Idiopathic pulmonary fibrosis (IPF): clinical guidance and practice resources

Pulmonologist showing CT scan of lungs to a patient with pulmonary fibrosis.

Learn how to spot the symptoms of this rare lung disease and help your patients get treatment.

As frontline health care providers, family physicians play an essential role in the early detection of idiopathic pulmonary fibrosis (IPF) and the timely referral to a pulmonologist. The disease is rare and includes signs and symptoms that make it difficult to distinguish among other interstitial lung diseases (ILDs).

By identifying suspected cases of IPF at primary care visits, you can refer patients earlier and enable diagnosis and treatment sooner. This makes education about IPF a key factor in early detection, which can potentially lead to better health outcomes.The diagnostic criteria and treatment options below are based on the 2022 ATS/ERS/JRS/ALAT Clinical Practice Guideline, which represents the most current international consensus on IPF diagnosis and management, with pharmacotherapy updates through 2025.28 This content has not been reviewed or endorsed by the American Academy of Family Physicians (AAFP). However, given the limited guidance on IPF, we present this information for consideration in management of the disease.

What is idiopathic pulmonary fibrosis?

IPF is a specific form of chronic, progressive fibrosing interstitial lung disease (ILD) of unknown cause.1 ILDs may be a result of a number of insults to the lungs (e.g., medication, connective tissue disease, occupational or environmental exposures).2

Common symptoms

IPF is characterized by a progressive breathlessness and cough, as well as a decline in lung function.1

The most common symptoms of IPF are dyspnea and cough.2 Dyspnea is usually exertional and associated with walking up inclines or steps.2 The cough is typically described as “dry” and “hacking,” and may start with a tickle in the throat.3 The severity of these symptoms varies.

Other possible symptoms of IPF are fatigue and problems with sleeping.3 Symptoms that have not been associated with IPF include chest pain, fever, rash, weight loss and myalgia or arthralgia,4 although these may be seen in various other forms of ILD.

Knowledge of a patient’s medical history and exposures is vital to diagnosing IPF and essential to excluding other ILDs. Questions should focus on the following:

  • Smoking history. Cigarette smoking is strongly associated with IPF, especially individuals with a history of more than 20 pack-years.2,4
  • Other medical conditions. Gastroesophageal reflux disease, hiatal hernia, pulmonary malignancy, coronary artery disease, obstructive sleep apnea, obesity, emphysema and pulmonary hypertension are comorbid conditions frequently associated with IPF.2,4
  • Occupational and environmental exposures. Chronic, repeated exposure to metal dusts (brass, lead and steel), wood dust (pine) and aerosolized organic antigens (primarily molds, bacteria, and bird antigens) have been associated with IPF. Relevant occupations with associated exposures include farming, raising birds, hair dressing and stone cutting and/or polishing.2,4
  • Medication history. Some medications may have pulmonary fibrosis as a potential toxicity.5

  • The physical examination should focus on two key signs:
  • Inspiratory crackles. This sign may be the earliest clinical finding and is the hallmark feature of IPF, reported in more than 90% of patients.4 These crackles sound like the ripping apart of Velcro and are heard at the posterolateral, basal aspects of the lungs.3 It is best to listen with the stethoscope applied directly to the skin.3 Inspiratory squeaks and wheezes are uncommon in IPF, and should prompt consideration of other diagnoses.6
  • Finger clubbing. This sign occurs in 25% to 50% of patients.4

Idiopathic pulmonary fibrosis is unlikely in the presence of signs of connective tissue disease, such as joint deformity, synovitis, muscle weakness and rash.4 Instead, these signs should prompt a workup for rheumatologic disease.4

Causes and risk factors

The cause of IPF is unknown. It was believed IPF was caused by generalized inflammation that progressed to widespread parenchymal fibrosis.13,14 This was questioned when IPF failed to respond to anti-inflammatory drugs and immune modulators.13 Studies now suggest exposure to external stimuli (e.g., smoke, environmental agents) can lead to damage of alveolar epithelial cells, subsequent activation of mesenchymal cells and excess accumulation of extracellular matrix.13,14 A genetic basis for IPF is still being explored.

