Imaging Findings in Pulmonary Vasculitis

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Vasculitis is a destructive inflammatory process affecting blood vessels. Pulmonary vasculitis may develop secondary to other conditions or constitute a primary idiopathic disorder. Thoracic involvement is most common in primary idiopathic large-vessel vasculitides (Takayasu arteritis, giant cell arteritis, Behçet disease) and primary antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides (Wegener granulomatosis, microscopic polyangiitis, Churg–Strauss syndrome). Primary pulmonary vasculitides are rare, and their signs and symptoms are nonspecific, overlapping with those of infections, connective tissue diseases, and malignancies. The radiologic findings in primary pulmonary vasculitis vary widely and can include vessel wall thickening, nodular or cavitary lesions, ground-glass opacities, and consolidations, among others. Diffuse alveolar hemorrhage usually results from primary small-vessel vasculitis in the lungs. To diagnose vasculitis, medical teams must recognize characteristic combinations of clinical, radiologic, laboratory, and histopathologic features.

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Classification

The vasculitides are disorders characterized by blood vessel inflammation leading to tissue or end-organ injury. The classification of vasculitis has been a challenging problem for decades. The size of the vessels predominantly involved strongly influences the clinical and radiologic features of the different forms of vasculitis and are therefore one of the major criterion for classification. In 1994, after the discovery of ANCA, the Chapel Hill Consensus Conference on the nomenclature of

Clinical and Radiologic Findings Suggestive of Vasculitis

Vasculitides are difficult to diagnose because their signs and symptoms overlap with those of infection, malignancy, thromboembolic disease, and connective tissue diseases.3, 4 Large-vessel vasculitis may present with signs and symptoms of ischemia. Small-vessel vasculitis often presents with nonspecific constitutional signs and symptoms, such as fever, myalgias, arthralgias, and malaise. Vasculitis should be suspected when an unusual constellation of signs and symptoms involving multiple organ

Large-Vessel Vasculitis

Large-vessel vasculitides predominantly affect the aorta and its largest branches. These vasculitides are suspected when there are signs and symptoms of ischemia. TA and GCA are the most common large-vessel vasculitides.12 We also include Behçet disease in this category because it may present similar findings in major arteries.

TA affects almost exclusively patients younger than 40 years, involves primarily the aorta and its major branches, and generally spares the cranial arteries. However,

Takayasu Arteritis

TA is an idiopathic chronic arteritis resulting in the stenosis of a large artery; it has a strong predilection for the aortic arch and its branches.13

Although the disease is more common in Asia, it is also found worldwide, usually affecting young women. The estimated annual incidence of TA is 0.12-0.26 cases/100.000.7

TA is characterized by granulomatous inflammation of the arterial wall with marked intimal proliferation and fibrosis of the media and adventitia, which eventually leads to

Giant Cell Arteritis

GCA (temporal arteritis) is the most common vasculitis of large- and medium-sized arteries, affecting almost exclusively individuals >50 years of age.20 In this age-group, the estimated prevalence of GCA is 278 per 100,000 persons in the United States and is even higher in northern Europe.21 GCA predominantly affects the extracranial carotid branches and the aorta and, rarely, the central pulmonary arteries. Histologically, GCA appears similar to TA.22

Until recently, GCA was believed to be a

Behçet Disease

Behçet disease is a chronic multisystemic vasculitis heralded by the clinical triad of recurrent oral and genital ulcers and uveitis. Behçet disease may also affect joints, the gastrointestinal system, central nervous system, cardiovascular system, and the lungs. Behçet disease usually presents in the second or third decade of life and the male-to-female ratio is almost equal.25 The reported prevalence of thoracic involvement in Behçet disease ranges from 1% to 8%.25 Vascular system involvement

Small-Vessel Vasculitis

Although by definition small-vessel vasculitis affects arterioles, venules, and capillaries, it may also affect arteries, thus overlapping with medium-sized and large-vessel vasculitides.11 Lung involvement is most common in primary idiopathic ANCA-associated small-vessel vasculitis: WG, CSS, and MPA. These vasculitides can affect people of all ages, but are most common in adults in their 50s and 60s; they affect men slightly more frequently than women.11

Wegener Granulomatosis

WG is the most common of the ANCA-associated vasculitides. The upper respiratory tract is involved in nearly all patients; in addition, the vast majority of patients with WG will also have pulmonary (90%) and renal (80%) involvement.30 The classic clinical triad of upper airway involvement (sinusitis, otitis, nasal mucosa ulcers, bone deformities, and subglottic stenosis), lower respiratory tract involvement (cough, chest pain, hemoptysis), and glomerulonephritis is often not present at initial

Churg–Strauss Syndrome

CSS is characterized by the clinical triad of asthma, hypereosinophilia, and necrotizing vasculitis.36 The diagnosis can be made if 4 or more of the following 6 findings are present: asthma, eosinophilia >10% on differential white blood cell count, mononeuropathy or polyneuropathy attributable to systemic vasculitis, migratory or transient pulmonary opacities, paranasal sinus abnormalities, and extravascular eosinophils on a biopsy specimen.37 Relatively late age of onset (mean 32 years)

Microscopic Polyangiitis

MPA is a nongranulomatous necrotizing systemic vasculitis. It is the most common cause of pulmonary-renal syndrome, a syndrome characterized by pulmonary hemorrhage and glomerulonephritis.43 The clinical manifestations are usually renal and, less commonly, pulmonary. More than 90% of patients present rapidly progressive glomerulonephritis, whereas 10%-30% present DAH;9, 22 in patients who develop lung disease, DAH with pathologic capillaritis is the most common manifestation.2 Chest symptoms

Diffuse Alveolar Hemorrhage

DAH is recognized by the clinical constellation of hemoptysis, anemia, diffuse radiographic pulmonary infiltrates, and hypoxemic respiratory failure.

However, chest radiographic and CT findings are nonspecific, and hemoptysis may be lacking.2, 22, 44 The diagnosis is usually made when serial bronchoalveolar lavage samples (from the same location) reveal an increasing red blood cell count.

In DAH, hemorrhagic filling of the airspaces causes widespread radiographic shadowing ranging in intensity

Diagnostic Approach of Pulmonary Vasculitis

The primary vasculitides are rare disorders manifesting with nonspecific signs and symptoms that overlap with those of infections, connective diseases, and malignancies, so an integrated clinical, radiologic, and sometimes histologic approach is needed to reach the correct diagnosis.

Signs and symptoms of ischemia should raise suspicion of large-vessel vasculitis (GCA or TA), which is strengthened by arterial wall thickening on imaging. Pulmonary artery aneurysms are usually caused by Behçet

Conclusions

The diagnosis of vasculitis is often delayed because other, more common, disorders can have the same clinical manifestations; therefore, it is essential to integrate clinical, laboratory, and imaging findings to make a specific diagnosis of pulmonary vasculitides.

Acknowledgment

The authors thank John Giba for linguistic aid.

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