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Diagnosis guide for clinicians

Idiopathic pulmonary fibrosis (IPF): when to suspect it and what to test

Not a doctor or health care professional? This page is for clinicians.Go to the patient version

Idiopathic pulmonary fibrosis is a chronic, progressive fibrosing interstitial pneumonia of unknown cause that occurs mainly in older adults and is limited to the lungs.Source ATS/ERS/JRS/ALAT 2018 guideline

The ATS/ERS/JRS/ALAT guideline says to consider it in adults with unexplained chronic exertional dyspnea, cough, bibasilar inspiratory crackles or digital clubbing.

Source ATS/ERS/JRS/ALAT 2018 guideline

In a Danish cohort of 204 patients, part-funded by an unrestricted Roche grant, the median delay from first symptoms to diagnosis was 2.1 years.

Source Hoyer 2019 Danish delay study
2.1 yearsMedian time from first symptoms to IPF diagnosis in a Danish cohort of 204 patients (IQR 0.9 to 5.0)Hoyer 2019 Danish delay study
41%Patients in that cohort whose respiratory symptoms were first attributed to another diagnosis, most often heart diseaseHoyer 2019 Danish delay study
Over 60Typical age in the guideline's definition of clinically suspected IPF, with bibasilar inspiratory crackles and bilateral fibrosis on imagingATS/ERS/JRS/ALAT 2018 guideline
J84.112ICD-10-CM code for idiopathic pulmonary fibrosisNLM ICD-10-CM lookup
Orphanet recordORPHA:2032 ↗
Inheritance
Multigenic/multifactorial
Age of onset
Adult
Prevalence
  • 1 to 5 per 10,000 point prevalence, Worldwide
  • 1 to 9 per 100,000 annual incidence, Worldwide
  • 1 to 9 per 100,000 point prevalence, United States
Also called
IPF

Live from Orphanet (INSERM) via Orphadata, licensed CC BY 4.0, data version 2026-06-23.

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Patterns to testRed flagsOften mistaken forWhat to testFamily screeningICD-10 codesOnce confirmedFor the patient

Patterns that should prompt a test for Idiopathic pulmonary fibrosis (IPF)

Each sign below is common on its own, which is why Idiopathic pulmonary fibrosis (IPF) is missed. Published studies describe these combinations as the picture to act on. Rows are ordered from the highest published figure to the lowest, within each kind of measure.

Where testing finds it

Share of each screened or biopsied group found to have Idiopathic pulmonary fibrosis (IPF), from the cited studies. A bar spans the lowest to the highest published figure.

  1. 1

    Probable UIP pattern on HRCT+Subpleural, basal reticulation with traction bronchiectasis

    Patients with a probable UIP pattern on HRCT in studies from expert settings, as summarized in the 2022 ATS/ERS/JRS/ALAT guideline

    The guideline puts the likelihood of histologic confirmation of UIP at 80% to 85%, lower in younger people and mild fibrosis (Raghu 2022).ATS/ERS/JRS/ALAT 2022 update

    80% to 85%
  2. 2

    Bilateral velcro crackles on auscultation+Clinically indicated chest HRCT

    People, 148 in all, whose lung sounds were recorded just before a clinically indicated chest HRCT, in a study part-funded by InterMune

    Fibrotic ILD, a wider group than IPF, was present on HRCT in 81.1% of those with bilateral velcro crackles (Sgalla 2018).Sgalla 2018 velcro crackles study

    81.1%
  3. 3

    Possible UIP pattern on HRCT, without honeycombing+Age 60 or older, male sex or more traction bronchiectasis

    Adults with biopsy-proven ILD and nondefinitive HRCT scans at 2 academic ILD centers, 385 in the derivation cohort and 166 in the validation cohort

    Surgical biopsy showed UIP in 62.5% of those with possible UIP on HRCT alone in one cohort and 94.4% in the other, where UIP was more prevalent. Adding age, sex and traction bronchiectasis improved the figure (Brownell 2017).Brownell 2017 HRCT and biopsy study