IPF is rare and a lack of large-scale studies makes it difficult to estimate the incidence of the disease. We do know the incidence of IPF increases with age and some patients are at higher risk, including those who:

  • Are older than 557

  • Are male7

  • Have a history of smoking4

  • Have been or are currently exposed to occupational or environmental antigens4

Among all individuals aged 55 to 64, the incidence is 19.3 cases per 100,000 person-years.7

Patients with IPF are at an increased risk for several comorbidities, including coronary artery disease, lung cancer, obstructive sleep apnea, emphysema, pulmonary hypertension, pulmonary infection, gastroesophageal reflux disease, hiatal hernia and diabetes mellitus.2,4,15

Diagnosis

Diagnosing IPF is challenging and an exercise in ruling things out. No single clinical factor indicates IPF. Rather, a patient’s entire clinical context should be considered when making a diagnosis.

Misdiagnosis and delays in diagnosis of IPF are common. In one study, IPF was most often misdiagnosed as asthma (13.5%), pneumonia (13.0%) or bronchitis (12.3%).8 Delays in diagnosis have been reported to be from one year to as long as three years, with longer delays associated with an increased risk of death.4,7

IPF is difficult to diagnose for several reasons, including:2

  • Symptoms (breathlessness on exertion or at rest, and a dry cough) are nonspecific

  • Clinical presentation is similar to that of many other pulmonary diseases

  • Many interstitial lung diseases may mimic IPF

  • Have been or are currently exposed to occupational or environmental antigens4

  • No biomarkers of the disease are available

Diagnostic criteria and medical tests

Current standards for IPF diagnosis include: a multidisciplinary discussion (MDD) among pulmonologists, radiologists, and pathologists discussing all features of the patient’s presentation, radiographic findings and pathologic findings (if available). The discussion should address the following:9

  • Exclusion of other known causes of interstitial lung disease (e.g., domestic and occupational environmental exposures, connective tissue disease and drug toxicity)

  • Presence of usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) in patients who do not have a surgical lung biopsy

  • Specific combinations of HRCT and surgical lung biopsy pattern (in patients who have surgical lung biopsy)

MDD enhances the accuracy of diagnosis, with family physicians playing a vital role through early detection and timely referral. Excluding other causes of disease relies on recognizing the signs and symptoms of the disease, as well as documenting risk factors9—taking a detailed, comprehensive history, carefully performing a physical examination and ordering appropriate diagnostic testing. Referrals should include a complete documentation of findings.10,11 Diagnosing IPF requires not only knowledge of the signs and symptoms, but also the ability to distinguish it from other diseases with similar clinical presentations.

Most objective testing is not part of the recommended diagnostic criteria. Nevertheless, the results of some tests can help exclude other diagnoses and/or add to the clinical context of IPF.

Although laboratory testing is not useful in diagnosing IPF, guidelines recommend serologic testing for most patients to exclude underlying connective tissue disease.9 Such testing may include rheumatoid factor, anti-cyclic citrullinated peptide, and anti-nuclear antibodies.9 An extractable nuclear antigen panel is often helpful to identify other connective tissue diseases, such as Sjögren’s syndrome, systemic lupus erythematosus or scleroderma, all of which are also associated with ILDs.12

The sensitivity of chest radiographs for the detection of subtle interstitial changes is low.4 An indication of IPF is symmetric peripheral, basilar reticular opacity with loss of volume in the lower lobe.4 However, some patients with IPF may have normal findings on chest radiographs.4

Pulmonary function tests are integral to monitoring progression of IPF and staging of disease severity.4 The results may also be helpful in establishing an initial diagnosis of IPF. The forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide are usually decreased, but these values may be normal early in the disease course.4

The radiographic standard for the diagnosis of IPF is an HRCT of the chest.9 An HRCT is a special, non-contrast chest computed tomography (CT) that obtains thin slice (< 2 millimeters), volumetric images of the lungs enhanced with special software algorithms. A pattern of UIP on HRCT is characterized by a subpleural, basilar predominance and honeycombing with or without peripheral traction bronchiectasis or bronchiolectasis with a relative paucity of ground-glass opacities.4,9

HRCT findings are categorized as UIP, probable UIP, indeterminate, or alternative diagnosis. A UIP or probable UIP pattern can support IPF diagnosis after multidisciplinary discussion without biopsy when clinical features suggest high pre-test probability—specifically males over age 60 with a history of smoking and absence of features suggesting connective tissue disease or hypersensitivity pneumonitis. Tissue sampling is typically required only for indeterminate patterns, though the decision should be individualized based on surgical risk.32

It is essential for HRCT images to be interpreted by a radiologist experienced with ILDs.

