Page 275 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Systemic Vasculitis and the Lung 249

syndrome [38]. Affected patients are typically men with a history of thrombophlebitis.
Pulmonary and bronchial artery aneurysms can rupture, thus leading to hemoptysis and
internal haemorrhage, or demonstrate progressive occlusion with thrombosis.

Figure 7. Main right pulmonary artery stenosis in a patient with Takayasu arteritis (CT-angiography,
frontal reconstruction and axial image)

Pulmonary Hypertension

There are anecdotal reports [39, 40] of pulmonary hypertension in patients with PAN and
AAV. Secondary pulmonary hypertension can result from constrictive pericarditis, restrictive
cardiomyopathy, and lung fibrosis [41].

Phrenic Palsy

Unilateral phrenic palsy due to mononeuritis multiplex can occur in GPA and EGPA [42,
43]. However, it can be related to pre-existing coincidental findings, presumably after a
trauma, or an unknown cause.

Large Vessel Vasculitides

Parenchymal lung manifestations occur so rarely in LVV that when present, should
prompt reconsideration of the diagnosis of vasculitis. However, involvement of pulmonary
arteries can be an associated feature of LVV, especially in TAK.

Giant Cell Arteritis

One-quarter of patients with GCA exhibit a dry non-productive cough. Less common
manifestations include alveolar hemorrhage, T-CD4+ lymphocytic alveolitis, pleural effusion
and thickening, multiple pulmonary infarctions, lung nodules and ILD [44-47].

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