Idiopathic Pulmonary Fibrosis Treatment & Management

Updated: Aug 11, 2016
  • Author: Amanda M K Godfrey, MD; Chief Editor: Ryland P Byrd, Jr, MD  more...
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Treatment

Medical Care

The goal of any disease management strategy should include assessment and treatment of comorbid medical conditions. Common comorbid medical conditions found in patients with idiopathic pulmonary fibrosis (IPF) include chronic obstructive pulmonary disease, obstructive sleep apnea, gastroesophageal reflux disease, and coronary artery disease. Therefore, if any of these comorbid illnesses are present, they should be managed according to current practice guidelines.

Given the high prevalence of gastroesophageal reflux (GER) in patients with idiopathic pulmonary fibrosis, a retrospective study was conducted to investigate the relationship of reflux-related variables and survival time in patients with idiopathic pulmonary fibrosis. Of the 204 included patients, 34% reported symptoms of GER, 45% had a history of GER disease, 47% reported use of medications for GER, and 5% of patients had previously undergone Nissen fundoplication. On adjusted analysis, the use of GER medications was associated with a longer survival time. Additionally, patients taking GER medications had a lower fibrosis score on HRCT. [39]

Guidelines on idiopathic pulmonary fibrosis by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Association include the following points [40, 41] :

  • The guidelines recommend against the use of the following agents for the treatment of idiopathic pulmonary fibrosis: anticoagulation (warfarin); imatinib, a selective tyrosine kinase inhibitor against platelet-derived growth factor (PDGF) receptors; combination prednisone, azathioprine, and N-acetylcysteine; and selective endothelin receptor antagonist (ambrisentan).
  • The guidelines also give a conditional recommendation against the use of sildenafil, macitentan, and bosentan.
  • The panel has continued the 2011 conditional recommendation against the use of N-acetylcysteine monotherapy for IPF. They have also continued the 2011 conditional recommendation for the use of antacid therapy in patients without symptoms of GER.
  • The recommendation for the use of the following agents for the treatment of idiopathic pulmonary fibrosis is conditional: nintedanib, a tyrosine kinase inhibitor that targets multiple tyrosine kinases, including vascular endothelial growth factor, fibroblast growth factor, and PDGF receptors; and pirfenidone.

Any patient with idiopathic pulmonary fibrosis who is a current smoker should be encouraged to quit and offered pharmacologic therapy if needed.

Patients with hypoxemia (PaO2 < 55 mmHg or oxygen saturation as measured using pulse oximetry [SpO2] < 88%) at rest or with exercise should be prescribed oxygen therapy to maintain a saturation of at least 90% at rest, with sleep, and with exertion.

Vaccination against influenza and pneumococcal infection should be encouraged in all patients with idiopathic pulmonary fibrosis.

See Medication for a discussion of the various drugs, experimental and otherwise, used in the treatment of idiopathic pulmonary fibrosis.

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Surgical Care

Lung transplantation for idiopathic pulmonary fibrosis (IPF) has been shown to confer a survival benefit over medical therapy. In May 2005, the lung allocation score (LAS) was implemented, which dramatically changed lung allocation from a system based purely on waiting time to an algorithm based on survival probability on the waiting list and after lung transplantation. [42] Therefore, the LAS is used to prioritize patients based on the difference between post-transplant 1-year survival and pretransplant urgency. Consequent to the use of LAS, idiopathic pulmonary fibrosis has now replaced chronic obstructive pulmonary disease as the most common indication for lung transplantation in the United States. [43]

Any patient diagnosed with idiopathic pulmonary fibrosis or probable idiopathic pulmonary fibrosis should be referred for lung transplantation evaluation, regardless of the vital capacity. [8] After a patient is referred for transplantation evaluation, the appropriate timing to list a patient on the lung transplantation list needs to be determined. Guidelines for listing a patient with idiopathic pulmonary fibrosis include a diffusion capacity of carbon monoxide (DLCO) less than 39% predicted, a 10% or greater decrement in forced vital capacity during six months of follow-up, a decrease in pulse oximetry below 88% during a 6-minute walk test (6MWT), or honeycombing on high-resolution computed tomography (HRCT) imaging (fibrosis score >2). [8]

A 2009 retrospective review of the United Network for Organ Sharing data to identify lung transplant recipients with idiopathic pulmonary fibrosis between 2005 and 2007 examined risk for 30-day, 90-day, and 1-year mortality for single lung transplant versus bilateral lung transplant. Data were examined across levels of the LAS (quartile 1, quartile 2, quartile 3, and quartile 4).

Patients in LAS quartile 4 were defined as high risk. A clear inverse relationship between wait-list times and LAS was seen, with a higher LAS score associated with shorter wait-list times. [43] Patients in the LAS quartile 4 had a 7.1% lower cumulative survival at 1 year when compared with patients in quartiles 1 to 3. Just over 21% more patients received bilateral lung transplantation in the highest LAS quartile than in the lowest LAS quartile. In the high-risk quartile, bilateral lung transplantation was associated with a 14.4% decrease in mortality 1 year after lung transplantation. [43] However, this study is limited by the retrospective nature and the need to see if these trends persist at 3 years and 5 years. The reported 5-year survival rates after lung transplantation in idiopathic pulmonary fibrosis are estimated at 50-56%. [1]

Outcomes were published in 2015 comparing single- and double-lung transplantation since the implementation of the Lung Allocation Score. Adults with idiopathic pulmonary fibrosis who underwent lung transplantation between May 04, 2005 and December 31, 2012 were identified in the United Network for Organ Sharing thoracic registry. In total, 4134 patients with idiopathic pulmonary fibrosis underwent lung transplantation. Of these, 2010 patients underwent sing-lung transplantation and 2124 patients underwent double-lung transplantation. After confounders for double-lung transplantation were controlled for with propensity score analysis, double-lung transplant was associated with better graft survival in patients with idiopathic pulmonary fibrosis, with an adjusted median survival of 65.2 months versus 50.4 months in single-lung transplant (P <.001). [44]

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Further Inpatient Care & Transfer

Further inpatient care

The clinical course of patients with idiopathic pulmonary fibrosis (IPF) is generally marked by a decline in pulmonary function over time. Increasingly, patients have been recognized as having an acute, and often fatal, clinical deterioration, termed an acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF).