    62.5% to 94.4%
  4. 4

    First-degree relative with pulmonary fibrosis+Chest CT screening

    Undiagnosed first-degree relatives, 105 in all, of patients with familial pulmonary fibrosis or sporadic IPF who agreed to CT screening

    Interstitial lung abnormalities, which are not the same as IPF, were found in 33 of all 105 relatives (31%), and 19 had further evidence of ILD. Reduced total lung or diffusion capacity carried 9.6 times the odds (Hunninghake 2020).Hunninghake 2020 relatives study

    31%

How often diagnosed patients show it

Share of patients already diagnosed who had the finding, in the group named on each row. Groups differ by age and type, so read each row with its group.

  1. 1

    Velcro crackles on lung auscultation+Fibrosis on chest imaging

    Patients already diagnosed with IPF, including the 191 with auscultation recorded in a Danish cohort and those diagnosed at a Barcelona ILD program

    Velcro crackles were present in 86.4% of the Danish patients (Hoyer 2019) and in every Barcelona patient with a final IPF diagnosis (Sellarés 2016).Hoyer 2019 Danish delay study

    86.4% to 100%
  2. 2

    Respiratory symptoms first attributed to another diagnosis, most often heart disease, asthma or chronic bronchitis

    Incident IPF patients, 204 in all, at 2 Danish ILD centers, in a study part-funded by an unrestricted Roche grant

    Symptoms were first attributed to another diagnosis in 82 of 204 (41%), most often heart disease in 12.3%. Of 61 given inhaled therapy, only 20 (33%) met criteria for asthma or COPD (Hoyer 2019).Hoyer 2019 Danish delay study

    41%
  3. 3

    Chest CT more than 3 years before the diagnosis+Pulmonologist visits and repeated scans before any IPF diagnosis code

    Medicare beneficiaries, 7306 in all, newly diagnosed with IPF in 2012, in a Genentech-funded claims study

    The first chest CT came more than 3 years before the IPF diagnosis in 32.5%, and more than 1 year before in 57.5% (Mooney 2019).Mooney 2019 Medicare study

    32.5%
  4. 4

    Digital clubbing+Fibrosis on chest imaging

    Outpatients with IPF among 153 people with fibrotic ILD at 2 tertiary referral centers

    Clubbing was found in 7% to 52% of those with IPF, depending on which assessment method was used (van Manen 2017).van Manen 2017 clubbing study

    7% to 52%

Also described, without a comparable figure

  • Age 60 or older + Reticulation over one-third or more of the lung on CT + No honeycombing: No observed share is given in the abstract; the model-based probability of IPF was 80% or greater at these thresholds (Salisbury 2016).Salisbury 2016 no-honeycombing study
  • Repeated consultations for breathlessness + Cough consultations in the same years: No percentage is given in the abstract; the odds of a breathlessness consultation 4 to 5 years before diagnosis were 2.79 times those of controls (Hewson 2017).Hewson 2017 UK primary care study

These figures describe published study groups. They are not the chance that any one patient has Idiopathic pulmonary fibrosis (IPF), and bars whose ranges overlap are not in a settled order.

Red flags for Idiopathic pulmonary fibrosis (IPF)

What the cited sources describe, grouped by life stage and organ. Each line links to its source.

Symptoms and history

  • Insidious onset of exertional dyspnea, typically in the sixth and seventh decades, according to the 2018 ATS/ERS/JRS/ALAT guideline.ATS/ERS/JRS/ALAT 2018 guideline
  • A persistent dry, hacking cough with shortness of breath, the most common signs and symptoms listed by MedlinePlus Genetics.MedlinePlus Genetics
  • More men than women are reported with IPF, and the majority of patients have a history of past cigarette smoking.ATS/ERS/JRS/ALAT 2018 guideline
  • In the INTENSITY survey of 600 adults with ILD, 72% first attributed some or all of their respiratory symptoms to advancing age.INTENSITY survey
  • Breathlessness consultations were more likely than in controls even 4 to 5 years before diagnosis in a UK primary care database study.Hewson 2017 UK primary care study

