A Clinicoradiologic Approach to Diagnosing Pulmonary Vasculitis (Slideshow)

The diagnosis and management of the primary vasculitides is often difficult given that the clinical features of each disease depend on site, degree of severity and other factors, and that many patients present with nonspecific symptoms. Pulmonary vasculitis describes vasculitides with increased involvement of the respiratory system and is usually a facet of systemic vasculitis.

Imaging techniques such as chest radiography, CT and MRI can often help a multidisciplinary team of experts move toward the correct diagnosis. When correlated with laboratory findings, rheumatology and pulmonology consults, imaging can be a vital tool in the diagnosis of pulmonary vasculitis.

The following images demonstrate the radiologic findings and diagnostic clues of several types of pulmonary vasculitis.

<p>A 38-year-old woman with Takayasu’s arteritis (TAK), a large vessel vasculitis involving large and medium pulmonary arteries in about 50 percent of cases. (A, B) TAK typically involves the aorta, seen here with contrast-enhanced CT of the chest. Note circumferential thickening of the walls of the descending thoracic and upper abdominal aorta (arrows). (C) Left pulmonary artery involvement in TAK, shown in CT image at level of the left atrial appendage. Occlusion of the lingular branch present (arrow). (D) A coronal MR angiography image in the same patient demonstrates a perfusion defect in the lingual secondary to lingular branch occlusion (arrows).</p>

<p>Giant cell arteritis (GCA) is a large or medium vessel vasculitis predominantly involving the aorta and its branches, much like TKA. Histological and CT and MR imaging findings are identical to those of TKA. Respiratory involvement has been reported in approximately 10 percent of patients. These images show a 70-year-old man with GCA. (A) Notice the circumferential thickening of the descending and ascending thoracic aorta (arrows) on these axial T2 short-tau inversion recover (STIR) images. (B) Sagittal T2 STIR imaging demonstrates thickening of the descending thoracic aorta (arrow) and aortic arch branch vessels (broken arrow). (C) Coronal MR angiography image shows narrowing of the aortic branch vessels. Specifically, both subclavian and axillary arteries are diffusely involved, left greater than right (arrows).</p>

<p>Granulomatosis with polyangiitis is the most common antineutrophil cytoplasm antibody-associated (ANCA) vasculitis. The classic triad upon clinical presentation includes upper airway disease, glomerulonephritis and lower respiratory tract involvement. Unlike other vasculitides, chest radiography of GPA can show abnormal findings such as cavitary and noncavitary pulmonary nodules. In this 20-year-old woman with GPA, (A) long segment stenosis of the bronchus intermedius (arrowheads) and moderate stenosis of the left mainstem bronchus (arrows) are revealed with coronal multiplanar reformatted CT imaging. This is one of the primary ways lung involvement in systemic vasculitis presents. (B) A flexible bronchoscopy further shows the severe stenosis of the bronchus intermedius (arrowhead). Origin of left mainstem bronchus is also shown (arrow). (C) After stenting, improved patency in the bronchus intermedius (arrowhead) and left mainstem bronchus (arrow) (coronal multiplanar reformatted CT image).</p>

<p>Eosinophilic granulomatosis with polyangiitis (EGPA) involves a triad of asthma, necrotizing vasculitis and eosinophilia. Lung involvement is EGPA’s characteristic feature, and almost all patients have asthma. Over 72 percent of EGPA cases show abnormal chest radiograph findings like nonsegmental opacities, diffuse linear opacities and patchy transient consolidation. (A) Axial CT image shows diffuse bronchial wall thickening (arrows) in a 54-year-old patient with EGPA. (B) Three months later, this patient’s CT image shows a new peribronchial nodular opacity (arrow), typical of EGPA. (C) T2 STIR short-axis cardiac MRI suggests myocardial inflammation (arrow shows increased signal in the distal anterior wall). (D) Corresponding phase-sensitive inversion recovery (PSIR) delayed enhancement MR short-axis image showing midmyocardial enhancement in the apical anterior and inferior segments consistent with myocarditis (arrows).</p>

<p>Behçet disease is a variable vessel vasculitis characterized by recurrent oral and/or genital aphthous ulcers. Associated, inflammatory lesions involve the skin and the gastrointestinal, ocular, articular and/or central nervous system. More than 50 percent of patients show hemorrhage or infarction as a wedge-shaped airspace consolidation on chest radiographs. In this 25-year-old man, (A) steady-state free precession (SSFP) image shows an aneurysm of the left lower lobar pulmonary artery (arrow) with associated wall thickening. Beçhet disease is the most common cause of pulmonary artery aneurysm. (B) SSFP image at a higher level shows a pulmonary embolism in the left pulmonary artery (arrow).</p>

<p>A 51-year-old man with history of cocaine abuse presented to the emergency department with hemoptysis. Crack cocaine use can cause pulmonary capillaritis resulting in diffuse alveolar hemorrhage, one of the three major pathologic mechanisms through which lung involvement in systemic vasculitis presents. (A) Chest radiograph and (B) CT images show diffuse consolidation and groundglass opacities (arrows) in both lungs, compatible with DAH. Other drugs such as propylthiouracil, gemcitabine, diphenylhydantoin and transretinoic acid also cause pulmonary capillaritis.</p>

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Images and some captions are republished with permission from an article published in Radiologic Clinics of North America. Authors include Shamseldeen Mahmoud, MD, a nuclear radiology fellow; Carol Farver, MD, Director of Pulmonary Pathology; and Joseph Azok, MD, Subha Ghosh, MD, Jason Lempel, MD, and Rahul Renapurkar, MD, all staff in the Imaging Institute.