Page 268 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

242 Christian Pagnoux and Gerard P. Cox

or, less commonly, in isolation, which implies diagnostic challenges. Treatment should be
adapted to the type and severity of the underlying vasculitic process and may combine
systemic and local therapies. Lung infection, pulmonary edema due to cardiac failure or renal
failure, cancer, and drug-induced lung toxicity can occur in patients with vasculitis.

This chapter focuses on the lung manifestations of systemic vasculitides, as defined by
the 2012 Revised Chapel Hill Consensus Conference (CHCC) [1], and prophylactic measures
to limit the risk of secondary complications.

Main Lung Manifestations in Systemic Vasculitis

A broad spectrum of lung manifestations may occur with systemic vasculitis and include
alveolar haemorrhage, lung nodules, interstitial disease, airway stenoses, fibrosis, pleural
effusion and pneumothorax. All of these may occur in small-vessel (SVV) antineutrophil
cytoplasm antibody (ANCA)-associated vasculitides (AAV) (granulomatosis with
polyangiitis [GPA], eosinophilic granulomatosis with polyangiitis [EGPA] and microscopic
polyangiitis [MPA]). Lung vessel involvement manifests as pulmonary artery aneurysms,
thrombosis and stenoses in the variable vessel vasculitis (VVV) Behçet disease (BD) and the
large-vessel vasculitis (LVV) Takayasu arteritis (TAK). The risk of pulmonary embolism
increases during disease flares in AAV.

Airway Disease

Nasal, sinus, mouth, throat and the upper airways are commonly sites in the respiratory
tract that may be involved in GPA leading to chronic erosive sinusitis, crusting rhinitis, nasal
septum perforation and subglottic stenosis. Allergic rhinitis, nasal polyposis, lower large- and
small-airway disease are manifestations associated with EGPA. Tracheo-bronchial stenosis is
more characteristic of GPA but can also occur in EGPA.

Tracheo-Bronchial Stenosis
Tracheal and bronchial involvement occurs in up to 70% of patients with GPA. Stenoses

of the main bronchus and its first branches are observed in less than 10% of patients [2-5].
Subglottic stenosis is the classic airway lesion and occurs in 7% to 15% of patients with GPA
[6-8]. Like subglottic stenosis, tracheal and bronchial stenoses cause dysphonia and dyspnea,
with or without stridor and wheezing. Large airway obstruction occasionally requires
emergency dilatation, often combined with local injections of corticosteroids, or
tracheostomy.

Complete bronchial occlusion can cause partial or complete collapse of the lung distally.
Endobronchial lesions can cause hemoptysis in cases of ulceration and erosion of an
underlying vessel. Such tracheal and bronchial lesions are best studied with a combination of
chest computed tomography (CT) (Figure 1) and direct visualization with fiberoptic
bronchoscopy because of possible multifocal involvement with strictures and granulomatous
endobronchial lesions. These airway lesions can parallel extra-respiratory manifestations of
GPA or may progress despite adequate control of the systemic vasculitis.

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