A New System For Prognostic Evaluation Of Patients With EGPA (Eosinophilic Granulomatosis With Polyangiitis) With Cardiac Involvement:Focus On The Heart

Background:Eosinophilic granulomatous polyangiitis is a systemic disease characterized by allergic rhinitis,asthma and a significant increasement of peripheral blood eosinophils.Its cardiac involvement has received widespread attention.With the improvement of risk stratification,standardization of glucocorticoid and immunosuppressive therapy,and production and application of biologicals,the prognosis of EGPA patients has been improved.However,cardiac involvement is still an important factor associated with poor prognosis.Objective:To compare the clinical features of cardiac involvementand,explore the role of cardiac involvement in the prognosis evaluation of EGPA,and refine the prognosis evaluation of EGPA with cardiac involvement.Methods:Patients with clinically diagnosed EGPA admitted to Peking Union Medical College Hospital between March 2012 and March 2023 were included in the study,as well as the EGPA cardiac involvement case reports retrieved from Pubmed(Chinese or English)up to March 2023.The information of the above patients was collected and followed up from the aspects of demography,clinical symptoms,organ involvement,laboratory examination,imaging examination,treatment and prognosis,and they were divided into two groups:cardiac involvement and without cardiac involvement.Endpoint event were divided into all-cause death,disease relapse,and composite end points(including death,instrument support/implantation,and heart transplantation).Results:A total of 207 patients clinically diagnosed with EGPA were included in this study,including 132 patients with cardiac involvement and 75 patients without cardiac involvement.The positive rate of ANCA in patients with cardiac involvement was significantly lower than that in the group without cardiac involvement(25.2%vs.41.3%,p=0.019).Although LVEF did not decrease significantly on echocardiography,the myocardial strain could be seen to have reduced local myocardial function.In terms of prognosis,the cardiac involvement group had a worse overall prognosis,and the incidence of death and composite end points was higher than that of the non-cardiac involvement group,while there is no statistical difference in relapse between the two groups.Due to the lack of a prognostic evaluation system for the heart itself,and the currently recognized five-factor prognostic score is difficult to evaluate the prognosis of patients in the cardiac involvement subgroup after excluding cardiac factors,this paper explored a five-item evaluation system for the heart in the heart-affected subgroup,including:①EOS ≥7.69 × 109/L,②ANCA negative;③The elevation of one or more markers of cardiac injury exceeded the threshold(cTn1≥1.741ng/ml,CK ≥ 371U/L,NT-proBNP≥ 10338pg/ml);④ Malignant arrhythmia,⑤LVEF ≤ 55%;The more items met,the worse the prognosis.Conclusions:Cardiac involvement is an independent risk factor for death,instrument support/implantation,and heart transplantation in patients with EGPA,and is not associated with disease relapse.Cardiac involvement needs to be evaluated at the time of diagnosis.In patients with existing myocardial involvement,the prognosis can be comprehensively evaluated by five evaluation items,including ANCA,EOS,markers of cardiac injury(cTnI,CK,NT-proBNP),malignant arrhythmia,and LVEF.