Abstract

Granulomatosis With Polyangiitis (Wegener's Granulomatosis) Complicated by Pericarditis: Our Experience of Two Cases and Comparative Review of Literature

Highlights

  • Granulomatosis with polyangiitis (GPA; formerly known as Wegener’s granulomatosis) is a disease characterized by necrotizing granulomatous vasculitis involving the upper and lower respiratory tracts and the kidneys.[1]

  • In the setting of classic chest pain, elevated inflammatory markers, and moderate pericardial effusion, the patient was diagnosed with GPA relapse manifesting as acute pericarditis with moderate pericardial effusion with only mild respiratory variation of Doppler inflows (Figure 3A–C; Video 2 available at www.onlinejase.com)

  • In the case of patient 2, the diagnosis of acute pericarditis was evident on presentation; the cardiac magnetic resonance imaging (MRI) evaluation illustrating the findings associated with acute inflammatory pericarditis aided with the treatment response

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Summary

INTRODUCTION

Granulomatosis with polyangiitis (GPA; formerly known as Wegener’s granulomatosis) is a disease characterized by necrotizing granulomatous vasculitis involving the upper and lower respiratory tracts and the kidneys.[1]. In the setting of classic chest pain, elevated inflammatory markers, and moderate pericardial effusion, the patient was diagnosed with GPA relapse manifesting as acute pericarditis with moderate pericardial effusion with only mild respiratory variation of Doppler inflows (Figure 3A–C; Video 2 available at www.onlinejase.com). Transthoracic echocardiogram showed no pericardial effusion or signs of constrictive physiology (Figure 4A and B; Videos 4 and 5 available at www.onlinejase.com). A cardiac MRI was performed due to persistent symptoms and poor response to medical therapy It revealed increased pericardial thickening of 2-3 mm with moderate circumferential enhancement of the pericardium on late gadolinium enhancement sequences, increased signal on T2 edema weighted imaging consistent with active pericarditis. The patient’s GPA is currently managed on low-dose prednisone and rituximab infusions with no recurrence of Figure 5 Chest CT scan axial view showing moderate-to-large pericardial effusion (yellow arrows). Pericarditis (Figure 7A and B; Videos 6–8 available at www. onlinejase.com)

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