 Hello everyone. Welcome back to our channel IndianRadiologists.com. Today I am here with an interesting case that I would like to discuss with you. I recently had the opportunity to present this case at the 2022 annual conference of the North American Society of Cardiovascular Imaging last month. A 32-year-old female presented to the emergency department with acute onset dyspnea and productive cuff. Her vitals included a rapid heart rate and respiratory rate with a pulse oximetry of 85%. She was started on BIPAP and electively intubated to help with the work of breathing. Her recent past history included outpatient treatment with prednisone for known seropositive rheumatoid arthritis. She also had known asthma and remote sinus surgery. Her rheumatoid arthritis was initially diagnosed in 2012 with a strong positive anti-CCP and negative rheumatoid factor. She had a sinus surgery in 2016, was diagnosed with asthma in 2018 and was being treated with steroids and long-acting beta-agonist inhaler. She also had an episode of angioedema in 2020. Recently, she suffered from an on-and-off productive cuff resolving with antibiotics but recurring. Her family history was unremarkable. On the current presentation, serial chest radiographs revealed fleeting opacities that rapidly progressed within a duration of five days. A seedy chest was obtained and revealed multifocal ground glass opacities in a crazy paving pattern and with a reversed halo sign. These findings can be characteristically seen in cryptogenic organising pneumonia or eosinophilic pneumonia. They can be commonly due to ARDS or bacterial pneumonia. They can also be seen with the broad differential of pneumocystis gerulisinimonia, edema, hemorrhage and pulmonary alveolar proteinosis. CT of the maxillary sinuses showed maxillary sinusitis. Patient was referred for a cardiac workup due to mildly reduced function on echocardiography. CT angiography was performed and showed normal coronaries. Right-heart catheterisation had normal findings. Patient was then referred for a cardiac MR. The cardiac MR was performed on a 1.5 tesla system. Cine cardiac MR showed global hypokinases with superimposed regional dyskinesis of the left ventricle and a mild mitral regurgitation. A native T1 map showing elevated native T1 values in the septum and the lateral wall. Late gadolinium enhancement cardiac MR showed extensive subendocardial and focal near transmural enhancement in the LV as well as the RV and the papillary muscles with the relative sparing of the apical segments. Blood work showed absolute eosinophilia. Rheumatology tests revealed the negative status for C-enka, P-enka and atypical enka. Infectious etiology workup was negative. Fish was negative for all these myeloproliferative diseases. Bronchoscopy and broncoalvular lavage had eosinophils for which the patient was started on steroids and anti-mass cell antibodies. Repeat bronchoscopy and endobronchial biopsies after intubation revealed necrotising eosinophilic pneumonia, a finding that is highly atypical for classic eosinophilic pneumonia or allergic bronchopulmonary aspergillosis. Endomyocardial biopsy from the RV free wall was negative for eosinophils. A note regarding endomyocardial biopsy, it is known that the yield is generally low for this indication often cited around 25%, which may increase to about 50% with voltage guidance. Voltage guided biopsy was discussed in the station but not pursued because the biopsy of the RV free wall carries a risk of perforation. Later, patient developed pulmonary thromboembolism in the right low bar segmental and subsegmental pulmonary arteries, as well as multiple bilateral lower extremity deep venous thrombosis. A constellation of all these symptoms were most concerning for hyper eosinophilic syndrome versus Anka negative eosinophilic granulometasis with polyangitis. The patient was started on high-dose steroids, empiric antibiotics and one dose of interleukin-5 inhibitor. Following this treatment five days later, a follow-up CT scan showed improvement in the ground glass opacities. Coming to the discussion, patients with hyper eosinophilia raises the possibility of following etiologies. It could be an idiopathic hyper eosinophilic syndrome or related to primary hyper eosinophilic syndrome, such as myeloproliferative disorders or T lymphocyte variants. Secondary hyper eosinophilic syndrome related to medications or parasites. Eosinophilic granulometasis with polyangitis or vasculitis and hyper eosinophilic syndrome of undetermined significance. In this scientific study, it was seen that patients with eosinophilic granulometasis and polyangitis had a history of asthma and fewer of these patients presented with arrhythmia than those with hypersensitivity. This pie chart shows the overall prevalence of eosinophilic granulometasis with polyangitis in patients with eosinophilic myocarditis as well as their prognosis in relation to freedom from death, hypertension and arrhythmia. To establish a diagnosis of eosinophilic granulometasis with polyangitis, the 1990 American College of Radiology criteria requires a positive biopsy for vasculitis and at least four of the six criteria below. In the prodomic allergic phase, asthma and rhinocenositis, eosinophilic phase, blood eosinophilia and tissue eosinophilia and a vasculitis phase that would show mono or polyneuropathy and migratory or transient pulmonary infiltrates. A previously published Lanham criteria roughly around 1984 required all the three features including asthma, peak peripheral eosinophilia more than 1500 and systemic vasculitis involving two or more extra pulmonary organs. With regards to the cardiac involvement, it is known that Anka-negative patients has a higher rate of cardiac involvement than the Anka-positive patients and the endomyocardial biopsies typically reveal eosinophilic infiltration and endomyocarditis but not vasculitis. The pathogenesis and pathophysiology of eosinophilic granulometasis with polyangitis is incompletely understood and is complex with an interplay of TH1 and TH2 activity. Asthma medications are known to enmask eosinophilic granulometasis with polyangitis and its manifestations. This is a great pictorial that explains the differences in organ involvement between Anka-positive and Anka-negative subtypes. Patients with Anka-negative status having a more of a cardiopulmonary involvement. The scientific paper shows that patients with EGPA have an Anka-negative status and would have a cardiomyopathy. Then the scientific paper shows that on cardiac MR, eosinophilic granulometasis with polyangitis has mostly a subendocardial diffused late gadolinium enhancement pattern and as we saw in our example apical sparing. The publication shows that the presence of cardiomyopathy is a poor prognostic indicator and a predictor of death in these patients in a univariable and a multivariable analysis. Coming to interval developments, our patient was presented at the multidisciplinary conference and after discussion between rheumatology, hematology and infectious diseases, a decision was taken to initiate cyclophosphamide. This treatment led to gradually improving symptoms and patient has been feeling well without any cardiac symptoms. A recent PET CT that was obtained 6 months post-treatment had no evidence of cardiac inflammation. To summarize in patients with eosinophilic granulometasis with polyangitis, asthma, rhinocinositis and peripheral eosinophilia can precede cardiac involvement by many years. Myocardial involvement is more likely to be noted in Anka-negative patients and cardiac MR have characteristic imaging features with a diffuse subendocardial late-catalanium enhancement with apical sparing that would help to completely avoid endomyocardial biopsies which have a particular low yield in this indication. These are some of the resources for further reading. Thank you so much for your attention.