Granulomatosis With Polyangiitis Complicated by Diffuse Alveolar Hemorrhage and Mitral Valve Chordal Rupture: An Uncommon Cardiopulmonary Manifestation

Abstract

Granulomatosis with polyangiitis (GPA) is an anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis that typically affects the upper and lower respiratory tract and kidneys. Cardiac complications, particularly those involving the valvular or subvalvular structures, are rare. We present the case of a 59-year-old woman with known anti-proteinase 3 (PR3)-ANCA-positive GPA who developed a severe disease flare characterized by diffuse alveolar hemorrhage (DAH) and mitral valve insufficiency due to chordae tendineae rupture. The diagnosis was confirmed by high-resolution computed tomography demonstrating ground-glass opacities consistent with DAH, markedly elevated PR3-ANCA titers, and echocardiography showing severe mitral regurgitation with ruptured chordae. She was treated with high-dose corticosteroids, rituximab, and avacopan, which resulted in marked clinical and radiological improvements. This case highlights the need for increased awareness of rare GPA manifestations, importance of multidisciplinary evaluation, and potential benefits of early immunosuppressive therapy.

Keywords: anca-associated vasculitis; avacopan; cardiac involvement in vasculitis; diffuse alveolar hemorrhage; granulomatosis with polyangiitis (gpa); mitral valve chordal rupture; multisystem vasculitis; pr3-anca; rituximab (rtx); steroid-sparing therapy.

Figure 1. High-resolution computed tomography demonstrating right-sided ground-glass opacities (arrow) with subpleural sparing suggestive of DAH, a severe GPA complication with long standing total left lung collapse due to GPA-induced left main stem bronchus narrowing.

Ground-glass opacities (i.e., “hazy areas reflecting alveolar filling”). DAH – diffuse alveolar hemorrhage, GPA – granulomatosis with polyangiitis

Figure 2. ECG: Inferolateral T-wave inversion in leads V4-V6-aVF and leads I-III.

Figure 3. Transthoracic echocardiography: severe mitral valve regurgitation.

Figure 4. High-resolution computed tomography performed two weeks after initiation of high-dose steroids and rituximab, showing marked improvement in ground-glass changes in the right lung (arrow).