What is the hospital workup and management for a patient with Interstitial Lung Disease (ILD) and pulmonary hypertension, previously in hospice care, presenting with acute shortness of breath on exertion?

From the Guidelines

For a patient with interstitial lung disease (ILD) and pulmonary hypertension who was in hospice and presents with acute shortness of breath on exertion, the hospital workup and management should prioritize symptom relief and comfort measures, with a focus on maintaining oxygen saturation above 90% and managing fluid overload, while also considering the patient's hospice status and goals of care. The initial evaluation should include vital signs, a thorough physical examination, and basic laboratory tests such as complete blood count, comprehensive metabolic panel, BNP, and troponin 1. Imaging studies like chest X-ray and possibly a CT pulmonary angiogram can help rule out pulmonary embolism if clinically suspected. An ECG and echocardiogram can assess cardiac function and pulmonary pressures.

Diagnostic Evaluation

• Obtain vital signs including oxygen saturation
• Perform a thorough physical examination
• Order basic laboratory tests including complete blood count, comprehensive metabolic panel, BNP, and troponin
• Imaging should include a chest X-ray and possibly a CT pulmonary angiogram to rule out pulmonary embolism if clinically suspected
• An ECG and echocardiogram can help assess cardiac function and pulmonary pressures
• Arterial blood gases may be useful to evaluate gas exchange

Management

• Provide supplemental oxygen to maintain saturation above 90% if hypoxemic
• Consider diuretics like furosemide 20-40mg IV if fluid overload is present
• For symptom relief, use opioids such as morphine 2-4mg IV/SC every 4 hours as needed for dyspnea
• If infection is suspected, obtain appropriate cultures and start empiric antibiotics
• Bronchodilators like albuterol nebulizer treatments may help if there's a bronchospastic component
• Throughout this process, it's crucial to have goals-of-care discussions with the patient and family, respecting the patient's hospice status and determining whether aggressive interventions align with their wishes, as suggested by the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases 1. The management approach should balance diagnostic evaluation with comfort measures, recognizing that the underlying ILD and pulmonary hypertension are progressive conditions with limited treatment options in the hospice setting, and considering the lack of correlation between lung function variables and pulmonary artery pressure in patients with IPF, as noted in the ACCF/AHA 2009 expert consensus document on pulmonary hypertension 1.

From the FDA Drug Label

The FDA drug label does not answer the question.

From the Research

Hospital Workup

• The hospital workup for a patient with interstitial lung disease (ILD) and pulmonary hypertension (PH) presenting with acute shortness of breath on exertion may include 2:
  • Right heart catheterization to assess pulmonary arterial pressure and cardiac output
  • Transthoracic echocardiography to evaluate right ventricular function and estimate pulmonary artery pressure
  • Blood tests to measure brain natriuretic peptide (BNP) levels and assess oxygenation status
  • Pulmonary function tests, such as diffusion capacity of the lungs for carbon monoxide (DLCO), to assess lung function
• A systematic review and meta-analysis found that the pooled prevalence of PH in patients with ILD was 36% using right heart catheterization and 34% using transthoracic echocardiography 3

Management

• The management of PH in patients with ILD may involve the use of pharmacological therapies, such as 4:
  • Sildenafil, a phosphodiesterase-5 inhibitor, which has been shown to improve exercise capacity and reduce BNP levels
  • Bosentan, an endothelin receptor antagonist, which has been shown to improve hemodynamic parameters and reduce morbidity and mortality
• Combination therapy with bosentan and sildenafil may be considered for patients who are not responding to monotherapy [(5,6)]
• The choice of therapy should be individualized based on the patient's specific condition, disease severity, and response to treatment [(2,3)]

Specific Considerations

• Patients with ILD and PH who are in hospice care may require careful consideration of their treatment goals and priorities 2
• The presence of PH in patients with ILD is associated with increased symptom burden and worse prognosis, highlighting the importance of early diagnosis and treatment 3