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You are here: Contents > 2017 > Volume 26 Number 1 January 2017 > CASE REPORTS > Granulomatosis with Polyangiitis Presenting with Acute Aortic and Mitral Regurgitation: Case Report and Big-Data Analysis

Granulomatosis with Polyangiitis Presenting with Acute Aortic and Mitral Regurgitation: Case Report and Big-Data Analysis

Sadeer G. Al-Kindi1, M. Amer Al-Aiti1, Michael Yang2, Richard A. Josephson1,3

1Harrington Heart and Vascular Institute, University Hospitals Cleveland Medical Center, Cleveland, Ohio, USA
2Department of Pathology, University Hospitals Cleveland Medical Center, Cleveland, Ohio, USA
3Electronic correspondence: richard.josephson@uhhospitals.org
Granulomatosis with polyangiitis (GPA) is a rare vasculitis that can have multisystem involvement, though cardiac involvement is very rare. The case is described of a 53-year-old woman who presented with acute aortic and mitral valve regurgitation requiring surgical intervention. Pathology from the excised aortic valve showed geographic necrosis concerning for GPA. Subsequent rheumatologic testing was positive for anti-serine proteinase 3 (PR3) antibody, consistent with GPA. A year after the valve surgery the patient was found to have a vegetation of the mitral valve and elevated PR3 antibody levels, and was successfully treated with an intensification of immunosuppression. The cardiac involvement of GPA is discussed and big data analyzed to identify the epidemiology of valvular involvement. In conclusion, GPA can have multiple different valvular manifestations with a high recurrence rate. GPA should be considered in patients who present with acute valvular disease without any clear precipitant.

The Journal of Heart Valve Disease 2017;26:103-106


Granulomatosis with Polyangiitis Presenting with Acute Aortic and Mitral Regurgitation: Case Report and Big-Data Analysis

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