When to Consider IPF

A diagnosis of IPF should be considered for all patients with unexplained chronic exertional dyspnea, and those who present with a cough, bibasilar inspiratory crackles and finger clubbing.9 The disease most often occurs in individuals older than 50, men, and smokers.4,9 The index of suspicion for connective tissue disease should be high for women younger than 60 years.9

An HRCT should be ordered for any patient who has abnormal findings on chest radiographs and clinical findings that are consistent with an ILD.4

A complete report of the family physician’s findings is an important aspect of a referral to confirm IPF. A thorough referral report should include the following:11

  • Symptoms and their duration

  • Smoking history

  • Comorbidities

  • Family history

  • Medical history

  • Occupational history

  • Environmental exposures, including hobbies, pets and other exposures outside of work

  • Results of physical examination, primarily the presence of inspiratory crackles and finger clubbing

  • Findings on chest radiographs

  • Description of previous treatments

  • Results of pulmonary function tests

  • Results of HRCT (plus images), if available

Pulmonary function tests are integral to monitoring progression of IPF and staging of disease severity. The results may also be helpful in establishing an initial diagnosis of IPF.

Management and treatment options

The goals of IPF treatment are to slow progression of the disease, reduce symptoms and improve quality of life. IPF is currently treated with a combination of antifibrotic drugs and pulmonary rehabilitation.2,4,17 The need for oxygen therapy should be assessed and lung transplantation is an option for moderate to severe disease in select patients.2,4 Clinical trials and registries may be available for patient involvement in your area.

Current treatments for IPF

Until 2014, no approved drugs were available for the treatment of IPF. But there now are 3 FDA-approved IPF drugs:

  • Pirfenidone (Esbriet)

  • Nintedanib (Ofev)

  • Nerandomilast (Jascayd)

Antifibrotic therapy should be initiated promptly at diagnosis without delay, as IPF is irreversibly progressive from the moment of diagnosis and a window of opportunity may exist during which treatment can have optimal outcomes. All three FDA-approved antifibrotic agents reduce disease progression and mortality, but require ongoing monitoring for liver toxicity and management of gastrointestinal side effects to maintain adherence. 28,29,30

Antifibrotic therapy is not limited to IPF. Progressive Pulmonary Fibrosis (PPF) is a term introduced in the 2022 guidelines for patients with fibrosing interstitial lung diseases other than IPF who demonstrate disease progression despite appropriate management. PPF is defined as meeting at least 2 of 3 criteria within one year:

  • Worsening respiratory symptoms

  • Physiological progression (FVC decline ≥5% or DLCO decline ≥10%)

  • Radiological progression on HRC

Patients with PPF may benefit from antifibrotic therapy. Nintedanib has a conditional recommendation for PPF, and nerandomilast was FDA-approved for PPF in December 2025.28

A systematic review of nine studies demonstrated that pulmonary rehabilitation is beneficial for people with interstitial lung disease, including IPF.18According to the findings, pulmonary rehabilitation was safe and was associated with short-term improvements in functional exercise capacity, dyspnea and quality of life.18

A more recent systematic review and meta-analysis (five randomized controlled trials) focused on only IPF and showed that pulmonary rehabilitation was associated with increased exercise tolerance and improved quality of life.19

Patients derive the most benefit from pulmonary rehabilitation early in the course of the disease.20 As such, pulmonary rehabilitation should begin immediately after diagnosis.

Patients with IPF should be assessed for the need for oxygen therapy. This is best accomplished by a six-minute walk study. If the patient’s oxygen saturation drops below 88%, then an oxygen titration component should be performed.2 This can help determine the least amount of oxygen necessary to maintain saturations above 88% with exertion.2

Most specialty centers advocate for management of comorbidities, though specific guidance varies by condition. Antacid medications (PPIs, H2 blockers) should not be prescribed for the purpose of improving IPF respiratory outcomes; the 2022 guidelines issued a conditional recommendation against antacid therapy for IPF treatment based on lack of evidence for respiratory benefit. However, antacids may still be appropriate for managing symptomatic GERD according to GERD-specific guidelines. Similarly, antireflux surgery should not be performed for the purpose of improving IPF respiratory outcomes.28

