Page 279 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Systemic Vasculitis and the Lung 253

prognosis, with mortality exceeding 25% after 7 years of follow-up. Aneurysms can rupture,
causing catastrophic hemorrhage and leading to thrombosis with later pulmonary
hypertension and lung infarction. Most patients have concomitant extra-pulmonary venous
thrombi and thrombophlebitis. A pulmonary aneurysm justifies cyclophosphamide and high-
dose corticosteroids, although a controlled trial has yet to assess their efficacy and consensus
is lacking on the role of anticoagulant and antiplatelet therapies. Surgery, embolization, and
endovascular procedures should be considered in combination with systemic therapy because
of the complications associated with active vasculitis. Atelectasis, nodular, or reticular
opacities and cavities can occur, corresponding to foci of pulmonary hemorrhage, infarction,
and diffuse alveolar hemorrhage due to SVV. Secondary organizing pneumonia, pleural
nodules or effusions resulting from vasculitis of the pleura or thrombosis of the superior vena
cava, ulcerative lesions of the trachea and/or proximal bronchia as well as mmediastinal
lymphadenopathies can also occur. Pulmonary embolism in BD is rare and most often due to
pulmonary artery thrombosis rather than embolic peripheral venous thrombosis.

Other Vasculitides

Lung manifestations occur in patients with lupus vasculitis, RAV, sarcoidosis, systemic
scleroderma, HCV infection, and illicit use of substances such as cocaine (80-84). Alveolar
hemorrhage can be due to capillaritis in SLE similar to that observed in MPA. Different
pathogenic mechanisms may be involved in the development of ILD and lung nodules in
RAV. Cocaine and Levamisole-tainted cocaine induce lung complications including a GPA-
like syndrome with skin, nasal sinus involvement and lung-nodule rather than alveolar
hemorrhage. ANCA-seropositivity can be present as well as ELISA findings of specificity to
PR3 and various patterns on indirect immunofluorescence [85, 86].

Treatment and Prognosis

Before initiating therapy for pulmonary manifestations, the treating physician should be
certain that other conditions, especially infections, cancers, and drug toxicity, are not
mimicking pulmonary vasculitis. These conditions may co-exist with active vasculitis, such
as supra- or opportunistic infection of a lung cavity with Mycobacteria and Aspergillus
species or cancer leading to paraneoplastic pulmonary disease [87, 88]. Treatment should be
individualized to the vasculitis identified, its severity and the type of organ involved.
Although lung manifestations in AAV do not necessarily confer poorer prognosis, alveolar
hemorrhage and tracheo-bronchial stenoses warrant aggressive management to avert excess
morbidity and mortality. The ongoing plasma exchange in vasculitis (PEXIVAS) study [89]
may help clarify treatment options including plasma exchange in severe diffuse alveolar
hemorrhage. Systemic treatment is disappointing in subglottic and bronchial stenoses because
of the fibrotic and atrophic component of the lesions. Endoscopic dilations with bougies,
balloons and repeated local injection of corticosteroids with or without mitomycin application
may be effective. Tracheal stents and surgery should be considered in refractory patients.
Patients with GPA and only some lung nodules, without any other threatening systemic

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