Introduction
Idiopathic Pulmonary Fibrosis (IPF) is a spontaneous, progressive, fibrosing disease of the lung, typically identified in adults ages 50 and older. Diagnosis is made via high-resolution CT (HRCT) when a Usual Interstitial Pneumonia (UIP) pattern is present; however, if diagnosis remains questionable, surgical lung biopsy (SLB) is often pursued.

Case Presentation
We present the case of a 39-year-old African-American male, current smoker, medical history of bronchiectasis accompanied by chronic, productive cough, and a social history of prior shipyard employment. He presented with two weeks of progressive exertional dyspnea, increased sputum, and fever.

Physical exam revealed tachycardia, bilateral inspiratory rales and digital clubbing. Chest x-ray showed progression of baseline bilateral interstitial opacities, and HRCT revealed diffuse interstitial thickening, ground-glass opacities with basilar predominance, and diffuse bronchiectasis. Evaluation for alpha-1 antitrypsin deficiency, cystic fibrosis mutations, celiac disease, autoimmune serologies, and immunoglobulin deficiency were unremarkable. Bronchoalveolar lavage analysis demonstrated neutrophil predominance with negative microbiologic testing. SLB revealed diffuse alveolar damage superimposed on patchy collagen fibrosis and architectural distortion in the form of honeycomb changes consistent with UIP. Per the 2019 IPF guidelines, he was diagnosed with IPF and given an “IPF Likely” grade.

Work in Progress
IPF is thought to develop from repetitive micro-injury to chronically dysregulated alveolar epithelium. Our patient’s tobacco history or occupational exposures may have been the nexus for his interstitial lung damage.

UIP is the radiologic/histopathologic hallmark of IPF, demonstrating reticular fibrosis and subpleural honeycombing with basilar predominance. Bronchiectasis in IPF results from fibrotic tissue “pulling” on bronchi. Conversely, our case demonstrates a diffuse freestanding bronchiectatic process that preceded fibrotic changes of IPF. The question remains whether our patient developed IPF from recurrent exacerbations of idiopathic bronchiectasis, or if his bronchiectasis was an unappreciated indicator of an underlying, progressive interstitial lung disease (ILD).

We encourage physicians to be mindful that patients with bronchiectasis may be at risk for IPF development. Society guidelines recommend imaging of bronchiectasis at time of diagnosis and clinical deterioration. We argue that periodic imaging should be tailored to bronchiectasis etiology, as otherwise unexplained disease may be an indicator of risk for ILD development.

References and Resources

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