Orthopnea and progressive hypoxemia are common complaints in the older aged patients. When these symptoms don’t seem to stem from a benign cause, there becomes a cause for concern of a severe underlying condition. First line interventions are then deployed and the hope is that they could resolve the matter with lesser invasive means. When the patient begins to have hemodynamic instability, this then calls for emergency intervention with a potential hospitalization. A patient could then be subject to numerous diagnostic tests and therapies in order to resolve the underlying condition. We present the case of a patient who was having progressively worsening orthopnea and likely would have died of acute respiratory failure due to idiopathic pulmonary fibrosis. 

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disorder characterized by scarring of the lungs from an unknown cause [1]. The condition has a poor long-term prognosis [1]. Classic features of IPF include the gradual onset of shortness of breath, progressive dyspnea, and a dry, nonproductive cough [1]. Orthopnea is caused by pulmonary congestion during recumbency [2]. In the horizontal position there is redistribution of blood volume from the lower extremities and splanchic beds to lungs [2]. In patients with congestive heart failure the pulmonary circulation may already be overloaded, and there may be reabsorption of edema fluid from previously dependent parts of the body [2]. Management of dyspnea in PF requires a multimodal approach addressing both physiological and perceptual aspects of breathlessness [3]. Pharmacologic strategies include antifibrotic therapy with nintedanib and pirfenidone, which slow disease progression and may help preserve lung function and mitigate increase of dyspnea [3]. Oxygen therapy improves exercise tolerance in patients with exertional or resting hypoxemia [3]

This article will present the interesting case of a patient who suffered from progressively worsening orthopnea and was found to have acute respiratory failure in the setting of newly diagnosed idiopathic pulmonary fibrosis.

The patient was a 70-year-old male with a past medical history of uncontrolled hypertension, hypertriglyceridemia, and tobacco abuse presented to the primary care clinic for a same day sick appointment. He complained of a 1-week history of progressive shortness of breath. He denied any recent bacterial or viral illnesses. Stated that he had been prescribed losartan for his hypertension but had not been taking it for a few months due to him running out. The patient had a Well’s Score of 4.5 which indicated a moderate risk of him having a pulmonary embolism. His pulse in the office was 108 BPM and his spO2=86% on room air. He received an EKG in office which showed sinus tachycardia, left anterior fascicular block, and a left axis deviation (Figure 1). 

Figure 1: depicts the EKG that the patient had at the PCP’s office.

The patient’s chest x-ray showed coarse heterogeneous airspace opacities throughout both lungs, most pronounced in the left lung base (Figure 2). The primary consideration at the time was an atypical infectious process. There were no definite pleural effusions. There was also mild cardiac enlargement. The patient was instructed by the physician to present to the emergency room, however he refused. When the patient presented to the clinic four days later, he had a CBC w/dif, D-Dimer, BNP, CMP, and procalcitonin ordered. The patient’s D-Dimer and BNP were found to be significantly elevated and the patient was called by the primary care physician to immediately present to the emergency room, and this time he did.

Figure 2: depicts the chest x-ray that the patient had at his primary care physician’s office.

When the patient presented to the emergency room, he informed the physician that he was having this progressive shortness of breath which is worsened by lying flat. At that time, he also noted that he had a significant family history of pulmonary fibrosis. He was also having a dry cough. A repeat EKG showed sinus rhythm with a left axis deviation. As the patient was having progressively worsening hypoxemia, he was then started on 5L of supplemental oxygen. A CT Pulmonary Angiogram was ordered to rule out a pulmonary embolism (Figure 3).

Figure 3: depicts the CT Pulmonary Angiogram that the patient had.

The CT Pulmonary Angiogram showed no pulmonary emboli. However, there was a degree of interstitial pulmonary fibrosis in a pattern suggesting usual interstitial pneumonitis. There was also probable reactive lymphadenopathy in the mediastinum and hilum. The patient was then admitted to the hospital overnight for the further treatment of acute hypoxic respiratory failure in the setting of idiopathic pulmonary fibrosis.

The patient was then admitted to a telemetry unit and was started on 6L of supplemental oxygen and intravenous steroids. He was placed on DuoNeb and Albuterol inhalers on an as needed basis. The admitting hospitalist did not place the patient on antibiotics as he did not display infectious symptoms. The next day the patient noted some improvement in his shortness of breath and his cough. Inpatient pulmonary consultation was not available at the time. The patient was then transitioned from 6L of supplemental oxygen down to 3L. On the third day of the patient’s hospitalization, he failed an oxygen road test despite his improvements. The patient was then placed on intravenous Lasix due to his persistent hypoxia and elevated BNP. He was also started on intravenous Ceftriaxone and Azithromycin. The patient then had a transthoracic echocardiogram ordered (Figure 4). The echocardiogram showed a normal systolic left ventricular function with an EF=50%. There was also a Grade 1 Diastolic Dysfunction. There was trivial tricuspid regurgitation.

Figure 4: depicts the transthoracic echocardiogram that the patient had.

Shortly thereafter, the patient’s condition seemed to be improving despite needing 8L of supplemental oxygen as compared to 6L of oxygen to maintain the spO2 at 90-92%. The belief at the time was that the patient’s new baseline requirement was 8L of supplemental oxygen. He had a home nebulizer and oxygen mask ordered for him. The patient had his intravenous Lasix discontinued. Intravenous Ceftriaxone and Azithromycin were continued. The next day the patient was discharged home with an ipratropium-albuterol inhaler, prednisone taper, and methocarbamol for muscle spasms. 

Firstly, the patient had gone a few months after being out of his losartan. This could have been due to a communication barrier to the clinic or a financial barrier. Having chronic uncontrolled hypertension could lead to him having a heart attack or a stroke. Secondly, the patient initially presented with the pathognomonic triad for a pulmonary embolism and even had a moderate risk of having one when the Well’s score was calculated. The patient’s main symptom was progressively worsening orthopnea. His initial chest x-ray was relatively unremarkable. It was important for the physician in this case to recognize that the patient’s condition was indeed deteriorating and that a further workup was needed. Had the patient complied with the physician’s initial plea to go to the emergency room, the patient likely would have had a shorter hospital stay and lesser complications. However, the physician was incredibly astute in deciding to call the patient after receiving the abnormal D-Dimer and BNP laboratory results. It is fortunate that the patient came to his senses and complied with the physician’s medical advice. It is necessary for a physician to understand the reasons for a patient’s refusal. Additionally, this patient was later found to have idiopathic pulmonary fibrosis and his only symptom was the orthopnea. He was not having fatigue, lethargy, or dyspnea on exertion. He seems to have an atypical presentation of the illness which highlights the current gaps in knowledge for it. Also, 6L of supplemental oxygen was quite a high dosage to need for only having one primary symptom of the condition. 

Orthopnea is a symptom for which there could be numerous underlying etiologies. Additionally, while pulmonary fibrosis is a common clinical ailment, there is still a plethora to learn about it. Had the physician in this case not been so vigilant in assessing the patient's rapidly deteriorating progressive, the patient likely may not have lived. It also must be considered that despite the physician’s pleas to a patient to present to the emergency room, the patient still has the power of autonomy to refuse.

Statement of Informed Consent:

Informed consent was obtained from the patient that was presented in the case report.