Review

Idiopathic pulmonary fibrosis: What primary care physicians need to know

Leslie B. Tolle, MD, Brian D. Southern, MD, Daniel A. Culver, DO and Jeffrey C. Horowitz, MD
Cleveland Clinic Journal of Medicine May 2018, 85 (5) 377-386; DOI: https://doi.org/10.3949/ccjm.85a.17018

Article Figures & Data

Figures

  • Figure 1
    Figure 1

    Idiopathic pulmonary fibrosis progresses in several ways. Some patients have a rapid deterioration with a poor short-term prognosis. In some, the disease progresses slowly with possible occasional exacerbations that cause a marked decline in lung function with no subsequent return to prior baseline function. In some cases, these patients continue to have slow disease progression, while others continue to have a stepwise decline in lung function. In a small number of patients, the disease is stable over time or progresses so slowly that the patient dies of another condition.

  • Figure 2
    Figure 2

    Histopathologic appearance of definite pattern of usual interstitial pneumonia (UIP), a key feature of idiopathic pulmonary fibrosis. A, dense fibrosis (plus sign) with a “honeycomb” change (asterisk) is prominent at the pleural surface (arrow) juxtaposed against normal lung tissue (star) toward the center of the lung parenchyma (hematoxylin and eosin, × 40). B, the same features appear at higher magnification, and fibroblastic foci (arrow) are seen at the leading edge of fibrosis (hematoxylin and eosin, × 100).

  • Figure 3
    Figure 3

    Radiographic patterns of definite usual interstitial pneumonia. Chest radiography (A) shows mildly decreased lung volumes with basilar-predominant coarse reticular (linear) opacities (arrow) and intervening areas of cystic lucencies, consistent with honeycombing (arrowhead). Axial high-resolution computed tomography (B, C, and D) shows coarse subpleural reticulation (arrow in B), traction bronchiectasis, and severe honeycombing, ie, rows of cysts stacked one on top of the other (arrowheads in C and D). These progressively worsen as the images move inferiorly.

  • Figure 4
    Figure 4

    A diagnostic algorithm for idiopathic pulmonary fibrosis (IPF). Patients with suspected IPF should be evaluated for causes of nonspecific dyspnea, and specifically for interstitial lung disease (ILD). If no cause is identified, then high-resolution computed tomography (HRCT) is recommended to determine the pattern of usual interstitial pneumonia (UIP). If a definite UIP pattern is seen, then a diagnosis of IPF can be made. Otherwise, surgical lung biopsy is the next step. A final diagnosis of IPF can be made if the histologic pattern is definite, probable, or possible UIP, and if the multidiscplinary team (MDD) concurs that IPF is the most likely diagnosis.

Tables

  • TABLE 1

    Overview of interstitial lung diseases

    Exposure-related
    Occupational
    Environmental
    Avocational
    Medication
    Connective tissue disease
    Scleroderma
    Rheumatoid arthritis
    Sjögren syndrome
    Polymyositis, dermatomyositis
    Sarcoidosis
    Idiopathic
    Idiopathic pulmonary ibrosis
    Idiopathic nonspecific interstitial pneumonia
    Respiratory bronchiolitis-associated interstitial lung disease
    Desquamative interstitial pneumonia
    Cryptogenic organizing pneumonia
    Acute interstitial pneumonia
    Other
    Vasculitis, diffuse alveolar hemorrhage
    Langerhans cell histiocytosis
    Eosinophilic pneumonia
    Neurofibromatosis
    Lymphangioleiomyomatosis
  • TABLE 2

    Idiopathic pulmonary fibrosis: Checklist for evidence-based practice

    Evaluation
    Assessment of symptoms
    Occupational or environmental exposures
    Medication history
    Pulmonary function testing
    6-minute walk test
    High-resolution computed tomography
    Laboratory testing for connective tissue disease, if appropriate
    Echocardiography
    Interventions
    Refer to center specializing in interstitial lung disease (ILD)
    Antifibrotic therapy, in consultation with ILD center
    Pulmonary rehabilitation
    Smoking cessation
    Vaccinations (influenza, pneumococcal)
    Referral for lung transplant
    Supplemental oxygen
    Screening for depression
    Therapy for gastroesophageal reflux disease
    Enrollment in a clinical trial
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