The vasculitis syndromes are a heterogenous group of disorders characterized by inflammation and necrosis of blood vessels with a wide variety of clinical manifestations. The pathological process may affect the arteries, veins, and capillaries of any organ. The majority of primary vasculitis syndromes have no known etiological factors. Secondary vasculitis can be seen in collagenoses, immunological disorders, and infectious diseases caused by Aspergillus and Mucor species, hepatitis B and C, and HIV.

There is no satisfactory clinical classification of vasculitis syndromes. However, each of the well-known vasculitis syndromes exhibits certain clinical features that aid in the diagnosis. Even though any vasculitis syndrome can potentially affect any organ system, respiratory involvement is more evident or predominant in certain types. In this chapter we focus on respiratory manifestations in primary vasculitis syndromes.

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