IS Case 7: Cardiac Involvement of Churg-Strauss Syndrome
Imaging Sciences URMC 2008
Publication Date: 2009-05-18
Delayed patchy enhancement within the right ventricle with mild thickening of the right ventricular myocardium. Subendocardial late enhancement accompanied by global hypokinesis with left ventricular hypertrophy.
Cardiac involvement of Churg Strauss syndrome.
The diagnosis of Churg Strauss syndrome was confirmed by histopathology.
Differential Diagnosis: There is marked endomyocardial fibrosis that is visualized by cardiac magnetic resonance imaging using a late enhancement technique. The findings are typical for delayed wash-out of contrast medium due to interstitial fibrosis with increased extracellular space.
Cardiac Manifestations of Churg Strauss syndrome
- Vasculitis of the epicardial coronary arteries
- Vasculitis of the small intramyocardial vasculature
- Granulomatous myocardial infiltration
- Eosinophilic myocarditis
- Myocardial fibrosis
- Right ventricular endomyocardial fibrosis
- Right ventricular and/or left ventricular intracavitary intra-cavitary thrombus
- Restrictive cardiomyopathy
- Dilated cardiomyopathy
- Mitral regurgitation due to papillary muscle dysfunction
- Acute pericarditis
- Pericardial tamponade
- Constrictive pericarditis
- Supraventricular arrhythmias
- Ventricular arrhythmias
- Atrio-ventricular and intra-ventricular conduction disorders
- Sudden cardiac death
- Loeffler’s Syndrome: Indistinguishable by MRI. Endocarditis is the classical manifestation of hypereosinophilic syndrome. Similarities between Loeffler and Churg–Strauss syndromes had been described previously. Potentially, endocardial involvement is more common even in Churg–Strauss syndrome. The significance of these findings remains to be shown.
- Myocardial Infarction: Similar, but regional limited findings are known for subendocardial fibrosis following myocardial infarction. MI is usually accompanied by local wall motion disturbances. Also, late enhancement imaging is sufficient to visualize myocarditis associated with Churg Strauss syndrome that is usually localized to the subepicardial layer.
Churg-Strauss Syndrome (CSS) is a rare systemic vasculitis that is non-heritable, non-transmissible and often mis-diagnosed. This disease was first described in 1951 by Dr. Jacob Churg and Dr. Lotte Strauss as a syndrome consisting of “asthma, eosinophilia [an excessive number of eosinophils in the blood], fever, and accompanying vasculitis of various organ systems”. The disease is characterized by three phases: 1) allergic rhinitis and asthma; 2) eosinophilic infiltrative disease, such as eosinophilic pneumonia or gastroenteritis; and 3) systemic medium- and small-vessel vasculitis with granulomatous inflammation. The vasculitic phase usually develops within three years of the onset of asthma, although it may be delayed for several decades. The most prominent symptoms and signs are those related to pulmonary, cardiac, dermatologic, renal, and peripheral nerve involvement.
The incidence of Churg Strauss syndrome in the US is 1-3 cases per 100,000 adults per year and approximately 2.5 cases per 100,000 adults per year worldwide. The principal causes of morbidity and mortality are myocarditis and myocardial infarction secondary to coronary arteritis. With treatment, the 1-year survival rate is 90% and the 5-year survival rate is 62%.
Typical histological alterations of Churg–Strauss syndrome include leukocytoclastic or necrotizing vasculitis, eosinophilic tissue infiltration, vascular and extravascular granulomas. Cardiac involvement occurs in up to 60% of patients with Churg Strauss syndrome and in up to 75% with hypereosinophilic syndrome with persistent eosinophilia of 1500/mm3. Cardiac involvement is usually characterized by acute and constrictive pericarditis, myocarditis, and ischemic cardiomyopathy.
Endomyocardial fibrosis of Churg Strauss syndrome, similar to Loeffler syndrome, has rarely been described in the medical literature even though cardiac involvement accounts for 48% of deaths. This observation was originally documented by Churg and Strauss during the autopsies of their patients. Since development of restrictive filling pattern is crucial for the prognosis, cardiac magnetic resonance imaging seems to be very useful to assess endocardial involvement in Churg–Strauss syndrome.
- Smedema JP, van Paassen P, van Kroonenburgh MJ, Snoep G, Crijns HJ, Tervaert JW. Cardiac involvement of Churg Strauss syndrome demonstrated by magnetic resonance imaging. Clin Exp Rheumatol. 2004; 22 (suppl. 36) S75-S78. [PMID: 15675140]
- Alter P, Maisch B. Endomyocardial fibrosis in Churg–Strauss syndrome assessed by cardiac magnetic resonance imaging. Int J Cardiol. 2006; 108: 112– 113. [PMID: 16516704]
- Petersen SE, Kardos A, Neubauer S. Subendocardial and papillary muscle involvement in a patient with Churg-Strauss syndrome, detected by contrast enhanced cardiovascular magnetic resonance. Heart 2005;91. [PMID: 15604316]
- Sonneville R, Lagrange M, Guidoux C, Michel M, Khellaf M, Russel S, Hosseini H. [The association of cardiac involvement and ischemic stroke in Churg Strauss syndrome]. [French] Rev Neurol (Paris). 2006 Feb; 162(2):229-32. [PMID: 16518264]