Chest examination and hands

Chest imaging

  • Subpleural, basal predominant reticulation with traction bronchiectasis, with or without honeycombing, the probable UIP and UIP patterns on HRCT.ATS/ERS/JRS/ALAT 2022 update
  • Fine reticulation or architectural distortion seen incidentally on chest or abdominal CT done for other reasons, including lung cancer screening.ATS/ERS/JRS/ALAT 2022 update
  • The 2022 guideline says at least 40% of people with such interstitial lung abnormalities show CT progression over 4 to 6 years.ATS/ERS/JRS/ALAT 2022 update
  • Pure ground-glass opacification is not typical of UIP and, in a patient with IPF, should raise the possibility of an acute exacerbation.ATS/ERS/JRS/ALAT 2018 guideline

Earlier contacts with care

  • Among 7306 Medicare beneficiaries with IPF, 32.5% had a first chest CT more than 3 years before diagnosis, in a Genentech-funded study.Mooney 2019 Medicare study
  • Of 61 Danish patients given inhaled therapy before their IPF diagnosis, only 20 (33%) met diagnostic criteria for asthma or COPD.Hoyer 2019 Danish delay study
  • About half of the Danish cohort, 51%, reported at least 1 pneumonia during the 2 years before their IPF diagnosis.Hoyer 2019 Danish delay study
  • In INTENSITY, which covered all ILD, 30.4% of respondents who first saw a primary care physician reported 4 or more visits before specialist referral.INTENSITY survey

Family history and younger patients

  • Patients under 50 are rare, the guideline says, and may have familial IPF or a connective tissue disease that was still subclinical.ATS/ERS/JRS/ALAT 2018 guideline
  • A family history of ILD is a risk factor, and some genetic forms bring bone marrow failure and liver disease.ATS/ERS/JRS/ALAT 2018 guideline
  • Familial IPF accounts for approximately 5% to 10% of cases and tends to occur at a slightly younger age, according to NORD.NORD

Idiopathic pulmonary fibrosis (IPF) is often mistaken for

Diagnoses patients commonly carry first, and why the sources say they are confused.

First diagnosisWhy it is confused
Heart diseaseHeart disease was the most common alternative diagnosis in the Danish cohort, given to 25 of 204 patients (12.3%) before IPF was found.Hoyer 2019 Danish delay study
AsthmaAsthma was the most common misdiagnosis in the INTENSITY survey, reported by 13.5% of 600 respondents with ILD.INTENSITY survey
COPD, chronic bronchitis or emphysemaIn the Danish cohort, 5.4% were first told chronic bronchitis, 4.9% COPD and 1.5% emphysema before the IPF diagnosis.Hoyer 2019 Danish delay study
Pneumonia or bronchitisPneumonia (13.0%) and bronchitis (12.3%) followed asthma as the most common misdiagnoses reported in the INTENSITY survey of adults with ILD.INTENSITY survey
Aging or deconditioningThe INTENSITY authors note that patients often attribute early symptoms to age or deconditioning, which delays seeking medical attention.INTENSITY survey
Fibrotic hypersensitivity pneumonitisThe 2020 ATS/JRS/ALAT guideline says fibrotic HP can be misdiagnosed as IPF, and no exposure is identified in up to 60% of patients.ATS/JRS/ALAT 2020 HP guideline
Connective tissue disease ILDThe 2022 guideline notes a UIP pattern also occurs in connective tissue disease and is often radiologically indistinguishable from IPF.ATS/ERS/JRS/ALAT 2022 update
Paraseptal emphysema on CTIts subpleural cysts can resemble honeycombing but are typically over 1cm, in a single layer, without reticulation or traction bronchiectasis.ATS/ERS/JRS/ALAT 2022 update

Codes patients often carry before this diagnosis

For spotting the pattern in a problem list or registry. Descriptions are checked daily against NLM's ICD-10-CM service; this is not coding advice.

