Page 276 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

250 Christian Pagnoux and Gerard P. Cox

Takayasu Arteritis

TAK can involve the pulmonary arteries, although less frequently than the aorta or other
arteries with stenoses, with or without dilations (Figure 7). The involvement can be associated
pulmonary hypertension, lung infarctions, arterial rupture and hemorrhage, regional
hypoperfusion, and lung ventilation and perfusion mismatches suggestive of pulmonary
embolism. There may be lung nodules, parenchymal consolidation, subpleural reticulo-linear
changes, pleural effusion and thickening, pulmonary-renal syndrome, and ILD [48-55].

Medium Vessel Vasculitides

Pulmonary artery and less often parenchymal involvement can occur in MVV.

Polyarteritis Nodosa

The lungs proper are not affected in PAN, and the occurrence of capillaritis with alveolar
hemorrhage should raise doubt about the diagnosis of PAN.

Prior to the 1994 CHCC [56], patients presenting purpura, hemoptysis, and pulmonary
hemorrhage that preceded the explosive phase of systemic necrotizing vasculitis affecting the
kidney and lungs with rapidly progressive glomerulonephritis and pulmonary capillaritis were
considered to have a microscopic form of PAN. This condition was later considered a
separate category of SVV, termed MPA [57]. Pulmonary edema and pleural effusion in PAN
are usually due to renal and/or cardiac insufficiency.

Patients with PAN can present interstitial pneumonia, lung nodules and cavitary lesions.
Although clinical and angiographic bronchial artery involvement is considered uncommon,
seven of ten patients were found to have bronchial arteritis on post-mortem examination
[58-60], so this manifestation of vasculitis may be unrecognized.

Kawasaki Disease

Children with Kawasaki disease (KD) show respiratory symptoms due to upper-
respiratory tract inflammation. Affected children present pneumonia, and up to 15% show
lung consolidation on chest radiography within 10 days of illness onset. Other radiographic
abnormalities include peribronchial cuffing, pleural effusion, atelectasis and air trapping [61].
The incidence of coronary artery lesions is greater in patients with abnormal chest
radiographs. Interstitial micronodular infiltrates and larger inflammatory pulmonary nodules
can occur [62-65].

Complimentary Contributor Copy