The following are diagnostic criteria for an AE-IPF: [45]

  • Previous or concurrent diagnosis of idiopathic pulmonary fibrosis
  • Unexplained worsening or development of dyspnea within 30 days
  • High-resolution computed tomography (HRCT) scan with new bilateral ground-glass abnormality and/or consolidation superimposed on a background reticular or honeycomb pattern consistent with a usual interstitial pneumonia pattern
  • Worsening hypoxemia from a known baseline arterial blood gas measurement
  • No evidence of pulmonary infection by endotracheal aspiration or bronchoalveolar lavage (BAL)
  • Exclusion of alternative causes, including left-sided heart failure, pulmonary embolism, and an identifiable cause of acute lung injury

Patients with idiopathic pulmonary fibrosis who develop an acute clinical deterioration often require hospitalization. These patients should undergo HRCT imaging of the chest to document the interval development of significant ground-glass opacities, which are suggestive of an AE-IPF. Additionally, a BAL should be completed to examine the possibility of infectious etiologies. Support with supplemental oxygen should be given to alleviate hypoxemia. [45]

Once infection has been acceptably ruled out and other alternative causes of the acute deterioration have been excluded, treatment with intravenous methylprednisolone (Solu-Medrol) at 2 mg/kg/d for 2 weeks followed by a prolonged taper can be given. [45] However, no randomized controlled trials support a particular therapy for an AE-IPF. If a patient with an AE-IPF develops respiratory failure and requires invasive mechanical ventilation, plateau pressures should be maintained at less than 30 cm water. [45] Patients with idiopathic pulmonary fibrosis who require mechanical ventilation have a poor prognosis.

In a retrospective review, of 461 patients with idiopathic pulmonary fibrosis, 20.8% of all subjects experienced an AE-IPF during the median follow-up period of 22.9 months. Approximately 50% of patients hospitalized for an AE-IPF died during the hospitalization. The 1-year and 5-year survival rates from the initial diagnosis of an AE-IPF were 56.2% and 18.4%, respectively. [46] Therefore, an acute exacerbation of idiopathic pulmonary fibrosis has a serious impact on the overall survival of patients with idiopathic pulmonary fibrosis.

Transfer

Lung transplantation for idiopathic pulmonary fibrosis has been shown to confer a survival benefit over medical therapy. Any patient diagnosed with idiopathic pulmonary fibrosis or probable idiopathic pulmonary fibrosis should be referred to a lung transplantation center for lung transplant evaluation, regardless of the vital capacity unless contraindications for transplantation exist. [8]

Patients with idiopathic pulmonary fibrosis should be referred to institutions where they can be counseled regarding enrollment in a trial of an investigational agent for the treatment of idiopathic pulmonary fibrosis.

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Consultations

Any patient suspected of having idiopathic pulmonary fibrosis, interstitial lung disease, or any another idiopathic interstitial pneumonia should be referred to a pulmonologist for further evaluation and management. Any patient diagnosed with idiopathic pulmonary fibrosis or probable idiopathic pulmonary fibrosis should be referred for lung transplantation evaluation, regardless of the vital capacity unless there are contraindications for transplantation. [8]

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Diet

Any patient with idiopathic pulmonary fibrosis who is overweight should be encouraged to meet with a nutritionist and make dietary changes to achieve ideal body weight. Maintaining adequate nutritional intake is important for quality of life in patients with idiopathic pulmonary fibrosis.

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Activity

Improving quality of life is an important goal in disease management. Deconditioning and subsequent functional impairment is a common problem in patients with idiopathic pulmonary fibrosis and negatively impacts quality of life. Two controlled trials of pulmonary rehabilitation in idiopathic pulmonary fibrosis have demonstrated an improvement in walk distance and symptoms or quality of life. [1] Therefore, patients with idiopathic pulmonary fibrosis should be evaluated for pulmonary rehabilitation and encouraged to participate in regular exercise to maintain a maximal degree of musculoskeletal conditioning. [2]

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Long-Term Monitoring

The rate of decline and progression to death in patients with idiopathic pulmonary fibrosis (IPF) may take several clinical forms, including slow physiologic deterioration with worsening severity of dyspnea, rapid deterioration and progression to death, or periods of relative stability interposed with periods of acute respiratory decline sometimes manifested by hospitalizations for respiratory failure. [10] Therefore, all patients with idiopathic pulmonary fibrosis should be seen by a pulmonologist on a regular basis for a complete history and physical examination. Patients must undergo disease-specific monitoring with serial assessments of lung physiology, gas exchange, exercise performance, and HRCT to further refine prognosis and management decisions. Patients must be asked about adverse medication effects.

Any patient with idiopathic pulmonary fibrosis who is a current smoker should be encouraged to quit and offered pharmacologic therapy if needed.

Vaccination against influenza and pneumococcal infection should be encouraged in all patients with idiopathic pulmonary fibrosis.

Patients with idiopathic pulmonary fibrosis should be evaluated for pulmonary rehabilitation and should be encouraged to participate in regular exercise to maintain a maximal degree of musculoskeletal conditioning.

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