Update in the diagnosis and management of pulmonary vasculitis.
The term vasculitis encompasses a number of distinct clinicopathologic disease entities, each of which is characterized pathologically by cellular inflammation and destruction of the blood vessel wall, and clinically by the types and locations of the affected vessels. While multiple classification schemes have been proposed to categorize and simplify the approach to these diseases, ultimately their diagnosis rests on the identification of particular patterns of clinical, radiologic, laboratory, and pathologic features. While lung involvement is most commonly seen with the primary idiopathic, small-vessel or antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides of Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome, one should remember that medium-vessel vasculitis (ie, classic polyarteritis nodosa), large-vessel vasculitis (ie, Takayasu arteritis), primary immune complex-mediated vasculitis (ie, Goodpasture syndrome), and secondary vasculitis (ie, systemic lupus erythematosus) can all affect the lung. However, for the purpose of this review, we will focus on the ANCA-associated vasculitides.
Frankel
Stephen K
SK
Interstitial Lung Disease Program, Department of Medicine, National Jewish Medical and Research Center, Denver, CO.
Cosgrove
Gregory P
GP
Interstitial Lung Disease Program, Department of Medicine, National Jewish Medical and Research Center, Denver, CO.
Fischer
Aryeh
A
Division of Rheumatology, Department of Medicine, National Jewish Medical and Research Center, Denver, CO.
Meehan
Richard T
RT
Division of Rheumatology, Department of Medicine, National Jewish Medical and Research Center, Denver, CO.
Brown
Kevin K
KK
Interstitial Lung Disease Program, Department of Medicine, National Jewish Medical and Research Center, Denver, CO. Electronic address: brownk@njc.org.
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D015267:Churg-Strauss Syndrome / Q000175:diagnosis / Q000628:therapy; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis* / Q000628:therapy*; D014657:Vasculitis / Q000175:diagnosis* / Q000628:therapy*
D019268:Antibodies, Antineutrophil Cytoplasmic