What is granulomatosis with polyangiitis (Wegener cardiomyopathy)?

Updated: Mar 02, 2021
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Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) is a small- to medium-sized vasculitis that affects the airways, lungs, kidneys, and heart. Cardiac manifestations include pericarditis, supraventricular tachycardias, and heart block. [60]  Myocardial involvement/systolic dysfunction has also been described, although not as commonly as the manifestations discussed above. [61, 62] Glucocorticoids and cyclophosphamide are the mainstay of therapy; however, keep in mind that cyclophosphamide itself may cause cardiomyopathy.

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