Clinicians presented the case of a patient with advanced idiopathic pulmonary fibrosis (IPF) who developed an acute exacerbation with multiple complications. The case report was published in Respirology Case Reports.

An 84-year-old woman presented with a 2-week history of dyspnea, chills, and rigors. She denied having a productive cough and had been self-isolating at home. She confirmed receiving 3 doses of the COVID-19 vaccine. 

The patient revealed that she had never smoked and had no other symptoms suggestive of thromboembolic disease. Two years prior, she was diagnosed with IPF following progressive dyspnea. She was initially prescribed nintedanib, which she stopped taking due to nausea and vomiting.

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Pirfenidone was suggested but declined by the patient due to fears of similar adverse effects. As a result, her pulmonary function tests over the years revealed a gradual deterioration in total lung capacity and diffusion capacity. 

Upon presentation, she was hypoxic (70% saturation on room air) and tachypneic (45 breaths per minute). Thoracic auscultation revealed widespread fine inspiratory crackles. She had leukocytosis (12.9×109 cells/L) and neutrophilia (10.1×109 cells/L). Her C-reactive protein level was extremely high (129 mg/L). High-resolution computed tomography (HRCT) revealed ground glass opacities. 

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Her physicians prescribed broad-spectrum antibiotics, high-flow oxygen therapy, and intravenous hydrocortisone. On day 2, in the absence of significant improvement, they prescribed a 3-day trial of pulsed methylprednisolone (1 g/daily). On day 5, she demonstrated symptomatic improvement but complained of transient central chest heaviness; serial troponin and an electrocardiogram were both negative. A repeat HRCT unexpectedly revealed a pneumomediastinum. 

Despite clinical improvement, on day 8, the patient reported breathlessness while standing. An arterial blood gas analysis was ordered with the patient standing and sitting, revealing a drop of 21 mmHg despite increased respiratory effort, resulting in respiratory alkalosis. 

Nevertheless, on day 14, her condition was stable enough for her to be discharged to inpatient rehabilitation. Another repeat HRCT demonstrated a resolved pneumomediastinum. Symptoms of platypnea-orthodeoxia had also improved significantly. 

“[Acute exacerbations] of IPF are seen annually in 10%-20% of people with IPF,” the authors of the report wrote. “The underlying pathophysiology is not completely elucidated, but likely involves an exaggerated inflammatory response due to an acute trigger such as infection or aspiration.” 


Ledger T, Braude S. Multiple complications of advanced IPF in an individual patient presentationRespirol Case Rep. 2023;11(4):e01118. doi:10.1002/rcr2.1118