Silent Ischemic Heart Disease in a Patient with Necrotizing Glomerulonephritis due to Wegener’s Granulomatosis

Abstract

Objective: Wegener’s granulomatosis (WG) is a necrotizing vasculitis that mainly affects the respiratory tract and kidneys, but can also affect other systems such as the eye, joints, skin, muscles, nerves, and gastrointestinal tract. Cardiac involvement is traditionally believed to be rare. We report a patient with silent myocardial infarction (MI) and review previously reported cases showing this association. Methods: A Medline database search of cases published between January 1978 and July 2008 both in English and Spanish, reporting silent MI complicating WG, was conducted. Results: We describe a typical patient with WG who had both respiratory and renal involvement and died unexpectedly following a silent MI after a period of clinical improvement induced by treatment with prednisone and cyclophosphamide. We report necropsy findings and the association with 5 additional cases of WG with silent MI reported in the literature. Conclusions: Clinicians should be aware of potential cardiac involvement due to WG. Careful evaluation of each patient, with or without cardiac symptoms, using ECG, echocardiogram, and myocardial enzymes is prudent.

Aortic Valve Involvement in a Patient With Granulomatosis With Polyangiitis

“…1 Cardiac involvement in GPA can be in the form of silent ischemia in the majority of patients; while in other patients, it may present as conduction abnormalities, pericarditis, granulomatous myocarditis, valvulitis, endocarditis, coronary arteritis, aneurysms, and myocardial infarction. [2][3][4] Our patient presented with predominantly renal and pulmonary involvement.…”

“…9 Cardiac involvement in GPA is seen less commonly, although some reports suggest that cardiac involvement is being recognized more often now. 1,10 This may be thanks to a better understanding of the pathophysiology of GPA and increased awareness among physicians that silent cardiac involvement may occur in these patients. The ischemia in these patients may be due to the involvement of the small vessels secondary to the vasculitic process rather than the atherosclerosis as it responds to immunosuppressive therapy with reversal of ischemic changes.…”

“…1 Cardiac involvement has been reported in GPA in the form of conduction abnormalities, pericarditis, myocarditis, valvulitis, coronary vasculitis, and myocardial infarction. 2,3 Valvular involvement in GPA has been described in the form of valvulitis leading predominantly to regurgitant lesions and sometimes to stenotic lesions.…”

Cardiac manifestations in antineutrophil cytoplasmic autoantibody (ANCA) — associated vasculitides

“…The ischaemia in GPA patients may be due to the involvement of the small vessels secondary to the vasculitic process rather than the atherosclerosis as it responds to immunosuppressive therapy with reversal of ischaemic changes [24]. There are several cases that described patients with GPA and myocardial infarction (MI) [25][26][27][28]. In the Danish National Hospital register patients had an increased rate of MI within five years of diagnosis of GPA when compared to the general population [29].…”

“…In some cases prompt treatment with cyclophosphamide in the acute phase of the disease is very successful and provides resolution of cardiac complications [41]. However, there is also a case of a patient who died due to silent MI even after improvement of pulmonary and renal function [28]. Shanahan et al [42] described a patient treated with cyclophosphamide, who developed cardiomyopathy with reduced left ventricular ejection fraction (LVEF) to 40%.…”