Cardiac Involvement in Churg-Strauss Syndrome

Abstract

Cardiac disease is a major contributor to disease-related death in Churg-Strauss syndrome (CSS). We conducted the current study to determine the prevalence and clinical impact of cardiac involvement in CSS patients. We performed a multicenter, cross-sectional analysis of patients diagnosed with CSS. Cardiac workup included electrocardiography, echocardiography, cardiac magnetic resonance imaging (MRI), and endomyocardial biopsy.We analyzed 49 patients with CSS: 22 patients had clinical evidence of cardiac involvement. A negative antineutrophil cytoplasmic antibodies (ANCA) test and much higher eosinophil counts (9947 vs. 3657/microL, respectively, p < 0.001) distinguished patients with cardiac involvement from those without. Impaired left ventricular function (50%), mild to severe valvular insufficiencies (73%), and pericardial effusions (41%) were common findings in these patients. Endomyocarditis was found in 13 patients (59%) as detected by cardiac MRI, cardiac thrombus formation, and endomyocardial biopsy, and was associated with impaired cardiac function. After a mean follow-up of 47 months, most patients had regained or maintained good cardiac function. However, patients with endomyocarditis had a more severe outcome. Two patients died (61 and 99 mo after diagnosis, respectively), both due to severe cardiomyopathy and heart failure.Cardiac involvement is common in patients with CSS and is associated with the absence of ANCA and high eosinophil counts. Endomyocarditis may represent the most severe manifestation eventually causing fatal outcome. A structured clinical assessment incorporating cardiac imaging with echocardiography and MRI can identify impaired cardiac function and endomyocardial abnormalities.

Churg–Strauss syndrome: retrospective study in Burgundian population in France in past 10 years

“…The difference between clinical manifestations of o ur study and the others series like more heart manifestation can maybe explain this low rate of ANCA positivity [22]. The survival rate at 5 years is similar to other series [23].…”

Unrecognized tamponade diagnosed pre-induction by focused echocardiography

“…Churg-Strauss syndrome is a rare systemic vasculitis associated with lateonset asthma, chronic sinusitis, hypereosinophilia, and nonspecific vasculitic symptoms, such as fever, weight loss, rash, and neuropathy. 9,10 Multiple organ system dysfunction may be present and may manifest with pulmonary (e.g., infiltrate, pleural effusion, hemorrhage), integumentary (e.g., purpura, maculopapular rash, ulcer), neurologic (e.g., stroke, polyneuropathy, mononeuritis multiplex), gastrointestinal (e.g., abdominal pain, diarrhea), renal (e.g., glomerulonephritis, renal insufficiency), or cardiac (e.g., ischemic cardiomyopathy, myocarditis, pericarditis, pericardial effusion) complications. 11 Although pericardial effusion is an established manifestation of Churg-Strauss syndrome, progression to cardiac tamponade is rare.…”

“…11 Although pericardial effusion is an established manifestation of Churg-Strauss syndrome, progression to cardiac tamponade is rare. 9,10,12 Establishing the diagnosis of cardiac tamponade by history or physical examination in a patient with preexisting cardiac disease presents significant challenges, as signs or symptoms may be attributed to coexisting disease. Dyspnea is the most sensitive symptom of tamponade (87-88% sensitivity), 13 but it is non-specific in a patient with coexisting cardiac or respiratory disease.…”

Eosinophilic Lung Disease: Accompanied with 12 Cases

“…Renal involvement (most commonly focal and segmental necrotizing glomerulonephritis) and gastrointestinal system involvement (stomachache caused by eosinophilic gastroenteritis and vasculitis) are also reported. Pancreatitis, gastrointestinal perforation, and hemorrhage indicate underlying vasculitis and are accepted as the indicator of a poor diagnosis [25,26,[33][34][35]. In our study, paranasal sinus involvement was present in 3 patients, nasal polyposes in 1 case, skin involvement in 3 cases, cardiac involvement in 2 cases, and neurological involvement in 1 case.…”