Distinctive pattern of fibrosis centered on membranous and respiratory bronchioles
Response to variety of environmental exposures or insults
Cluster 2 hypersensitivity pneumonitis (HP)
Gastroesophageal reflux disease (GERD)
Hiatal hernia
Systemic sclerosis in setting of common variable immune deficiency (CVID)
Adverse drug reaction
Inhalational injury (e.g., chemical fumes)
Chronic/recurrent infection
Smoking (< 50%); may contribute to airway injury
IMAGING
General Features
Radiographic Findings
CT Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Bois MC et al: Could prominent airway-centered fibroblast foci in lung biopsies predict underlying chronic microaspiration in idiopathic pulmonary fibrosis patients? Hum Pathol. 53:1-7, 2016
Kuranishi LT et al: Airway-centered interstitial fibrosis: etiology, clinical findings and prognosis. Respir Res. 16:55, 2015
Virk RK et al: Interstitial lung diseases that are difficult to classify: a review of bronchiolocentric interstitial lung disease. Arch Pathol Lab Med. 139(8):984-8, 2015
Allaix ME et al: Idiopathic pulmonary fibrosis and gastroesophageal reflux. Implications for treatment. J Gastrointest Surg. 18(1):100-4; discussion 104-5, 2014
Herbst JB et al: Hypersensitivity pneumonia: role of surgical lung biopsy. Arch Pathol Lab Med. 136(8):889-95, 2012
Myers JL: Hypersensitivity pneumonia: the role of lung biopsy in diagnosis and management. Mod Pathol. 25 Suppl 1:S58-67, 2012
Fenton ME et al: Hypersensitivity pneumonitis as a cause of airway-centered interstitial fibrosis. Ann Allergy Asthma Immunol. 99(5):465-6, 2007
Churg A et al: Airway-centered interstitial fibrosis: a distinct form of aggressive diffuse lung disease. Am J Surg Pathol. 28(1):62-8, 2004
Colombat M et al: Lung transplantation in a patient with airway-centered fibrosis. Am J Surg Pathol. 28(11):1540-2, 2004
Yousem SA et al: Idiopathic bronchiolocentric interstitial pneumonia. Mod Pathol. 15(11):1148-53, 2002
Distinctive pattern of fibrosis centered on membranous and respiratory bronchioles
Response to variety of environmental exposures or insults
Cluster 2 hypersensitivity pneumonitis (HP)
Gastroesophageal reflux disease (GERD)
Hiatal hernia
Systemic sclerosis in setting of common variable immune deficiency (CVID)
Adverse drug reaction
Inhalational injury (e.g., chemical fumes)
Chronic/recurrent infection
Smoking (< 50%); may contribute to airway injury
IMAGING
General Features
Radiographic Findings
CT Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Bois MC et al: Could prominent airway-centered fibroblast foci in lung biopsies predict underlying chronic microaspiration in idiopathic pulmonary fibrosis patients? Hum Pathol. 53:1-7, 2016
Kuranishi LT et al: Airway-centered interstitial fibrosis: etiology, clinical findings and prognosis. Respir Res. 16:55, 2015
Virk RK et al: Interstitial lung diseases that are difficult to classify: a review of bronchiolocentric interstitial lung disease. Arch Pathol Lab Med. 139(8):984-8, 2015
Allaix ME et al: Idiopathic pulmonary fibrosis and gastroesophageal reflux. Implications for treatment. J Gastrointest Surg. 18(1):100-4; discussion 104-5, 2014
Herbst JB et al: Hypersensitivity pneumonia: role of surgical lung biopsy. Arch Pathol Lab Med. 136(8):889-95, 2012
Myers JL: Hypersensitivity pneumonia: the role of lung biopsy in diagnosis and management. Mod Pathol. 25 Suppl 1:S58-67, 2012
Fenton ME et al: Hypersensitivity pneumonitis as a cause of airway-centered interstitial fibrosis. Ann Allergy Asthma Immunol. 99(5):465-6, 2007
Churg A et al: Airway-centered interstitial fibrosis: a distinct form of aggressive diffuse lung disease. Am J Surg Pathol. 28(1):62-8, 2004
Colombat M et al: Lung transplantation in a patient with airway-centered fibrosis. Am J Surg Pathol. 28(11):1540-2, 2004
Yousem SA et al: Idiopathic bronchiolocentric interstitial pneumonia. Mod Pathol. 15(11):1148-53, 2002
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