Other forms of dyspnea, or shortness of breath R06.09, R06.02

Insidious exertional dyspnea is the typical presentation in the 2018 guideline.ATS/ERS/JRS/ALAT 2018 guideline

Chronic cough R05.3

A persistent dry, hacking cough is among the most common symptoms listed by MedlinePlus Genetics.MedlinePlus Genetics

COPD, unspecified, or unspecified asthma, uncomplicated J44.9, J45.909

Only 33% of Danish patients given inhaled therapy before an IPF diagnosis met criteria for asthma or COPD.Hoyer 2019 Danish delay study

Heart failure, unspecified I50.9

Heart disease was the most common alternative diagnosis before IPF in the Danish cohort; the paper does not break it down by type.Hoyer 2019 Danish delay study

Pneumonia, unspecified organism, or bronchitis, not specified as acute or chronic J18.9, J40

Pneumonia and bronchitis were among the 3 most common misdiagnoses in the INTENSITY survey, which covered all ILD.INTENSITY survey

Clubbing of fingers R68.3

Digital clubbing is one of the findings the 2018 guideline says should prompt consideration of IPF.ATS/ERS/JRS/ALAT 2018 guideline

Pulmonary fibrosis, unspecified, or interstitial pulmonary disease, unspecified J84.10, J84.9

The guideline pathway starts from newly detected ILD of apparently unknown cause and then assigns an HRCT pattern.ATS/ERS/JRS/ALAT 2018 guideline

Testing for Idiopathic pulmonary fibrosis (IPF), step by step

The testing order the cited guidance describes, by who it applies to. It summarizes published guidance and is not a plan for any one patient.

Adults with unexplained chronic exertional dyspnea or cough

  1. Listen at the lung bases

    The 2018 guideline says IPF should be considered when these symptoms come with bibasilar inspiratory crackles or digital clubbing and no signs of multisystem disease.ATS/ERS/JRS/ALAT 2018 guideline

Anyone clinically suspected of having IPF

  1. HRCT of the chest

    The guideline's recommendations rest on HRCT patterns. It describes a noncontrast volumetric scan with thin sections of 1.5mm or less, supine inspiratory and expiratory images, and optional prone images.ATS/ERS/JRS/ALAT 2018 guideline

  2. Multidisciplinary discussion

    The 2018 guideline suggests multidisciplinary discussion for diagnostic decision-making, a conditional recommendation.ATS/ERS/JRS/ALAT 2018 guideline

Radiologists reading fibrotic lung disease

  1. Assign 1 of 4 HRCT patterns

    The 2022 update keeps 4 categories: UIP, probable UIP, indeterminate for UIP and findings suggestive of an alternative diagnosis. Honeycombing separates UIP from probable UIP.ATS/ERS/JRS/ALAT 2022 update

Anyone with newly detected ILD of unknown cause

  1. Medication and exposure history

    The 2018 guideline recommends a detailed history of medication use and of environmental exposures at home, work and other places the patient frequently visits.ATS/ERS/JRS/ALAT 2018 guideline

  2. Serologic testing for connective tissue disease

    The 2018 guideline recommends serological testing to exclude connective tissue disease as a potential cause of the ILD.ATS/ERS/JRS/ALAT 2018 guideline

Patients with newly identified ILD whose differential diagnosis includes HP

  1. Look for hypersensitivity pneumonitis

    The 2020 HP guideline advocates a thorough history of exposures known to be associated with HP. It also suggests serum IgG testing against potential antigens, with very low confidence in the evidence.ATS/JRS/ALAT 2020 HP guideline

HRCT pattern of UIP

  1. UIP pattern on HRCT: no biopsy

    The 2018 guideline strongly recommends against surgical lung biopsy, transbronchial biopsy and cryobiopsy, and conditionally against BAL cellular analysis, in these patients.ATS/ERS/JRS/ALAT 2018 guideline

HRCT pattern of probable UIP

  1. Probable UIP pattern

    The 2022 update manages these patients like those with UIP. IPF can be diagnosed after multidisciplinary discussion without biopsy in the appropriate clinical setting.ATS/ERS/JRS/ALAT 2022 update