While the number of single-lung transplantations has remained steady, the number of double-lung transplantations has increased since the mid-1990s.22 There is no evidence showing the benefit of single- versus double-lung transplantation.17

  • FVC <80% predicted or DLCO <40% predicted

  • Significant FVC decline (≥5% over 6 months)

  • Oxygen desaturation <88% on 6-minute walk test

  • Honeycombing on HRCT28

Lung transplantation is the only treatment associated with prolonged survival in IPF. Patients at increased risk of mortality should be referred for transplant evaluation at the time of diagnosis, not delayed until moderate-to-severe disease. Early referral allows adequate time for evaluation and waitlisting. Consider referral for patients with:

Living with IPF

The course of IPF is unpredictable, and many people experience acute exacerbations of the disease. In one study, 72% of 1,735 patients with IPF sought urgent, outpatient care because of a suspected exacerbation of the disease, and 39% of the patients had at least one all-cause hospitalization.16

IPF has a substantial impact on health-related quality of life, primarily attributed to a high-symptom burden and functional limitations. People with IPF have reported that the most troublesome symptoms are dyspnea, severe coughing and persistent fatigue.23 Limited mobility is also an issue.23 One study reported that individuals with IPF were sedentary for more than nine hours per day.24 People with IPF become frustrated as they lose the ability to engage in activities they once enjoyed.23

Difficulty interacting with friends and family and the financial strain are also primary challenges.23 Depression is common and is also a substantial factor of health-related quality of life.25-27 In one study, nearly 26% of patients with IPF experienced symptoms of depression.27

In addition to their vital role in early detection and referral, family physicians play an important role in the ongoing care of their patients with IPF. Family physicians can contribute to the care of patients with IPF in the following ways:

  • Oversee the treatment of comorbidities

  • Encourage participation in pulmonary rehabilitation and monitor progress

  • Vaccinate against influenza, pneumococcus and pertussis

  • Assess emotional and mental health

  • Recommend support groups for patients and their caregivers

  • Monitor the need for oxygen therapy

  • Discuss treatment preferences and end-of-life care

Patients value a trusted source of information and may ask their family physician for information and advice. Family physicians should provide their patients with guidance for self-management of their disease and recommend credible resources for patient education.

Monitoring and recognizing disease progression

Pulmonology typically directs the IPF monitoring schedule, which includes spirometry every 3–4 months and DLCO measurement every 6–12 months. Family physicians play a key role in recognizing interval changes that warrant earlier reassessment. Contact the patient's pulmonologist or consider urgent referral for:

  • FVC decline ≥5–10% from base

  • Worsening dyspnea or exercise tolerance

  • New oxygen requirement or increased supplemental oxygen needs

  • Acute respiratory deterioration (may indicate acute exacerbation, which is a life threatening complication) 28

IPF quiz: Test your knowledge

Comparing breath sounds

Take the IPF quiz

Answer key below

IPF quiz answer key

1. The breath sounds associated with IPF are

B. Crackles

Related resources for family physicians

Produced in collaboration with Boehringer Ingelheim Pharmaceuticals, Inc. PC-US-110950

