Pulmonary vasculitis
Updates to Article Attributes
Pulmonary vasculitis refers to vasculitides that affect the lung or pulmonary vessels. If this definition is used, a large group of conditions can fall into this category. The respiratory system may be potentially involved in all systemic vasculitides, although to a variable degree.
Pathology
When using the term "vasculitis" this implies that an inflammatory component is present within the pulmonary vessels.
Small vessel vasculitides
The group that classically forms the dominant pulmonary vasculitis tends to include the small vessel inflammatory processes that affect the pulmonary vessels:
- ANCA associated vasculitides
- idiopathic, pauci-immune pulmonary capillaritis: may or may not be ANCA associated
- non-ANCA associated pulmonary renal syndrome: Goodpasture syndrome 2
- rheumatoid pulmonary vasculitis 8: rare
- cryoglobulinaemic vasculitis (affecting the lungs)
Large vessel vasculitides
Large vessel vasculitides can also affect the lung and pulmonary vessels:
- Takayasu arteritis: 50-80% pulmonary involvement 1
- giant cell arteritis
- Behçet disease: 1-8% thoracic involvement 1
- Hugh Stovin syndrome
Granulomatous disease
In addition, certain granulomatous processes in the lung can also have an angiocentric component 3:
Classification
Various classification systems have been published including the popular Chapel Hill Consensus Conference classification system 7.
-<li><a href="/articles/microscopic-polyangiitis-mpa">microscopic polyangiitis</a></li>- +<li><a title="microscopic polyangiitis" href="/articles/microscopic-polyangiitis">microscopic polyangiitis</a></li>
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