HRCT indeterminate for UIP or suggesting an alternative diagnosis

  1. BAL and surgical lung biopsy

    The 2018 guideline suggests BAL cellular analysis and surgical lung biopsy when HRCT shows probable UIP, indeterminate for UIP or an alternative diagnosis. Both are conditional recommendations, and the probable UIP step above reflects the 2022 change.ATS/ERS/JRS/ALAT 2018 guideline

ILD of undetermined type, at experienced centers

  1. Transbronchial lung cryobiopsy

    The 2022 update suggests cryobiopsy as an acceptable alternative to surgical biopsy. Its diagnostic yield was 79%, with pneumothorax in 9% and any bleeding in 30%.ATS/ERS/JRS/ALAT 2022 update

ILD of undetermined type having transbronchial forceps biopsy

  1. Genomic classifier

    The 2022 committee made no recommendation for or against genomic classifier testing, because its members did not sufficiently agree.ATS/ERS/JRS/ALAT 2022 update

Patients with affected relatives or features of a genetic form

  1. Family history and genetic testing

    A Pulmonary Fibrosis Foundation work group says genetic testing, in the appropriate setting, allows recognition of extrapulmonary manifestations and assessment of unaffected relatives.PFF genetic testing work group

Where testing goes wrong

  • Earlier inhaled therapy was linked to a longer total diagnostic delay in the Danish cohort (IRR 1.99), but airway obstruction was not.Hoyer 2019 Danish delay study
  • Among Medicare beneficiaries who saw a pulmonologist in the 5 years before diagnosis, 34.7% had that first visit over 3 years before the IPF diagnosis.Mooney 2019 Medicare study
  • Longer delay from dyspnea onset to tertiary center evaluation was linked to higher mortality, independent of forced vital capacity, among 129 adults with IPF.Lamas 2011 delayed access study
  • A UIP pattern is not specific to IPF; the 2022 guideline notes it also occurs in fibrotic HP, connective tissue disease and exposure-related ILD.ATS/ERS/JRS/ALAT 2022 update
  • Agreement between observers on honeycombing is inconsistent, most often because traction bronchiolectasis, paraseptal emphysema or subpleural cysts mimic it.ATS/ERS/JRS/ALAT 2018 guideline
  • Evidence that probable UIP predicts histologic UIP in 80% to 85% comes from expert settings and is weaker in younger people and mild fibrosis.ATS/ERS/JRS/ALAT 2022 update
  • A possible UIP pattern had a PPV for biopsy UIP of 62.5% in one cohort and 94.4% in another where UIP was more prevalent.Brownell 2017 HRCT and biopsy study
  • Absent honeycombing does not exclude IPF; age 60 or more with reticulation in one-third or more of the lung gave a model-based IPF probability of 80% or greater.Salisbury 2016 no-honeycombing study
  • The 2022 guideline says diagnostic confidence may need downgrading when histology comes from cryobiopsy, given smaller samples and greater sampling error.ATS/ERS/JRS/ALAT 2022 update
  • The 2018 guideline strongly recommends against measuring serum MMP-7, SPD, CCL-18 or KL-6 to distinguish IPF from other ILDs.ATS/ERS/JRS/ALAT 2018 guideline
  • Methods of assessing clubbing agreed poorly in a study at 2 tertiary referral centers, and its measured prevalence in IPF ranged from 7% to 52%.van Manen 2017 clubbing study
  • INTENSITY was sponsored by Veracyte, a diagnostics company, with the Pulmonary Fibrosis Foundation collaborating. It relied on self-report, and only 46.5% of respondents had IPF.INTENSITY survey
  • Orphanet records IPF as ORPHA:2032 and maps it to WHO ICD-10 category J84.1 as an inclusion term without its own code.Orphanet cross-references

