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SESSION TITLE: Occupational and Environmental Lung Disease Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: We describe a case of acute progression of chronic hypersensitivity pneumonitis (HP) in an adult, previously misdiagnosed as COPD for 13 years due to severe emphysematous changes seen on imaging. He was also found to have acutely worsened disease as a result of Covid-19. CASE PRESENTATION: A 64-year-old male presented to the pulmonary clinic with dyspnea on minimal exertion. He reported respiratory complaints for 13 years, treated with 2 L/min of oxygen overnight, and budesonide-formoterol and tiotropium inhalers. These complaints were previously associated with brief occupational mold exposure and possible COPD. His respiratory distress worsened one year ago when he was hospitalized for Covid-19. On discharge, his oxygen requirement had increased to 6 L/min. CT chest showed air-trapping in the mid-zones bilaterally, mosaic attenuation, and peri-bronchial thickening. PFTs showed an FEV1 33% and FVC 55% of predicted, consistent with severe obstruction and reduction in lung volume. As the patient was a lifetime non-smoker, alternative diagnoses were pursued. Alpha-1 antitrypsin levels and immunologic testing, including scleroderma and myositis panels, were within normal limits. Positive findings included CCP IgG/IgA antibodies at 96 units and HP panel positive for pigeon serum antibodies. Prompted by this testing, the patient revealed that he had parakeets in his home for the past 15 years. He also reported significant symptom improvement on occasions that he took a course of steroids. Based on these findings, a diagnosis of chronic fibrotic hypersensitivity pneumonitis with bronchiolitis obliterans was considered. The patient's severe airflow obstruction and respiratory failure precluded surgical lung biopsy. Empiric management was initiated with 30 mg of prednisone daily with a slow taper and instruction to eliminate exposure to exotic birds. DISCUSSION: HP is commonly caused by inhalation of and sensitization to an aerosolized environmental antigen;a common subtype is bird fancier's lung due to repetitive exposure of avian antigen. Continuous antigen exposure increases the risk for development of fibrosis, which was also seen in our patient. The most commonly described radiologic findings in HP are ground-glass opacities, ill-defined centrilobular nodules, and focal areas of air trapping resulting in mosaic attenuation and fibrosis. More than 20% lymphocytosis on bronchoalveolar lavage is also a sensitive tool in detecting alveolitis. The relationship between Covid-19 and disease progression in HP is not well studied. CONCLUSIONS: Chronic hypersensitivity pneumonitis from avian antigens, or Bird fancier's lung, can present with severe emphysematous changes on CT imaging, along with obstructive pattern of PFTs. This should be an important differential, especially in patients who are non-smokers. Covid-19 causes disease progression in HP, this relationship needs to be further explored. Reference #1: Funke M., Fellrath J.-M. Hypersensitivity pneumonitis secondary to lovebirds: a new cause of bird fancier's disease. Eur. Respir. J. 2008;32:517–521. DOI: 10.1183/09031936.00108507 Reference #2: Pereira C., Gimenez A., Kuranishi L., Storrer K. Chronic hypersensitivity pneumonitis. J. Asthma Allergy. 2016;9:171–181. DOI: 10.2147/JAA.S81540 Reference #3: C.S. Glazer, C.S. Rose, D.A. Lynch Clinical and radiologic manifestations of hypersensitivity pneumonitis J. Thorac. Imag., 17 (4) (2002), pp. 261-272. DOI: 10.1097/00005382-200210000-00003 Morell F, Roger A, Reyes L, Cruz MJ, Murio C, Muñoz X Bird fancier's lung: a series of 86 patients. Medicine (Baltimore). 2008;87(2):110-130. DOI: 10.1097/MD.0b013e31816d1dda DISCLOSURES: No relevant relationships by Momina Amjad No relevant relationships by Amit Chopra No relevant relationships by Rafeh Safdar
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SESSION TITLE: Critical Thinking SESSION TYPE: Case Reports PRESENTED ON: 10/19/2022 09:15 am - 10:15 am INTRODUCTION: Compressive therapies to improve respiratory mechanics, such as abdominal compression, have been described in literature in patients with COVID-19 induced acute respiratory distress syndrome (COVID-19 ARDS) 1–3. These compressive therapies minimize the risk of barotrauma by equal distribution of pressure across the alveoli. Hence, they help with lung protective ventilation. This phenomenon of paradoxical improvement in respiratory compliance with increase in intraabdominal pressure (IAP) has not been described in ILD population. We describe a case of end-stage fibrotic ILD, secondary to hypersensitivity pneumonitis (HP), exhibiting a paradoxical improvement in respiratory compliance with sustained abdominal compression. CASE PRESENTATION: 56-year-old female with history of NASH-related cirrhosis was transferred to our hospital for expedited work-up of lung transplant due to rapid progression of biopsy-proven steroid-unresponsive fibrotic HP. Due to worsening hypoxic respiratory failure, she was intubated on arrival to our hospital. Following intubation, she was sedated and paralyzed and was found to have high peak and plateau pressures in supine and reverse Trendelenburg positions. However, on