

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

“…Although endomyocardial infiltration is the dominant condition, pericarditis, vasculitis, valvular heart defects, and abnormalities may also occur [52]. In addition, patients with EGPA have an increased risk of venous thromboembolic events, such as deep venous thrombosis and/or pulmonary embolism [53].…”

Churg-Strauss syndrome: remember cardiac complications too

“…An observational study of 32 ambulatory CSS patients detected cardiac involvement in 62%. 2 In those with abnormal cardiac magnetic resonance imaging (MRI), echocardiography could detect cardiac involvement with 83% sensitivity and 80% specificity. In those with echocardiographic abnormalities, cardiac MRI detected cardiac involvement with 88% sensitivity, but only 72% specificity.…”