Key Points
- Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause occurring in adults.
- Radiologic and/or histopathologic patterns are consistent with usual interstitial pneumonia.
- Significant advances have been recently made in the clinical management of IPF.
- All of the recommendations in favor of treatment are conditional, meaning that decisions to treat patients with IPF should be individualized. Clinicians should be cautious to not interpret recommendations with the same grade as having the same net effects.
- The recommendations in favor of treatment put a higher value on the potential benefit of patient-important outcomes such as disease progression as measured by FVC decline and mortality and a lower value on potentially significant adverse effects and the cost of treatment.
Treatment
Table 1. Recommended Drugsa
| Brand | Generic | How Supplied | Dose |
|---|---|---|---|
| Esbriet® | Pirfenidone | 267 mg capsules | With food: Day 1-7: 1 capsule tid Day 8-14: 2 capsules tid Day 15 onward: 3 capsules tid |
| OFEV® | Nintedanib | 150 mg capsules 100 mg capsules | With food: 150 mg capsule q12h (Temporary interruption or reduction to 100 mg bid for adverse effects) |
Table 2. Recommendations
| For patients with IPF, the guideline committee recommends: | |||
|---|---|---|---|
| Quality of Evidence | Strength of Recommendation | Confidence in Estimates | |
| Clinicians NOT use imatinib. | ⊕⊕⊕⊖ | Strong | Moderate |
| Clinicians NOT use warfarin anticoagulation in patients who do not have a known alternative indication for its use. | ⊕⊕⊖⊖ | Strong | Low |
| Clinicians NOT use the combination therapy of N-acetylcysteine, azathioprine, and prednisone. | ⊕⊕⊖⊖ | Strong | Low |
| Clinicians NOT use ambrisentan, a selective ER-A endothelin receptor antagonist, regardless of the presence or absence of PH. | ⊕⊕⊖⊖ | Strong | Low |
| For patients with IPF, the guideline committee suggests: | |||
|---|---|---|---|
| Quality of Evidence | Strength of Recommendation | Confidence in Estimates | |
| Clinicians use nintedanib. | ⊕⊕⊕⊖ | Conditional | Moderate |
| Clinicians use pirfenidone. | ⊕⊕⊕⊖ | Conditional | Moderate |
| Clinicians NOT use sildenafil, a phosphodiesterase-5 inhibitor. | ⊕⊕⊕⊖ | Conditional | Moderate |
| Clinicians NOT use bosentan or macitentan, both dual ER-A and ER-B endothelin receptor antagonists. | ⊕⊕⊖⊖ | Conditional | Low |
| Clinicians NOT use N-acetylcysteine monotherapy. | ⊕⊕⊖⊖ | Conditional | Low |
| Clinicians use regular anti-acid treatment. | ⊕⊖⊖⊖ | Conditional | Very Low |
Table 3. Quality of the Evidence (GRADE) and Implications
| Quality of Evidence | Symbol | Implications |
|---|---|---|
| High | ⊕⊕⊕⊕ | Further research is very unlikely to change confidence in the estimate of effect. |
| Moderate | ⊕⊕⊕⊖ | Further research is likely to have an important impact on confidence in the estimate of effect and may change the estimate. |
| Low | ⊕⊕⊖⊖ | Further research is very likely to have an important impact on confidence in the estimate of effect and is likely to change the estimate. |
| Very low | ⊕⊖⊖⊖ | The guideline committee is very uncertain about the estimate. |
Abbreviations
IPF, idiopathic pulmonary fibrosis; q12h, every 12 hours; tid, 3 times a day
Source
An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis: an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192:
e3–e19. Available at: http://www.atsjournals.org/doi/abs/10.1164/rccm.201506-1063ST
The guidelines were a cooperative effort among the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society.
Disclaimer
This Guideline attempts to define principles of practice that should produce high-quality patient care. It is applicable to specialists, primary care, and providers at all levels. This Guideline should not be considered exclusive of other methods of care reasonably directed at obtaining the same results. The ultimate judgment concerning the propriety of any course of conduct must be made by the clinician after consideration of each individual patient situation.
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