Where to test

Family screening and referral

Who else in the family

  • MedlinePlus Genetics reports TERT and TERC variants in about 15% of familial pulmonary fibrosis and a smaller percentage of sporadic IPF.MedlinePlus Genetics
  • Familial pulmonary fibrosis appears to follow autosomal dominant inheritance with reduced penetrance, according to MedlinePlus Genetics.MedlinePlus Genetics
  • The 2018 guideline says at least 30% of patients with sporadic or familial pulmonary fibrosis have known genetic predisposing factors.ATS/ERS/JRS/ALAT 2018 guideline
  • In a screening study of 105 undiagnosed first-degree relatives, 33 (31%) had interstitial lung abnormalities and 19 had further evidence of ILD.Hunninghake 2020 relatives study
  • That study found no evidence that prevalence differed between relatives of familial cases and relatives of patients with sporadic IPF.Hunninghake 2020 relatives study
  • Relatives with reduced total lung capacity or diffusion capacity had 9.6 times the odds of interstitial lung abnormalities in that study.Hunninghake 2020 relatives study
  • A 2023 European Respiratory Society statement covers which patients and relatives may benefit from sequencing, telomere length measurement and clinical screening.ERS familial pulmonary fibrosis statement
  • The 2022 guideline lists validating family history and genetics in diagnostic algorithms as a research need, since they might avoid invasive procedures.ATS/ERS/JRS/ALAT 2022 update

Who to refer to

Find a specialist by state

From the CMS NPI Registry, a public directory of US clinicians by registered specialty. A listing is not an endorsement and says nothing about experience with Idiopathic pulmonary fibrosis (IPF); patient organizations and academic centers can often name experienced clinicians.

ICD-10-CM codes for Idiopathic pulmonary fibrosis (IPF)

For orders, referrals and prior authorization. Each code is checked daily against NLM's ICD-10-CM service.

J84.112Idiopathic pulmonary fibrosisCurrent in NLM's ICD-10-CM listNLM ICD-10-CM lookup

Once the diagnosis is confirmed

Idiopathic pulmonary fibrosis (IPF) drugs for prescribers2 prescribing guides, side by side: Esbriet, Jascayd. The page also lists 1 other FDA-approved medicine without a guide yet.Open trials: 106Screen a de-identified patient against every recruiting Idiopathic pulmonary fibrosis (IPF) study on ClinicalTrials.gov.Patient and family pageThe plain-language Idiopathic pulmonary fibrosis (IPF) page, with trials, treatments and help paying.

For the patient: why your doctor ordered this test

Plain language a patient can read or take home.

Your doctor ordered these tests because some findings can be signs of lung scarring. They include breathlessness on exertion, a dry cough that will not go away and crackling sounds at the base of the lungs.

Idiopathic pulmonary fibrosis, or IPF, is one cause of that scarring. It mostly affects people over 60. Its early symptoms look like more common heart and lung problems, which is why it can take time to find.

The main test is a detailed CT scan of the chest. You may also have blood tests and questions about your medicines, your work and home, birds, mold and dust, because other conditions can scar the lungs in a similar way.

Many people do not need a lung biopsy. If scarring is found, lung specialists, radiologists and other doctors or health care professionals often review the results together, and close relatives may be asked about their own breathing.

Questions clinicians ask about Idiopathic pulmonary fibrosis (IPF) testing

Which combinations of findings should prompt testing for idiopathic pulmonary fibrosis (IPF)?

In published screening and biopsy studies, the highest yields came from probable UIP pattern on HRCT plus subpleural, basal reticulation with traction bronchiectasis (80% to 85% of patients with a probable UIP pattern on HRCT in studies from expert settings, as summarized in the 2022 ATS/ERS/JRS/ALAT guideline), and from bilateral velcro crackles on auscultation plus clinically indicated chest HRCT (81.1% of people, 148 in all, whose lung sounds were recorded just before a clinically indicated chest HRCT, in a study part-funded by InterMune). Among patients already diagnosed, the most common combination began with velcro crackles on lung auscultation, seen in 86.4% to 100% of patients already diagnosed with IPF, including the 191 with auscultation recorded in a Danish cohort and those diagnosed at a Barcelona ILD program. These figures describe study groups, not any one patient's chance of having idiopathic pulmonary fibrosis (IPF).

