Introduction. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss syndrome, is a rare vasculitis of small-medium blood vessels. We report a case of a young woman with severe cardiovascular, neurological and respiratory problems caused by the syndrome.
Case report. A 45-year-old woman, affected by bronchial asthma and nasal polyposis, experiencing dyspnea and paresthesia on the left lower limb, underwent a total body PET-CT which demonstrated left segmental pulmonary thromboembolism, lung pseudo-nodular infiltration, and pericardial and pleural effusions. Thus, she entered Emergency Department. Hepatomegaly, left lower limb hot and edematous were observed. The ECG revealed low QRS voltages in the peripheral leads and poor r-wave progression in the precordial leads. The trans-thoracic echocardiography showed dilatated and hypokinetic right (RV) and left ventricle (LV) with severe reduction of global systolic function (LV ejection fraction, LVEF = 20%), bi-atrial dilation, circumferential pericardial effusion and thrombotic stratification at the apex. Blood test examination displayed severe eosinophilia, raised NT-Pro BNP and troponin I levels. A heart/coronary CT was performed, documenting epicardial coronaries free from significant atheromatous pathology. In addition, a cardiac MRI showed late gadolinium enhancement in the sub-endocardial area with diffuse involvement of the mid-apical ventricular segments and intraventricular septum, as in fibrotic outcomes. The subsequent total body CT scan confirmed the known thromboembolism, pulmonary lesions and pleuro-pericardial effusion; cardiogenic liver cirrhosis, and thrombosis of the right uterine vein and left gonadal vein were also reported. The auto-antibodies blood testing showed negativity for ANCA. In the presence of bronchial asthma, nasal polyposis, motoneuritis and hypereosinophilia, we diagnosed ANCA-negative eosinophilic vasculitis with cardiac involvement. Genetic testing was negative for FIP1L1-PDGFRA, excluding treatment with imatinib. Thus, we started immunosuppression therapy. However, albeit optimal medical treatment for ventricular dysfunction, the implantation of a subcutaneous cardiac defibrillator was required.
Conclusion. The possible cardiac manifestations in the context of EGPA may be several. Therefore, a prompt diagnosis results in a better prognosis for the patients.
