Churg-Strauss syndrome

A 79–year-old woman with a history of asthma and chronic sinusitis came to our emergency department because of dyspnoea, abdominal pain, nausea, vomiting and weight loss. There were petechiae on the patient’s lower back and lower extremities. Complete blood count showed hypereosinophilia (>> 0.5 x 10⁹ cells/ L).

Because cardiac enzymes were elevated (troponin of > 1800 µg/L), echocardiography was performed, showing a moderate/severe reduction of the left ventricular function (LVF) and some pericardial effusion. Computed tomography (CT) of chest and abdomen revealed bilateral pleural effusion, mediastinal and hilar lymphadenopathy and a clearly distinctive hypodense aspect of the LV endocardium (Fig. 1). To further evaluate the hypereosinophilic blood count, the patient underwent skin and bone marrow biopsies. They all showed eosinophilia, but no evidence of eosinophil dysplasia was found. The patient was tested negatively for antineutrophil cytoplasmatic antibodies (ANCA). On cardiac MR (CMR) an overall decrease in systolic contraction and an impaired thickening of the LV wall was shown. There was diffuse subendocardial late gadolinium enhancement in the LV (Fig. 2). Prior coronary angiography (CAG) excluded the possibility of gadolinium enhancement to be caused by obstructive coronary artery disease (CAD). Because the patient also developed neurological symptoms, an MRI of the brain was performed. There were signs of small cortical petechial haemorrhages (Fig. 3).

Churg-Strauss syndrome (CSS) or eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disorder characterised by chronic rhinosinusitis, asthma and prominent peripheral blood eosinophilia [1-3]. The exact pathogenesis of CSS is unknown. Although a positive test for serial ANCA is obtained in 40 to 60 percent of patients with CSS, patients with cardiac involvement are less likely to have a positive ANCA [4]. The diagnosis requires a set of criteria composed by the American College of Rheumatology [5]. Comarmond et al. included 383 patients in a multicenter cohort study, to see what organs are most commonly affected in CSS. They found frequent involvement of the lungs, followed by skin, cardiovascular-, gastro-intestinal-, renal- and central nervous system [6]. During prodromal phase, asthma and allergic rhinosinusitis are main symptoms. The eosinophilic phase is characterised by peripheral blood eosinophilia and eosinophilic infiltration of several organs, causing multiple organ damage. In the last stage of CSS, the vasculitic phase, inflammation of medium and small vessels may lead to life-threatening situations. Clinical manifestation of cardiac disease in CSS includes signs of heart failure or pericarditis and cardiac rhythm abnormalities [4]. Endo- and myocardial damage in CSS usually occurs in three phases. During the acute necrotic phase, infiltration of eosinophils into myocardial tissue causes necrosis and formation of sterile microabscesses. Elevations in serum troponin levels can be sensitive indicators of early and ongoing eosinophil-associated myocardial damage [7]. Several reports have shown that contrast-enhanced CMR is able to reliably detect all stages of eosinophil-mediated heart damage in CSS [8, 9]. During the first stage, the hyperenhanced regions of the LV endocardium may be suggestive for Löffler endocarditis, specifically seen in patients with hypereosinophilia. The second phase of heart damage is characterised by the formation of thrombus [10]. During the third, fibrotic stage, scarring of myocardial tissue may lead to restrictive cardiomyopathy. CMR may show thickening of the LV wall or valve leaflet and a decrease in LV systolic and diastolic function. When neurological manifestation is present, peripheral nerves are most commonly affected. Peripheral neuropathy is seen in more than 75 percent of patients with CSS [11]. Although the central nervous system is rarely affected in CSS [12], some cases of subarachnoid haemorrhages have been reported [13,14]. Small and localised haemorrhages are thought to be caused by vasculitis involvement of medium and small vessels in de brain. The resulting subarachnoid petechial haemorrhages can be well visualised on MRI.

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