How long does it take to diagnose idiopathic pulmonary fibrosis?

A median of 2.1 years passed from first symptoms to diagnosis in a Danish cohort of 204 patients published in 2019. A cohort of 129 adults found a median of 2.2 years from dyspnea onset to first evaluation at a tertiary care center. In the Danish study, 41% first had their symptoms attributed to another diagnosis, most often heart disease.

Which HRCT patterns allow an IPF diagnosis without lung biopsy?

Either of 2 patterns, UIP or probable UIP, can support the diagnosis without biopsy in the appropriate clinical setting, according to the 2022 ATS/ERS/JRS/ALAT update. The 2018 guideline strongly recommends against surgical biopsy, transbronchial biopsy and cryobiopsy when HRCT shows UIP. The 2022 update cites histologic confirmation of UIP in 80% to 85% of patients with probable UIP.

Which blood tests are part of the IPF workup?

No blood test confirms IPF, and the 2018 guideline strongly recommends against 4 serum biomarkers, MMP-7, SPD, CCL-18 and KL-6, for telling it from other ILDs. The same guideline recommends serological testing to exclude connective tissue disease. The 2020 hypersensitivity pneumonitis guideline suggests serum IgG testing against potential antigens.

Should relatives of a patient with pulmonary fibrosis be screened?

In 1 screening study of 105 undiagnosed first-degree relatives, 31% had interstitial lung abnormalities on CT, and the authors suggest screening might be warranted. MedlinePlus Genetics reports TERT and TERC variants in about 15% of familial pulmonary fibrosis. A 2023 European Respiratory Society statement addresses which relatives are eligible for sequencing and clinical screening.

What is the ICD-10 code for idiopathic pulmonary fibrosis?

The ICD-10-CM code is J84.112, idiopathic pulmonary fibrosis, current in NLM's ICD-10-CM lookup checked November 11, 2026. Pulmonary fibrosis, unspecified is J84.10. Orphanet maps IPF to the broader WHO ICD-10 category J84.1 as an inclusion term.

