Page 280 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

254 Christian Pagnoux and Gerard P. Cox

manifestations or major organ involvement, can be managed with less toxic drugs such as
methotrexate. We do not have consensus on the clinical and laboratory monitoring of patients
with vasculitic and lung manifestations, but obtaining repeated chest radiographs and PFTs at
3- to 6-month intervals seems reasonable. Repeat CT should be reserved to resolve
uncertainties or to evaluate complications or new manifestations of disease. The intensity of
follow-up would vary, depending on the severity of the lung lesions and the impairment of
breathing parameters as well as the presence of subglottic and bronchial stenotic lesions.

Conclusion

Lung manifestations are common in several vasculitides and can present various features,
some of them potentially life-threatening. After exclusion of conditions mimicking vasculitis
and proof of the latter by tissue biopsy, treatment with systemic and local therapies can be
initiated with the expectation of improvement. Identification of effective and less toxic
therapies including plasma exchange for the most severely affected patients, those with
chronic lung complications such as anti-MPO-ANCA–associated pulmonary fibrosis, and
others with AAV will be clarified by emerging clinical trials.

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