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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Airway-centered interstitial fibrosis (ACIF)
Synonyms
- Centrilobular fibrosis
- Idiopathic bronchiolocentric interstitial pneumonia
- Peribronchiolar metaplasia
Definitions
- 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
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Airway-centered interstitial fibrosis (ACIF)
Synonyms
- Centrilobular fibrosis
- Idiopathic bronchiolocentric interstitial pneumonia
- Peribronchiolar metaplasia
Definitions
- 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
