CONGRESS ABSTRACT

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare small-vessel vasculitis. Cardiac involvement is present in 15 to 60% of cases. In 23% of these cases EKG shows no alterations Clinical Case: 59-year-old woman. History of childhood asthma and chronic sinusitis June 2024 onset of otalgia and pharyngodynia. Blood: WBC 11.7 109/dl with marked eosinophilia 32%; CRP 4.1 mg/dl (<0.5). Subsequently appearance of paresthesias in the limbs and petechiae in the legs. For suspected EGPA performs: Electromyography: sensory-motor polyneuropathy in the 4 limbs, predominantly axonal. Skin biopsy: extensive extravasation of red blood cells (purpura) mainly in the superficial dermis with dermo-epidermal detachment and with a blood-filled blister, with fibrin and neutrophil granulocytes, presence of karyorrhexis and little vascular involvement, where eosinophils are observed but without significant vascular aggression or formation of granulomas or fibrinoid necrosis; eosinophils are also observable in the interstitial area: finding compatible with EGPA Antibody panel: ANA weakly positive homogeneous pattern (AC-1), ENA negative, pANCA present (myeloperoxidase>134 – nv< 3.5 UI/ml) Chest/abdomen CT: minimal bilateral pulmonary consolidations, pericardial effusion 12 mm, suspected vasculitic involvement of the ileum and kidneys EKG: normal Echocardiogram: EF 45% with septal dyskinesia, moderate MR, left atrial dilation, PAPs 41 + 5 mmHg, no pericardial effusion Cardiac MRI: bi-ventricular dysfunction, LVEF 40%, active myocardial inflammation in the lateral wall, absence of areas of replacement fibrosis. Left atrial dilation. Pericardial effusion, loculated, max 1 cm, no evidence of thickening or altered signal of the parietal pericardium. Definitive diagnosis of EGPA with cardiac, neurological, cutaneous, nephrological involvement. Immunosuppressive therapy is set with cortisone and rituximab After therapy cutaneous, hematological and nephrological improvement. At echo EF 63% and at cardiac MRI: persistence of myocardial edema plus appearance of areas of LGE with associated hypo-akinesia of the involved myocardial segments, LVEF 50%. Improvement of pericardial effusion (maximum thickness at the level of the infero-lateral wall of 4 mm) Conclusions: Our case confirms that in EGPA, the absence of cardiac symptoms and a normal EKG are not decisive in excluding cardiac involvement and an echocardiogram must always be considered in the diagnostic framework