The spectrum of pulmonary vasculitis

Wegener's granulomatosis and Churg-Strauss syndrome are the predominant pulmonary vasculitides. Next in frequency are the various diffuse alveolar haemorrhage syndromes, which may be related to the antineutrophil cytoplasmic autoantibody (ANCA)-associated diseases, such as Wegener's granulomatosis and Churg-Strauss syndrome, or may be a part of a collagen vascular disease, such as lupus erythematosus, or associated with antiglomerular basement membrane antibody (AGBM) and fall within the definition of Goodpasture's syndrome. Whereas Behçlet's disease and Takayasu's arteritis have major pulmonary manifestations, they are rare diseases. Entities previously confused with pulmonary vasculitis include lymphomatoid granulomatosis or polymorphic reticulosis, and benign lymphocytic angiitis and granulomatosis, which are probably in the spectrum of T-cell lymphomas. Necrotizing sarcoid and sarcoidosis can involve blood vessels, but do not follow a typical course associated with the traditional concept of vasculitis.