Sources

  1. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. American Journal of Respiratory and Critical Care Medicine 2018;198(5):e44-e68 (Raghu G, Remy-Jardin M, Myers JL et al for the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society and Latin American Thoracic Society), via Oxford Academic, 2018-09-01.
  2. 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;205(9):e18-e47 (Raghu G, Remy-Jardin M, Richeldi L et al; supported by the 4 societies; many authors report drugmaker ties), via PubMed Central, 2022-05-01.
  3. Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline. American Journal of Respiratory and Critical Care Medicine 2020;202(3):e36-e69 (Raghu G, Remy-Jardin M, Ryerson CJ et al), via PubMed Central, 2020-08-01.
  4. Risk factors for diagnostic delay in idiopathic pulmonary fibrosis. Respiratory Research 2019;20(1):103 (Hoyer N, Prior TS, Bendstrup E, Wilcke T, Shaker SB; supported by 2 Danish foundations and an unrestricted Roche grant), via PubMed Central, 2019-05-24.
  5. Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey. BMC Pulmonary Medicine 2018;18(1):9 (Cosgrove GP, Bianchi P, Danese S, Lederer DJ; sponsored by Veracyte Inc. and conducted in collaboration with the Pulmonary Fibrosis Foundation; 1 author is a Veracyte employee), via PubMed Central, 2018-01-17.
  6. Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study. American Journal of Respiratory and Critical Care Medicine 2011;184(7):842-847 (Lamas DJ, Kawut SM, Bagiella E, Philip N, Arcasoy SM, Lederer DJ), via PubMed abstract; funding and disclosures not seen, 2011-10-01.
  7. Potential Delays in Diagnosis of Idiopathic Pulmonary Fibrosis in Medicare Beneficiaries. Annals of the American Thoracic Society 2019;16(3):393-396 (Mooney J, Chang E, Lalla D et al; financial support from Genentech, with Genentech authors), via PubMed Central, 2019-03.
  8. Timing of onset of symptoms in people with idiopathic pulmonary fibrosis. Thorax, online October 11, 2017 (Hewson T, McKeever TM, Gibson JE, Navaratnam V, Hubbard RB, Hutchinson JP; 2 authors report GSK grants for another study), via PubMed abstract, 2017-10-11.
  9. "Velcro-type" crackles predict specific radiologic features of fibrotic interstitial lung disease. BMC Pulmonary Medicine 2018;18(1):103 (Sgalla G, Walsh SLF, Sverzellati N et al; supported by InterMune Europe and InterMune UK, the Wellcome Trust and the NIHR), via PubMed Central, 2018-06-18.
  10. Auscultation of Velcro Crackles is Associated With Usual Interstitial Pneumonia. Medicine (Baltimore) 2016;95(5):e2573 (Sellarés J, Hernández-González F, Lucena CM et al; supported by SEPAR and IDIBAPS; no conflicts declared), via PubMed Central, 2016-02.
  11. Clubbing in patients with fibrotic interstitial lung diseases. Respiratory Medicine 2017;132:226-231 (van Manen MJG, Vermeer LC, Moor CC et al), via PubMed abstract; funding and disclosures not seen, 2017-11-02.
  12. The use of pretest probability increases the value of high-resolution CT in diagnosing usual interstitial pneumonia. Thorax 2017;72(5):424-429 (Brownell R, Moua T, Henry TS et al; NIH funded), via PubMed Central, 2017-01-12.
  13. Predictors of idiopathic pulmonary fibrosis in absence of radiologic honeycombing: A cross sectional analysis in ILD patients undergoing lung tissue sampling. Respiratory Medicine 2016;118:88-95 (Salisbury ML, Xia M, Murray S et al; data from the NHLBI-supported Lung Tissue Research Consortium), via PubMed abstract, 2016-07-28.
  14. Interstitial Lung Disease in Relatives of Patients with Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine 2020;201(10):1240-1248 (Hunninghake GM, Quesada-Arias LD, Carmichael NE et al), via PubMed abstract; funding and disclosures not seen, 2020-05-15.
  15. The Role of Genetic Testing in Pulmonary Fibrosis: A Perspective From the Pulmonary Fibrosis Foundation Genetic Testing Work Group. Chest 2022;162(2):394-405 (Newton CA, Oldham JM, Applegate C et al; commissioned by the Pulmonary Fibrosis Foundation), via PubMed abstract; funding and disclosures not seen, 2022-03-23.
  16. European Respiratory Society statement on familial pulmonary fibrosis. European Respiratory Journal 2023;61(3):2201383 (Borie R, Kannengiesser C, Antoniou K et al; several authors report drugmaker fees or grants outside the work), via PubMed abstract, 2023-03-16.
  17. Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. Journal of Heart and Lung Transplantation 2021;40(11):1349-1379 (Leard LE, Holm AM, Valapour M et al), via PubMed Central, 2021-07-24.
  18. Idiopathic pulmonary fibrosis: MedlinePlus Genetics. National Library of Medicine, last updated July 1, 2020, 2020-07-01.
  19. Idiopathic Pulmonary Fibrosis - Symptoms, Causes, Treatment | NORD. National Organization for Rare Disorders, last updated January 9, 2017, 2017-01-09.
  20. Idiopathic pulmonary fibrosis, ORPHA:2032, rare disease cross-referencing (Orphadata API). Orphanet, INSERM, via Orphadata, CC BY 4.0, 2026-06-23.
  21. NIH Genetic Testing Registry tests for idiopathic pulmonary fibrosis, concept C1800706 (7 clinical tests listed in NCBI E-utilities on November 11, 2026). National Center for Biotechnology Information, NIH, 2026-11-11.
  22. ICD-10-CM search results for "J84.112" (Clinical Table Search Service). National Library of Medicine, 2026-11-11.

A reference for licensed professionals, checked against its sources on November 11, 2026. It summarizes what the cited guidance says and does not tell anyone how to diagnose or treat a specific patient. Guidelines change; the current guideline and the treating clinician's judgment govern. Trial Friend is an independent information service, not a medical provider.