application of abdominal pressure, her peak and plateau pressure showed a dramatic reduction in absolute values. This reduction was sustained during the entire duration of the maneuver. Overall, it reduced driving pressures and improved the static compliance of the respiratory system. We subsequently applied abdominal binder (table 1) and found a similar decrease in pressures (see images). Unfortunately, due to functional disability, patient was not deemed a candidate for lung and liver transplant and was transitioned to comfort measures. DISCUSSION: Paradoxical improvement in respiratory compliance has been demonstrated in late-stage COVID ARDS1,2. The mechanism behind this is unclear. In theory, increase in IAP increases intrapleural pressures, reduces end-expiratory volume and overdistention of aerated lung1,2. We hypothesize that patients with end-stage ILD behave similarly to patients with COVID-ARDS. However, this is purely exploratory as our observations are limited by lack of intrapleural measurements. Use of abdominal compression is a simple maneuver, which can be performed at the bedside to assess for the paradoxical phenomenon. Even though we postulate that long-term abdominal compression is well tolerated, we do not know the effects of sustained long-term abdominal compression on gas-exchange and chest wall dynamics. CONCLUSIONS: Patients with end-stage fibrotic lung disease, exhibiting high-driving pressures on mechanical ventilator in supine and reverse Trendelenburg positions, can be screened for reduction in peak and plateau pressures with abdominal compression. Use of this maneuver may help in lung-protective ventilation and minimize ventilator-induced lung injury. Reference #1: Elmufdi FS, Marini JJ. Dorsal Push and Abdominal Binding Improve Respiratory Compliance and Driving Pressure in Proned Coronavirus Disease 2019 Acute Respiratory Distress Syndrome. Crit Care Explor. 2021;3(11):e0593. doi:10.1097/cce.0000000000000593 Reference #2: Julia Cristina Coronado. Paradoxically Improved Respiratory Compliance With Abdominal Compression in COVID-19 ARDS. Is COVID-19 a risk factor Sev preeclampsia? Hosp Exp a Dev. 2020;(January):2020-2022. Reference #3: Stavi D, Goffi A, Shalabi M Al, et al. The Pressure Paradox: Abdominal Compression to Detect Lung Hyperinflation in COVID-19 Acute Respiratory Distress Syndrome. Am J Respir Crit Care Med. 2022;205(2):245-247. doi:10.1164/rccm.202104-1062IM DISCLOSURES: No relevant relationships by Abhishek Bhardwaj No relevant relationships by Brandon Francis no disclosure on file for Marina Freiberg;No relevant relationships by Simon Mucha No relevant relationships by Arsal Tharwani
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Rationale: Fibrotic hypersensitivity pneumonitis (FHP) is an irreversible lung disease with high morbidity and mortality. We sought to evaluate the safety and effect of pirfenidone on disease progression in such patients.Methods: In a phase 2 double-blind, single-center trial, we randomly assigned, in a 2:1 ratio, adults with FHP to receive either oral pirfenidone (2403 mg/day) or placebo for 52 weeks. Patients had to have CT lung fibrotic abnormalities affecting ≥5%, worsening respiratory symptoms, and either an increase in the extent of fibrosis on CT or relative decline in the FVC% of ≥5% within the 24-months before screening. The primary endpoint was the mean change from baseline to week 52 in %FVC. Secondary endpoints included progression-free survival (PFS, time to the first occurrence of any one of the following: a relative decline of ≥10% in FVC and/or DLCO, acute exacerbation, a decrease of ≥50 m in the 6-minute walk distance, increase in background prednisone by ≥10 mg or introduction of corticosteroids and/or steroid-sparing drugs, or death), change from baseline to week 52 in FVC slope and mean %DLCO, all-cause hospitalizations, CT progression of lung fibrosis, and safety. Results: After 40 patients had been randomized (mean age 67.1 years, 42.5% males) the study was stopped due to slow recruitment due to the COVID-19 pandemic. At baseline, demographics, smoking and inciting antigen exposure history, lung function, 6-minute walk distance, extent of CT lung fibrosis, and immunosuppressive therapy were balanced in both groups. There was no significant difference between the pirfenidone and placebo groups after adjusting for baseline %FVC and concomitant immunosuppressive therapy (p=0.88) in mean change from baseline to week 52 in %FVC. Secondary endpoints showed no difference between groups in change from baseline to week 52 in FVC slope, mean %DLCO, all-cause hospitalization and CT progression of lung fibrosis. However, a decrease in PFS favored pirfenidone (Table). The percentages of patients with any adverse events (AE) were similar in both groups. Nausea and rash, respectively, led to transient dose reduction of study treatment in 2 patients in the pirfenidone group. There were no treatment-related serious AE or AE leading to discontinuation of study treatment. No death occurred in the pirfenidone group and one death (respiratory) occurred in the placebo group. Conclusions: The trial was under powered to detect a difference in the primary endpoint. Pirfenidone was found to be tolerable and safe and compared to placebo reduced PFS in patients with FHP.