  1. Torrisi SE, Pavone M, Vancheri A, Vancheri C. When to start and when to stop antifibrotictherapies. Eur Respir. Rev. 2017;26(145).
  2. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
  3. National Jewish Health. PeerView Institute for Medical Education. Idiopathic pulmonar fibrosis: a guide for providers. Accessed July 26, 2018.
  4. Oldham JM, Noth I. Idiopathic pulmonary fibrosis: early detection and referral. Respir Med. 2014;108(6):819-829.
  5. Schwaiblmair M, Behr W, Haeckel T, Markl B, Foerg W, Berghaus T. Drug induced interstitial lung disease. Open Respir Med J. 2012;6:63-74.
  6. Antin-Ozerkis D. Interstitial lung disease: a clinical overview and general approach. Thoracic key. Chapter 54. Section 11. Accessed July 26, 2018.
  7. Raghu G, Chen SY, Hou Q, Yeh WS, Collard HR. Incidence and prevalence of idiopathic pulmonary fibrosis in US adults 18-64 years old. Eur Respir J. 2016;48(1):179-186.
  8. Cosgrove GP, Bianchi P, Danese S, Lederer DJ. Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey. BMC Pulm Med. 2018;18(1):9.
  9. Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198(5):e44-e68.
  10. Lamas DJ, Kawut SM, Bagiella E, Philip N, Arcasory SM, Lederer DJ. Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study. Am J Respir Crit Care Med. 2011;184(7):842-847.
  11. Purokivi M, Hodgson U, Myllarniemi M, Salomaa ER, Kaarteenaho R. Are physicians in primary health care able to recognize pulmonary fibrosis? Eur Clin Respir J. 2017;4(1):1290339.
  12. Hiepe F, Dorner T, Burmester G. Antinuclear antibody- and extractable nuclear antigen-related diseases. Int Arch Allergy Immunol. 2000;123(1):5-9.
  13. Harari S, Caminati A. IPF: new insight on pathogenesis and treatment. Allergy. 2010;65(5):537-553.
  14. Sgalla G, Iovene B, Calvello M, Ori M, Varone F, Richeldi L. Idiopathic pulmonary fibrosis: pathogenesis and management. Respir Res. 2018;19(1):32.
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  16. Yu YF, Wu N, Chuang CC, et al. Patterns and economic burden of hospitalizations and exacerbations among patients diagnosed with idiopathic pulmonary fibrosis. J Manag Care Spec Pharm. 2016;22(4):414-423.
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  18. Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2014;10:CD006322.
  19. Gomes-Neto M, Silva CM, Ezequiel D, Conceicao CS, Saquetto M, Machado AS. Impact of pulmonary rehabilitation on exercise tolerance and quality of life in patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis. J Cardiopulm Rehabil Prev. 2018;Jan 18 [Epub ahead of print].
  20. Holland AE, Hill CJ, Glaspole I, Goh N, McDonald CF. Predictors of benefit following pulmonary rehabilitation for interstitial lung disease. Respir Med. 2012;106(3):429-435.
  21. Thabut G, Mal H, Castier Y, et al. Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis. J Thorac Cardiovasc Surg. 2003;126(2):469-475.
  22. Christie JD, Edwards LB, Kucheryavaya AY, et al. The registry of the International Society for Heart and Lung Transplantation: 29th adult lung and heart-lung transplant report—2012. J Heart Lung Transplant. 2012;31(10):1073-1086.
  23. Center for Drug Evaluation and Research. The voice of the patient. Idiopathic pulmonary fibrosis. U.S. Food and Drug Administration. Washington, DC: 2015. https://www.fda.gov/downloads/ForIndustry/UserFees/PrescriptionDrugUserFee/UCM440829.pdf. Accessed April 18, 2018.
  24. Atkins C, Baxer M, Jones A, Wilson A. Measuring sedentary behaviors in patients with idiopathic pulmonary fibrosis using wrist-worn accelerometers. Clin Respir J. 2018;12(2):746-753.
  25. Glaspole IN, Chapman SA, Cooper WA, et al. Health-related quality of life in idiopathic pulmonary fibrosis: data from the Australian IPF registry. Respirology. 2017;22(5):950-956.
  26. Matsuda T, Taniguchi H, Ando M, et al. Depression is significantly associated with health status in patients with idiopathic pulmonary fibrosis. Intern Med. 2017;56(13):1637-1644.
  27. Lee YJ, Choi SM, Lee YJ, et al. Clinical impact of depression and anxiety in patients with idiopathic pulmonary fibrosis. PLoS One. 2017;12(9):e0184300.
  28. Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022;205(9):e18-e47.
  29. Interstitial Lung Diseases. Lancet. 2022. Wijsenbeek M, Suzuki A, Maher TM.
  30. Focus on Idiopathic Pulmonary Fibrosis: Advancing Approaches to Diagnosis, Prognosis, and Treatment. Chest. 2018. Martinez FJ, Lederer DJ.
  31. Nerandomilast in Patients with Idiopathic Pulmonary Fibrosis. The New England Journal of Medicine. 2025. Richeldi L, Azuma A, Cottin V, et al.
  32. Idiopathic Pulmonary Fibrosis (An Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/­ERS/­JRS/­ALAT Clinical Practice Guideline. American Journal of Respiratory and Critical Care Medicine. 2022. Raghu G, Remy-Jardin M, Richeldi L, et al.

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