Introduction: Eosinophilic granulomatosis with poly-angiitis, known as Churg-Strauss syndrome is a rare systemic vasculitis, almost invariably accompanied by asthma. Cardiac involvement has been reported in various forms (myocarditis, pericarditis, myocardial infarction due to coronary vasculitis, conduction abnormalities) and represents a poor prognosis criteria. Dilated cardiomiopathy secondary to the disease is however not commonly described. We present this case in order to emphasize the importance of multi-modality imaging in identifying the etiology of dilated cardiomiopathy.
Case presentation: We report the case of a 35-year-old man, without cardiovascular risk factors, admitted for dyspnea. Medical history reveals he had been diagno-sed with asthma and chronic rhinosinusitis. His symp-toms started a year before, when chest X-ray showed bilateral opacities. Lab results showed eosinophilia and high levels of inflammatory markers. To identify the etiology of the dyspnea associated with eosinophilia, tests for Toxocara, Echinococcus and Aspergillus were taken and were negative. cANCA and pANCA were also negative. Bronchoalveloar lavage was performed showing high number of eosinophils and no bacteri-al, fungal or tumoral elements. Bone marrow biopsy showed normal cellularity, but increased eosinophils. To exclude primary eosinophilia, genetic testing was performed, FIP1L1 and PDGFR A, B being negative. No other cause was found, therefore the clinico-bi-ological anomalies were considered to be in the con-text of asthma and corticotherapy (Prednison 7.5mg) was added to bronchodilatators. On admission in our clinic,the patient presented dyspnea on exertion. Phy-sical exam did not reveal anything remarkable, no signs of cardiac or pulmonary congestion being present. Lab tests showed high BNP (2065 pg/ml), but no other ano-malies, while chest X-ray revealed the absence of the opacities anteriorly described. Echocardiography reve-aled dilated cardiomiopathy with LV ejection fraction 20% and severe mitral reurgitation. Cardiac MRI was conducted revealing intramyocardial signal abnormali-ties and diffuse subendocardial fibrosis consistent with microvascular ischemia.Therefore, after corroboration of all the data (the presence of asthma being essential) eosinophilic granulomatosis with polyangiitis diagnosis took shape. Looking at the ACR criteria for EGPA we realize that 5out of 6 were fulfilled at the beginning of the symptoms: asthma; eosinophilia>10%; extra-vascular eosinophils, transient pulmonary infiltrates, chronic rhinosinusitis. The absence on admission of initial anomalies was due to corticotherapy.
Conclusions: Consequently, cardiac dysfunction was considered to be secondary to Churg-Strauss syndrome and treatment with Azathioprine (50mg/day) was ini-tiated. Also, treatment with ACE inhibitor beta blocker and diuretic was associated. After three months of me-dical therapy, we discover an improvement in the the patient’s effort capacity and an improved function of the left ventricle (LVEF 30%). In conclusion, this case is particular not only because of its rarity, but also due to the fact that the diagnosis was established in retrospec-tive, taking into consideration all the clinico-biological findings present at the beginning of the symptoms.