Distinct histopathology of acute onset or abrupt exacerbation of hypersensitivity pneumonitis |
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Authors: | Hariri Lida P Mino-Kenudson Mari Shea Barry Digumarthy Subba Onozato Maristela Yagi Yukako Fraire Armando E Matsubara Osamu Mark Eugene J |
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Affiliation: | a Department of Pathology, Massachusetts General Hospital, Boston, MA 02114, USAb Pulmonary and Critical Care Unit, Massachusetts General Hospital, Boston, MA 02114, USAc Department of Radiology, Massachusetts General Hospital, Boston, MA 02114, USAd Department of Pathology, University of Massachusetts Medical Center, Worcester, MA 01605e Department of Pathology, National Defense Medical College, Tokorozawa, Japan |
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Abstract: | Hypersensitivity pneumonitis is an inflammatory lung disease that develops in response to exposure to antigen. Cases can be stratified by the duration of exposure and speed of symptom progression into acute, subacute, and chronic hypersensitivity pneumonitis. Although the pathologic features of subacute hypersensitivity pneumonitis are well established and those of chronic hypersensitivity pneumonitis have been reported, little is known about the histopathology of acute hypersensitivity pneumonitis. We evaluated the pathologic features of 5 patients with clinically confirmed hypersensitivity pneumonitis and rapid onset of symptoms and 3 patients with subacute or chronic hypersensitivity pneumonitis with symptom exacerbation. Histopathologic features assessed in each case included those characteristic of subacute hypersensitivity pneumonitis (bronchiolocentric chronic inflammation, histiocytic aggregates, and bronchiolitis obliterans), those associated with acute inflammation (fibrin deposition and neutrophilic infiltrate), and fibrosis. The classic features of hypersensitivity pneumonitis were identified in all 8 cases, with 1 also exhibiting fixed fibrosis confirming underlying chronic hypersensitivity pneumonitis. Fibrin deposition was present in 8 (100%) of 8 cases, and its extent was significant (28% surface area fibrin deposition/total disease area on average). Two had intra-alveolar fibrin so marked that it resembled acute fibrinous and organizing pneumonia. In addition, prominent interstitial neutrophilic infiltrate (≥5 cells/high-power field) was seen in all cases. These features have not been reported as characteristics of subacute or chronic hypersensitivity pneumonitis. Increased fibrin deposition and neutrophilic infiltrate may characterize acute hypersensitivity pneumonitis or abrupt exacerbation of hypersensitivity pneumonitis, and these along with characteristic features of subacute hypersensitivity pneumonitis (granulomatous inflammation and bronchiolocentricity) are sufficient to establish a morphologic diagnosis, particularly in conjunction with clinicoradiologic features. |
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Keywords: | Hypersensitivity pneumonitis Extrinsic allergic alveolitis Acute Interstitial lung disease Acute fibrinous and organizing pneumonia Fibrin |
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