A 65yearold male with a history of Parkinson’s, hypertension, and smoking presented 2 weeks after an uncomplicated left shoulder arthoplasty with one week of worsening shortness of breath, fever, cough, and profound hyoxemia. His exam was significant for mild respiratory distress with crackles loudest at the bases. Initial CT found bibasilar consolidation and ground glass opacifications in the background of interstitial lung disease with no evidence of pulmonary emboli. He was treated empirically for hospitalacquired pneumonia with mild volume overload. With no improvement after one week and concerns for an untreated infection a bronchoscopy was performed, which was largely unrevealing. Given the lack of clear diagnosis, on hospital day ten he underwent an open lung biopsy. The pathology showed a usual interstitial pneumonia pattern and fibrosis with superimposed organizing diffuse alveolar damage. This pathology in the clinical context supported a diagnosis of acute exacerbation of idiopathic pulmonary fibrosis (IPF). He was started on high dose steroids and over the subsequent three weeks he remained dependent on highflow supplemental oxygen. His recovery was complicated by an open chest wound and persistent broncopleural fistula. Repeat CT showed evolution of the consolidation and ground glass to overt lung fibrosis. After a two month hospitalization the family decided to move toward comfort care and the patient was transitioned to hospice.
Idiopathic pulmonary fibrosis (IPF) is the most common subcategory of the idiopathic interstitial pneumonias. The typical clinical course is a chronic progressive deterioration of lung function. Episodes of acute decline in lung function are usually associated with infections, pulmonary emboli or worsening cardiac function and often present to the hospital for intensive support. When no clear inciting event can be identified, it is classified as an acute exacerbation of IPF. The term was first used in the early 1990’s and the pathophysiology remains poorly understood. Many etiologies have been postulated including viruses, other infectious material, and procedures such as biopsy, bronchoscopy, or mechanical ventilation. Treatment strategies include immunomodulation with high dose steroids and immunosuppressive medications, and empiric anticoagulation. However, the current evidence shows no clear superior treatment strategy and the overall outcomes remain very poor with inhospital mortality estimated between 2950%.
The diagnosis of acute exacerbation of IPF is a diagnosis of exclusion. There are currently no clearly beneficial treatments and the prognosis remains poor. This case serves to highlight the difficulties in diagnosis and management of this rare and interesting condition.
To cite this abstract:Dharia A. Adding Insult to Injury a Case of Acute Exacerbation of Ipf. Abstract published at Hospital Medicine 2012, April 1-4, San Diego, Calif. Abstract 97769. Journal of Hospital Medicine. 2012; 7 (suppl 2). https://www.shmabstracts.com/abstract/adding-insult-to-injury-a-case-of-acute-exacerbation-of-ipf/. Accessed May 26, 2019.