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Review ArticleClinical Review

Is It Idiopathic Pulmonary Fibrosis or Not?

Mary Salvatore, Genta Ishikawa and Maria Padilla
The Journal of the American Board of Family Medicine January 2018, 31 (1) 151-162; DOI: https://doi.org/10.3122/jabfm.2018.01.170288
Mary Salvatore
From the Department of Radiology, Icahn School of Medicine at Mount Sinai, New York, NY (MS); Department of Pulmonary Medicine, Icahn School of Medicine at Mount Sinai, New York (GI, MP).
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Genta Ishikawa
From the Department of Radiology, Icahn School of Medicine at Mount Sinai, New York, NY (MS); Department of Pulmonary Medicine, Icahn School of Medicine at Mount Sinai, New York (GI, MP).
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Maria Padilla
From the Department of Radiology, Icahn School of Medicine at Mount Sinai, New York, NY (MS); Department of Pulmonary Medicine, Icahn School of Medicine at Mount Sinai, New York (GI, MP).
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Article Figures & Data

Figures

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  • Figure 1.
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    Figure 1.

    A “Usual interstitial pneumonitis (UIP) pattern” radiographically was defined by American Thoracic Society as subpleural basilar predominant fibrosis, reticulations, honeycombing, and absence of features that would support an alternate diagnosis.

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    Figure 2.

    If there is no honeycombing but other criteria for a usual interstitial pneumonitis (UIP) pattern are met, the diagnosis is a “possible UIP pattern” radiographically as per the American Thoracic Society (ATS) criteria.

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    Figure 3.

    Features that would suggest an alternative diagnosis include consolidation as can be seen in organizing pneumonia, which follows the bronchovascular bundles in this example.

  • Figure 4.
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    Figure 4.

    Cellular nonspecific interstitial pneumonitis (NSIP) is associated with ground glass opacities and has minimal volume loss. Cellular NSIP is more likely to respond to steroid treatment.

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    Figure 5.

    Fibrotic nonspecific interstitial pneumonitis (NSIP) has less ground glass opacity and more volume loss then cellular NSIP. The dilated esophagus in this photograph points to the cause of fibrosis, which was scleroderma.

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    Figure 6.

    Chronic hypersensitivity pneumonitis (CHP) is upper-lobe predominant, airway centered, and frequently has air trapping. The most useful feature radiographically is its airway-centered distribution seen on this image.

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    Figure 7.

    Stage 4 sarcoidosis is an upper-lobe predominant fibrosis, which helps to differentiate it from usual interstitial pneumonitis (UIP). In addition, it is not peripheral but instead is airway centered.

Tables

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    Table 1.

    Serologies and Their Implications in Workup of Lung Fibrosis

    Lab TestImplications When Elevated
    Anti-nuclear antibodyCollagen vascular diseases
    Rheumatoid factorRheumatoid arthritis
    Anti-Scl-70 antibodySystemic scleroderma
    Anti-centromere antibodyLimited cutaneous scleroderma
    Anti-synthetase antibodyDermatomyositis
    Polymyositis
    Anti-synthetase syndrome
    Anti-Ro (SS-A) antibodySjögren syndrome
    Lupus erythematosus
    Anti-La (SS-B) antibodySjögren syndrome
    Lupus erythematosus
    AntiribonucleoproteinMixed connective-tissue disease LUPUS erythematosus
    AldolaseDermatomyositis
    Polymyositis
    Creatine kinaseDermatomyositis
    Polymyositis
    Erythrocyte sedimentation rateCollagen vascular diseases
    Anti-cyclic citrullinated peptideRheumatoid arthritis
    • View popup
    Table 2.

