The hospitalized patient with interstitial lung disease: a hospitalist primer
Interstitial lung disease (ILD) is a diverse group of disorders typically with insidious onset. Diagnosis and management largely occur in the outpatient setting; however, ILD can present acutely necessitating hospitalization. Effective inpatient management requires the clinician to establish an accurate diagnosis and understand the natural history and treatment responsiveness of each ILD subtype. We propose a general framework for approaching the evaluation of hospitalized patients with ILD, and provide focused guidance on key inpatient diagnostic and management decisions. Journal of Hospital Medicine 2017;12:580-584. © 2017 Society of Hospital Medicine
© 2017 Society of Hospital Medicine
When should hospitalized ILD patients be referred for inpatient lung transplant evaluation?
A subset of hospitalized patients with ILD will not respond to supportive and pharmacologic care, particularly those with advanced lung fibrosis. In these cases, lung transplantation may be the only remaining treatment option. This is particularly true for patients presenting with IPF, and it is 1 of the most common indications for lung transplantation. Patients with respiratory failure and ILD should be evaluated early in the hospital course for transplantation or considered for transfer to a transplant center. General contraindications to transplant are age older than 70 years, underweight or elevated BMI (generally higher than 30), malignancy within the last 2 years (with the exception of cutaneous squamous and basal cell tumors), untreatable major organ dysfunction other than the lung, noncurable chronic extrapulmonary infection (chronic active viral hepatitis B, hepatitis C, human immunodeficiency virus), significant chest wall deformity, untreatable psychiatric or psychologic disease, substance addiction within the last 6 months, or lack of dependable social support.40 In select patients with ILD and gas exchange abnormalities, mechanical ventilation or extracorporeal membrane oxygenation may be used to bridge a patient to lung transplantation.41
What should you tell your ILD patient to expect at discharge?
Accurate diagnosis is important not only for acute inpatient management, but for informing long-term prognosis. Acute-onset ILD tends to be more reversible, to be responsive to medical therapy, and to have a more favorable overall outcome. On the other hand, acute exacerbations of established ILD, particularly IPF, can have a more unfavorable and treatment-refractory course. Once a diagnosis is established, it is important both to provide patients with information and ensure appropriate outpatient follow-up. The Pulmonary Fibrosis Foundation (the largest U.S. advocacy and support organization for patients with ILD) provides information on ILD to patients and families and can serve as an important educational source.42 Prior to discharge, it is important to evaluate the oxygen needs of patients at rest and with exertion. Referral to an ILD center at discharge is important whenever possible, to monitor clinical symptoms and lung function, initiate or assess response to treatment, and provide supportive care, including oxygen therapy, pulmonary rehabilitation, and outpatient lung transplant referral.
CONCLUSION
ILD is a group of heterogeneous disorders characterized by lung inflammation and fibrosis. Although the onset of disease is typically insidious, patients can present acutely requiring hospitalization. Inpatient management varies significantly depending on ILD subtype, and, therefore, accurate diagnosis is key in determining treatment and prognosis. As we develop an improved understanding of the mechanisms of acute presentations of ILD, and our approaches to detection and treatment improve as a result of clinical trials, we anticipate continued modifications to this shared framework.
Disclosure
Dr. Collard reports personal fees from Alkermes, aTyr Pharmaceuticals, Bayer, Boehringer Ingelheim, Bristol-Myers Squibb, Global Blood Therapeutics, Genoa, ImmuneWorks, Moerae Matrix, Navitor, Parexel, Patara, Pharma Capital Partners, Prometic, Takeda, Toray, and Xfibra, outside the submitted work. Drs. Farrand and Shah report no financial conflicts of interest.
