Teaching Case

• Female, 44 years old
• Smoker
• No previous pathologies in patient history
• No chronic therapy
• Not pregnant

First Rx scan shows hardly distinguishable areas of opacity with a central excavation in both of the apices of the lung. In the second Rx Scan, both apical opacities are easily distinguishable, and also central excavation is well-defined.

Enhanced CT scan of the head and neck shows an enlarged, inhomogeneous right parotid with hypodense region within the gland parenchyma of several enlarged right-sided peri-parotid and cervical lymph nodes are also present.

In maxillofacial CT scan, we can appreciate the maxillary sinus mucosa thickening; we can also notice a slight right-convex deviation of the nasal septum.

The Chest CT scan indicates cavitating lesions of the lung at both apices, further solid-nodular lesions with central cavitation are evident in the lung bases, and some small mediastinal lymph nodes are evident.

In the MRI scan the parotid gland is enlarged with inhomogeneous post-contrast enhancement and diffuse signal restriction on DWI.

Granulomatosis with polyangiitis (GPA), previously known as Wegener granulomatosis, is a vascular antineutrophil cytoplasmic antibody–associated disease that involves medium and small-sized vessels with evidence of vascular wall inflammatory infiltrations and granulomas.

GPA may involve any organ, but most frequently the upper airways, lungs and kidneys. On histology, the typical findings are granulomas with central necrosis and cavitation, which are usually found in the small artery wall, capillaries and venules of the airways and lung as in the renal glomeruli.

Clinical presentation is variable, and nose involvement is often the first sign in most people [1, 2].

Involvement of the upper respiratory tract is seen in nearly all people with GPA [3]. Upper respiratory tract involvement includes cough, fever, dyspnea, asthenia, chest pain, persistent rhinorrhea, epistaxis, hemoptysis, sinusitis, weight loss, arthralgia, otitis, arthritis, and mucosal ulceration.

Imaging findings in the parotid gland are nonspecific and consist of heterogeneous gland enlargement and hyper and heterogeneous enhancement after contrast injection; the evidence of high signal intensity on T2-weighted MR images, related to necrosis.

Imaging findings in nose and paranasal sinuses include bone involvement in paranasal sinuses, sphenoid bone, osseous erosions due to granulomas and mucosal thickening.

Lung is the organ most commonly involved, mainly bilaterally, with multiple pulmonary nodules and masses, from a few millimeters to 10 cm in diameter. In larger lung lesions, it is more common to find cavitation.

Peripheral hemorrhage around lung nodules appears on HRCT images as ground-glass opacity surrounding the nodule, which is known as the “halo” sign [4]. Consolidation and spots within ground glass opacities can be seen in perihilar areas and in the middle lower lung.

The final diagnosis was made by increased PCR value, c-ANCA antibody positivity and biopsy result.

The therapy consists in corticosteroids, immunosuppressants and cyclophosphamide. New therapies have been tested, such as methotrexate, etanercept and rituximab.

If no renal complications occur, 5 years after diagnosis, the current survival rate for patients is 100%, while at 10 years, it drops to 75-88%.

GPA disease can mimic different infectious diseases including tuberculosis. Is important to evaluate all these findings and consider autoimmune disease as alternative diagnosis when cavitary lesions are discovered. Manifestations are often identified at chest CT, and the radiologist may be the first to suggest GPA diagnosis. CT is the imaging modality of choice for diagnosis, surveillance, and follow-up in patients with GPA [5].

All patient data have been completely anonymised throughout the entire manuscript and related files.