    Overview of Common Fibrotic Lung Diseases

    PulmonaryRadiologyTreatment
    UIPOlder ageSubpleuralAnti fibrotic medications
    Male sexBasilar predominantConsider referral for lung transplant soon after diagnosis as course is unpredictable and inexorable
    Smoking historyHoneycombingAnti-inflammatory and immunosuppressive Rx NOT indicated (may be harmful)
    Crackles at lung bases
    Clubbing
    NSIPYounger ageHomogeneousAnti-inflammatory medications
    Female sexBronchovascularConsider referral for lung transplant if advanced disease
    Connective tissue diseaseLower lobe
    Positive serologiesDilated esophagus
    Peripheral sparring
    CHPOlder agePeribronchiolar fibrosisAnti-inflammatory medications
    FemaleAir trappingRemoval of causative antigen
    Bird ownerUpper lobeConsider referral for lung transplant if advanced disease
    Mold exposure
    S4Younger ageUpper lobe posteriorAnti-inflammatory medications
    Less symptomaticCalcified lymph nodesConsider transplantation for advanced disease (PHN, hypoxemia, declining PFT's)
    Peribronchiolar fibrosis
    • CHP, chronic hypersensitivity pneumonitis; NSIP, non-specific interstitial pneumonitis; S4, stage 4 sarcoidosis; PFT, Pulmonary Function Test; PHN, pulmonary hypertension; UIP, usual interstitial pneumonitis.

    • View popup
    Table 3.

    Putting It All Together: Typical Scenarios for Patients with Lung Fibrosis

    IPFNSIPCHPS4
    Clinical presentation74-year-old male with history of smoking, cough, and progressive DOE45-year-old female with history of connective tissue disease and shortness of breath68-year-old female. male with shortness of breath and parakeet/parrot (hobbies/occupations)30-year-old male with cough, DOE
    Absent history of smoking
    ExamsCrackles at lung bases on physical examEvidence of rash on extensor surfaces.Squeaks and airway sounds as well as rales testing for sensitivity to antigens causing fibrosisRestrictive, obstructive or mixed pulmonary function tests
    Digital clubbingCapillary testing of fingersRestrictive pulmonary function tests with decreased DLCOHx of sarcoidosis or evidence of granulomatous inflammation and multisystemic involvement
    Restrictive pulmonary function tests with decreased DLCORestrictive pulmonary function tests with decreased DLCO
    RadiologySubpleural basilar predominant fibrosis with honeycombing compatible with ATS criteria for a UIP patternLower lobe fibrosis which follows the bronchovascular bundles and is homogeneous. Sometimes there is peripheral sparringHeterogeneous fibrosis that follows the bronchovascular bundles with slight upper lobe predominanceUpper lobe posterior fibrosis sometimes with calcified mediastinal lymph nodes
    There is usually air trapping
    TreatmentAnti-fibrotic medicationAnti-inflammatory medicationsAnti-inflammatory medicationsAnti-inflammatory medications
    Pulmonary rehabilitationRheumatology consultRemoval of antigen causing disease if known
    Treat esophageal reflux disease
    Role of transplantReferral for transplant evaluation early after diagnosis or for progressive diseaseReferral for transplant evaluation for advanced diseaseReferral for transplant evaluation for advanced diseaseReferral for transplant evaluation for advanced disease
    • CHP, chronic hypersensitivity pneumonitis; DLCO, Diffusion Capacity of Lungs for Carbon Monoxide; DOE, dyspnea on exertion; IPF, idiopathic pulmonary fibrosis; NSIP, nonspecific interstitial pneumonitis; S4, stage 4 sarcoidosis.

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The Journal of the American Board of Family     Medicine: 31 (1)
The Journal of the American Board of Family Medicine
Vol. 31, Issue 1
January-February 2018
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Is It Idiopathic Pulmonary Fibrosis or Not?
Mary Salvatore, Genta Ishikawa, Maria Padilla
The Journal of the American Board of Family Medicine Jan 2018, 31 (1) 151-162; DOI: 10.3122/jabfm.2018.01.170288

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Is It Idiopathic Pulmonary Fibrosis or Not?
Mary Salvatore, Genta Ishikawa, Maria Padilla
The Journal of the American Board of Family Medicine Jan 2018, 31 (1) 151-162; DOI: 10.3122/jabfm.2018.01.170288
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    • UIP/IPF
    • Mimicker No. 1: Fibrotic NSIP
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