Airway-centered interstitial fibrosis (ACIF) is described as one of the interstitial lung diseases (ILDs) with rare histologic patterns. It is characterised by predominant airway involvement with centrilobular fibrosis, peribronchiolar metaplasia and bronchiolocentric inflammatory changes. We report the case of a female who presented with pneumothorax and central diabetes insipidus, diagnosed as ACIF on lung biopsy.
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