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Airway-centered Interstitial Fibrosis: An Unusual Presentation
The Indian Journal of Chest Diseases & Allied Sciences
|September 6, 2018
Summary
Airway-centered interstitial fibrosis (ACIF), a rare interstitial lung disease (ILD), typically involves airway inflammation and fibrosis. This case highlights ACIF presenting with pneumothorax and central diabetes insipidus.
Area of Science:
- Pulmonology
- Pathology
- Endocrinology
Background:
- Airway-centered interstitial fibrosis (ACIF) is a rare interstitial lung disease (ILD) characterized by specific histologic patterns.
- ACIF predominantly involves airway structures with centrilobular fibrosis, peribronchiolar metaplasia, and bronchiolocentric inflammation.
Observation:
- A female patient presented with spontaneous pneumothorax.
- The patient also exhibited symptoms of central diabetes insipidus.
Findings:
- Lung biopsy confirmed the diagnosis of Airway-centered interstitial fibrosis (ACIF).
- This case demonstrates an unusual presentation of ACIF.
Implications:
- This case expands the known clinical spectrum of ACIF.
- Highlights the importance of considering ACIF in patients with interstitial lung disease and concurrent pneumothorax or central diabetes insipidus.
- Suggests potential links between ACIF and endocrine dysfunction.
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