Pulmonary Vasculitis: Spectrum of Imaging Appearances

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Key points

  • •

    Vasculitis can be broadly classified into small vessel, medium vessel, and large vessel vasculitis, depending on the size of the predominantly affected vessel.

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    Pulmonary involvement in vasculitis is uncommon, and is most commonly seen with small vessel vasculitis.

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    Granulomatosis with polyangiitis is the most common anti-neutrophil cytoplasm antibody–associated vasculitis and can manifest with lung and airway findings.

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Historical background and evolution of classification systems

Since the first classification system was proposed by Zeek9 in 1952, the nomenclature and classification of pulmonary vasculitis have undergone several revisions and modifications. Most of the early revisions were built on these existing criteria and introduced additional parameters, such as size of the vessel, histologic features, and whether the vasculitis was primary or secondary. These modifications were fueled by better understanding of the disease processes and their respective molecular

Pulmonary vasculitis

Pulmonary vasculitis is usually a facet of systemic vasculitis, except in the rare instances in which it presents as an isolated or single-organ vasculitis.25, 26, 27 The term “pulmonary vasculitis” can be used to describe inflammation of vessel walls (of any size) involving the lower respiratory tract; however, it commonly refers to vasculitides with increased involvement of the respiratory system, specifically the anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides. Lung

Diagnostic clues

Although there is no algorithm that can comprehensively allow the systematic classification of vasculitis, radiologists must be aware of clinicoradiologic clues that can help to narrow the differential diagnoses. A diagnostic approach using imaging manifestations correlated with laboratory and clinical findings is shown in Fig. 17.

Summary

The diagnosis of vasculitis is often challenging and is complicated by nonspecific and protean clinical findings. Laboratory tests can help to narrow the differential diagnoses, although these results show a degree of overlap with other vasculitides and with other disease processes. The unpredictable nature of these disorders, with their potential for flares and relapses, adds to the diagnostic conundrum. Chest radiography and CT and MR imaging can highlight specific findings in some instances

Acknowledgments

The authors acknowledge Megan Griffiths, scientific writer of Imaging Institute, Cleveland Clinic for her editorial assistance.

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    Disclosures: None.

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