The Heart in Wegener’s Granulomatosis

Abstract

Wegener’s granulomatosis (WG) is a necrotizing vasculitis that mainly affects the respiratory tract and kidneys. Of note, involvement of the heart is being increasingly recognized in these patients. Cardiac manifestations can arise from the coronary arteries, pericardium, myocardium, endocardium, valves, conduction system and great vessels, but in most cases cardiac involvement is clinically silent. Consequently, a regular cardiovascular evaluation, including echocardiography, should be performed in every patient with WG, while patients with WG flare should be closely monitored for cardiac complications even in the absence of specific symptoms.

Development of Multi-Organ Involvement Including a Left Atrial Myxoma-like Lesion in a Patient with Granulomatosis with Polyangiitis

“…Cases of a mass in the left ventricle have also been reported (10), and severe manifestations include congestive cardiomyopathy (11) and pericardial tamponade (12). Furthermore, valvular/endocardial diseases that occasionally mimic left atrial myxoma or bacterial endocarditis have been reported to be manifestations of GPA (13,14), similar to that observed in the present patient. Although the mass observed in this case was not assessed histologically, we assumed the presence of inflammatory cell infiltration with or without granulomas or myxomatous degeneration (13).…”

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

“…Pericardial pathology has demonstrated diffuse, as well as focal, areas of involvement. Pericardial effusions and cardiac tamponade have been reported [4,7]. The endocardium (including valves), myocardium, and epicardium are also targets of the vasculitic process.…”

“…The endocardium (including valves), myocardium, and epicardium are also targets of the vasculitic process. The epicardium has shown granulomatous inflammatory foci [4,7] and the myocardium has shown granulomatous foci, perivascular inflammation and necrotizing arteriolitis. The endocardium and valves were also involved in several cases with inflammation, fibrinoid necrosis, and granulomatous formation of the mitral and, less commonly, tricuspid valves [7,12].…”

“…Cardiac involvement in WG, including subclinical forms, is not as rare as previously considered, ranging between 6 and 44% of cases [4]. The high range is associated with more advanced disease and a higher rate of postmortem examinations [5].…”

Risk factors of atherosclerosis and clinical and morphological comparisons in systemic vasculitides

“…У пациентов с системными васкулитами причиной сердечно-сосудистых симптомов может быть специфическое поражение миокарда или сосудов сердца. У больных гранулематозом Вегенера описаны случаи ИМ, связанного с некротизирующим васкулитом коронарных артерий [22,23].…”