Pulmonary vasculitis and pulmonary hemorrhage syndromes

Pathology

“…GPA is usually associated with cytoplasmic type ANCA (c-ANCA) in contrast to the other two ANCAassociated vasculitides. 211 Radiographic studies reveal multiple and bilateral lung masses that frequently cavitate and may be found in all lung zones but are most frequent in the lower lobes (see eFigs. 60-1 and 60-2).…”

“…The elastic layer shows abrupt disruption and fragmentation, and the arterial intima may contain abnormal fibrosis with infiltration of mononuclear cells. [211][212][213] Immunofluorescence studies for evaluation of antibody deposition are very weak or negative and are only useful to rule out other entities in the differential diagnosis, such as immune complex-mediated vasculitis. Electron microscopy studies are not indicated.…”

“…In this setting, immunofluorescence studies are useful to exclude other causes of diffuse alveolar hemorrhage such as Goodpasture syndrome, lupus pneumonitis, cryoglobulinemia, or other immune complex-mediated diseases. [211][212][213]216,217 …”