What is granulomatosis with polyangiitis (Wegener cardiomyopathy)?

Updated: Nov 28, 2018
  • Author: Vinh Q Nguyen, MD; Chief Editor: Gyanendra K Sharma, MD, FACC, FASE  more...
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Answer

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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