Other Pulmonary Diseases of the Pulmonary Vasculature

Wegener's granulomatosis is a vasculitis (inflammatory condition) of the vascular bed that can manifest either with shortness of breath or hemoptysis or with progressive pulmonary fibrosis caused by repeated small hemorrhages at the alveolar level. It is now categorized as a systemic antineutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitis, and it usually combines pulmonary features with glomeru-lonephritis. Tissue biopsy of either the lung or the kidney may be diagnostic. Clinical manifestations result from the combination of vasculitis, glomerulonephritis, and necrotiz-ing granulomas of the respiratory tract. CT may be more sensitive than MRI for diagnosis of pulmonary lesions.

Diffuse alveolar hemorrhage may result from autoimmune collagen vascular disease or vasculitis, Goodpasture's syndrome, and other vasculitides. Goodpasture's syndrome results from the formation of anti-glomerular basement membrane antibodies, which can also attack the lung capillary membranes. Primary pulmonary vasculitides affect mostly small vessels, but systemic conditions can affect vessels of all sizes. Churg-Strauss syndrome is a small-vessel vasculitis that often manifests first as asthma. Most patients also have maxillary sinusitis, allergic rhinitis, or nasal polyposis. Gastrointestinal, neurologic, and cardiac involvement often follows. The condition responds well to systemic steroids, but patients can require long-term low-dose prednisone as maintenance therapy (Guillevin et al., 2004).

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