Correct!
3. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
The patient has a number of features consistent with eosinophilic granulomatosis with polyangiitis, formerly referred to as Churg-Strauss syndrome. She has a history of recurrent rhinitis and CT scan evidence of paranasal sinusitis, possibly a history of nasal polyposis, and a clinical diagnosis of severe asthma. She has clear evidence of a non-ischemic cardiomyopathy with a cardiac MRI pattern suggestive of myocarditis, and elevated perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) with myeloperoxidase specificity. Importantly she also has evidence of peripheral eosinophilia (greater than 10%) that appeared to be “unmasked” when her corticosteroid therapy was withdrawn, occurring in the context of fluctuating imaging opacities. While both chronic eosinophilic pneumonia and organizing pneumonia could result in the imaging opacities, and chronic eosinophilic pneumonia would be expected to produce the peripheral eosinophilia, neither chronic eosinophilic pneumonia nor organizing pneumonia would be expected to produce the myocarditis and left ventricular dysfunction noted at cardiac MRI. Some of this patient’s presentation and imaging findings could fit with a connective tissue disorder, but the balance of the information available favors the diagnosis of eosinophilic granulomatosis with polyangiitis, aka, Churg-Strauss syndrome.
Diagnosis: Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)
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