Comorbid Conditions in Idiopathic Pulmonary Fibrosis: Recognition and Management

Abstract

Idiopathic pulmonary fibrosis (IPF), a fibrosing interstitial pneumonia of unknown etiology, primarily affects older adults and leads to a progressive decline in lung function and quality of life. With a median survival of 3–5 years, IPF is the most common and deadly of the idiopathic interstitial pneumonias. Despite the poor survivorship, there exists substantial variation in disease progression, making accurate prognostication difficult. Lung transplantation remains the sole curative intervention in IPF, but two anti-fibrotic therapies were recently shown to slow pulmonary function decline and are now approved for the treatment of IPF in many countries around the world. While the approval of these therapies represents an important first step in combatting of this devastating disease, a comprehensive approach to diagnosing and treating patients with IPF remains critically important. Included in this comprehensive assessment is the recognition and appropriate management of comorbid conditions. Though IPF is characterized by single organ involvement, many comorbid conditions occur within other organ systems. Common cardiovascular processes include coronary artery disease and pulmonary hypertension (PH), while gastroesophageal reflux and hiatal hernia are the most commonly encountered gastrointestinal disorders. Hematologic abnormalities appear to place patients with IPF at increased risk of venous thromboembolism, while diabetes mellitus (DM) and hypothyroidism are prevalent metabolic disorders. Several pulmonary comorbidities have also been linked to IPF, and include emphysema, lung cancer, and obstructive sleep apnea. While the treatment of some comorbid conditions, such as CAD, DM, and hypothyroidism is recommended irrespective of IPF, the benefit of treating others, such as gastroesophageal reflux and PH, remains unclear. In this review, we highlight common comorbid conditions encountered in IPF, discuss disease-specific diagnostic modalities, and review the current state of treatment data for several key comorbidities.

Physiology of the lung in idiopathic pulmonary fibrosis

“…Because IPF typically affects older patients and smokers, multiple comorbidities can affect patients with IPF [100,101]. In particular, chronic obstructive pulmonary disease (COPD) and pulmonary emphysema are common.…”

Impact of Depression on Patients With Idiopathic Pulmonary Fibrosis

“…We and others, highlight the importance of applying holistic care approaches for the treatment of such complex and heterogeneous diseases, such as IPF (6). Assessment and management of comorbidities should be an integral part of our every-day clinical practice when dealing with patients with IPF, considering that almost 40% of these patients die from non-IPF causes (13). With regards to depression, it is now generally accepted that all patients, irrespective of disease severity should be carefully screened and monitored for depressive symptoms, during disease clinical course.…”

“…Importantly, IPF is an age-related disease and thus presents with major comorbidities that significantly impact survival and quality of life (7)(8)(9). Besides the traditionally recognized comorbidities of lung cancer, pulmonary hypertension and gastroesophageal reflux disease (7,(10)(11)(12)(13), conditions related to the psychological impact of the disease have recently gained much of attention.…”

Diabetes and Lung Disease: A Neglected Relationship

“…Many comorbid conditions are known to occur in IPF, including coronary artery disease, pulmonary hypertension, gastroesophageal reflux, and diabetes [63]. It remains unclear whether the presence of diabetes influences survival in patients with IPF.…”