Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis.
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.
Neumann
Thomas
T
From Department of Internal Medicine 3 (TN, GW, GH), Department of Internal Medicine 1 (CK), and Institute of Diagnostic and Interventional Radiology (AH, WAK), University of Jena, Jena; Department of Internal Medicine 3 and Institute for Clinical Immunology (BM, GS, JZ), and Department of Internal Medicine 2 (MS), University of Erlangen-Nuremberg, Erlangen; Department of Cardiology (DR), Evangelisches Krankenhaus, Hamm; and Institute of Pathology (GM), Klinikum Darmstadt, Darmstadt, Germany.
Manger
Bernhard
B
Schmid
Michael
M
Kroegel
Claus
C
Hansch
Andreas
A
Kaiser
Werner A
WA
Reinhardt
Dirk
D
Wolf
Gunter
G
Hein
Gert
G
Mall
Gerhard
G
Schett
Georg
G
Zwerina
Jochen
J
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D015267:Churg-Strauss Syndrome / Q000150:complications*; D003430:Cross-Sectional Studies; D004359:Drug Therapy, Combination; D004452:Echocardiography; D004562:Electrocardiography; D004804:Eosinophils / Q000473:pathology; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009205:Myocarditis / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D009206:Myocardium / Q000473:pathology; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D055815:Young Adult
D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents