An Unusual Presentation of Granulomatosis With Polyangiitis (Wegener's) After SARS-CoV-2 Infection

Abstract

In this article, we present an unusual case of granulomatosis with polyangiitis (GPA) in a 41-year-old man. The initial presentation of the disease was atypical, with persistent fever, cough, and fatigue, accompanied by elevated inflammatory markers in association with a large, solitary lung lesion observed at the chest X-ray. Despite the presence of an initial radiological picture suggesting pneumonia, the lack of response to antibiotics necessitated a more in-depth evaluation. The diagnosis was confirmed through a lung biopsy and serological tests positive for anti-neutrophil cytoplasmic antibodies (c-ANCA). GPA is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, a systemic autoimmune disease characterized by necrotizing granulomatous inflammation and pauci-immune small vessel vasculitis. This case posed diagnostic challenges due to the atypical presentation, initially mistaken for a respiratory tract infection versus cancer. However, the lack of improvement with antibiotics and persistent inflammation raised suspicions of an underlying complex condition. The diagnosis was confirmed through a lung biopsy and positive c-ANCA serological tests. The patient had reported a prior SARS-CoV-2 infection, raising questions about the possible connection between COVID-19 and GPA, as suggested by previous studies. The diagnostic workup ruled out common and rare pulmonary infections, autoimmune diseases, and neoplasms. However, the presence of positive c-ANCA antibodies was pivotal for the GPA diagnosis. Treatment involved the use of high-dose corticosteroids and rituximab to suppress the autoimmune response. Early diagnosis and timely treatment are essential for improving outcomes in patients with GPA.

Keywords: anca associated vasculitis; covid-19 related issues; differential diagnosi; granulomatosis with polyangiitis (gpa); pulmonary cavitary lesion; unusual case; wegener’s granulomatosis.

Figure 1. Chest X-ray front

A coarse area of parenchymal hypodensity with rounded margins measuring 13 x 11 cm, without an air bronchogram.

Figure 2. Chest X-Ray lateral

Same lesion, lateral view. The lesion demonstrates a greater extension on the posterior aspect, which comes into contact with the pleura.

Figure 3. Axial CT scan

Extensive parenchymal consolidation involving two-thirds of the right lower lobe without an air bronchogram.

Figure 4. Coronal CT scan

A lesion with heterogeneous density closely adheres to the subcostal pleura, causing compression and obliteration of the tributary bronchi.

Figure 5. Chest X-ray after biopsy

Chest X-ray for follow-up, performed after a CT-guided biopsy. The lesion shows an air-fluid level.