Chest
Volume 148, Issue 1, July 2015, Pages 242-252
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Recent Advances in Chest Medicine
Management of Idiopathic Pulmonary Fibrosis in the Elderly Patient

https://doi.org/10.1378/chest.14-2475Get rights and content

Idiopathic pulmonary fibrosis (IPF) is strongly associated with advanced age. Making an accurate diagnosis of IPF is critical, as it remains only one of many potential diagnoses for an elderly patient with newly recognized interstitial lung disease. Optimal management of IPF, especially in older-aged patients, hinges on such factors as balancing the application of standard-of-care measures with the patient's overall health status (robustness vs frailty) and considering the patient's wishes, desires, and expectations. IPF is known to be associated with certain comorbidities that tend to be more prevalent in the elderly population. Until recently, options for the pharmacologic management of IPF were limited and included therapies such as immunosuppressive agents, which may pose substantial risk to the elderly patient. However, the antifibrotic agents pirfenidone and nintedanib have now become commercially available in the United States for the treatment of IPF. The monitoring and treatment of patients with IPF, especially elderly patients with comorbid medical conditions, require consideration of adverse side effects, the avoidance of potential drug-drug interactions, treatment of comorbidities, and the timely implementation of supportive and palliative measures. Individualized counseling to guide decision-making and enhance quality of life is also integral to optimal management of the elderly patient with IPF.

Section snippets

How Should a Confident Diagnosis Be Made in the Elderly Patient?

IPF is one of many potential diagnoses for a patient with newly recognized ILD. Therefore, accurately diagnosing IPF remains one of the most basic, yet critical steps in providing optimal care for the elderly patient with ILD.6, 7 The American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association consensus statement on the diagnosis and management of IPF provides a useful diagnostic algorithm for evaluating a patient with possible IPF.8

What Is the Impact of Disease Complications and Comorbidities?

It is well established that the prognosis of IPF is significantly worse in elderly patients than in younger patients.14, 15 There are a number of potential explanations, including a more aggressive disease course in the elderly, lead-time bias with or without a delay in diagnosis, and a more significant impact of IPF-associated comorbidities on outcomes in elderly patients. A variety of comorbidities and complications16 (Table 2) can have a profound impact on quality of life and outcomes,

What Are Key Considerations and Challenges for Disease Management in the Elderly Patient?

Once a confident diagnosis of IPF has been attained, optimal management must balance standard-of-care measures with the patient's overall health status as well as the patient's wishes, desires, and expectations. For serial monitoring of health status, it is usual practice to see patients with IPF every 3 to 6 months. At each visit, consideration should be given to pulmonary function testing, a 6-min walk test (6MWT), and oxygen titration walk study.28, 29, 30, 31, 32 However, clinical testing

What Are Current Treatment Considerations?

Finding effective pharmacologic treatment of IPF has mostly proven elusive despite the completion of many clinical trials.7, 36 Medical therapies prescribed for patients with IPF have ranged from corticosteroids to cytotoxic drugs or various combinations, but these were previously adopted in the absence of robust clinical trial results.36, 37 Indeed, immunosuppressive therapies that were suggested and commonly administered prior to publication of the current American Thoracic Society/European

What Are Important Prognostic Considerations?

IPF is a heterogeneous disorder with a clinical course that varies from prolonged periods of stability to episodes of rapid progression8, 14; therefore, clinicians must carefully consider many factors when providing patients with a prognosis. Patients with a lesser degree of fibrotic change on HRCT scan are likely to have a better survival with relatively stable lung function when compared with those with HRCT scan changes that indicate “definite” UIP, a greater degree of fibrotic change, or

How Can Patient-Centered Care and Personalized Management Be Provided?

Traditionally, the care of patients with IPF has largely been driven by a disease-centered model, which assumes that treating the disease treats the patient and, thus, leads to improved quality and quantity of life. However, adopting a patient-centered care model may be far more effective, especially for managing older patients with IPF.71 In the elderly patient population, IPF occurs in the context of accumulating age-related comorbidities. Therefore, evaluating elderly patients for treatable

When and How Should Disease-Focused Therapies Be Transitioned to Supportive and Palliative Care?

Symptom management and palliation should be a component of patient care from the time that the initial diagnosis is established.73 When patients have progressive lung function loss and worsening quality of life despite a disease-centered approach with antifibrotic therapies and other interventions, management should increasingly focus on patient support and symptom control, which are integral aspects of palliative care. Pulmonary rehabilitation, supplemental oxygen use, cough suppression,

Summary

The prevalence of IPF is highest in the population of affected patients who are elderly, making optimal IPF management in this group an important priority for their health-care providers. IPF can be effectively managed even in the “oldest old” (Table 4). Critical steps include an accurate and timely diagnosis that minimizes risk of harm; careful assessment of disease status; provision of disease-specific, supportive, and palliative care as needs evolve; and appropriate monitoring. Additionally,

Acknowledgments

Financial/nonfinancial disclosures: The authors have reported to the following conflicts of interest: Dr Meyer is a consultant for InterMune and Medimmune in the field of IPF, and he has received research funding from Actelion Pharmaceuticals Ltd; Boehringer Ingelheim GmbH; Bristol-Myers Squibb; FibroGen, Inc; InterMune; and the National Institutes of Health in the field of IPF. Dr Danoff participates in an industry advisory committee for Boehringer Ingelheim GmbH and the ASCEND Mortality

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