| Patient Education |
|
Click here for patient education.
|
|
You are in: eMedicine Specialties >
Radiology > CHEST
Idiopathic Pulmonary Fibrosis
Article Last Updated: Jan 19, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 9  
Author: Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR, LRCP, Chairman of Medical Imaging, Professor of Radiology, NGHA, King Fahad National Guard Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia
Ali Nawaz Khan is a member of the following medical societies: American Institute of Ultrasound in Medicine, Radiological Society of North America, Royal College of Physicians, Royal College of Physicians and Surgeons of the United States, Royal College of Radiologists, and Royal College of Surgeons of England
Coauthor(s):
Klaus L Irion, MD, PhD, Consulting Staff, The Cardiothoracic Centre Liverpool NHS Trust, The Royal Liverpool University Hospital, UK;
Alberto Alonso, MD, MRCP, Specialist Registrar in Radiology, Department of Radiology, Manchester Royal Infirmary, UK;
Anitha James, MBBS, DMRD, Specialist Registrar, Manchester Radiology Training Scheme, Hospitals NHS Trust, UK;
Velauthan Rudralingam, MBBCh, BAO, FRCS, FRCR, Staff Physician, Gastrointestinal and Body Imaging Block, Hope Hospital and Wytenshawe Hospital, UK
Editors: Jeffrey A Miller, MD, Associate Professor of Clinical Radiology, University of Medicine and Dentistry of New Jersey; Associate Chief of Service, Department of Radiology, Veterans Affairs of New Jersey Health Care System; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; John D Newell, Jr, MD, FACR, FCCP, FASER, Co-Director of Thoracic Imaging, UCDHSC; Director of Lung Imaging Center, Professor of Radiology and Professor of Medicine, Department of Radiology, University of Colorado Health Sciences Center, National Jewish Medical and Research Center; Univ. Colorado Hospital; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Kavita Garg, MD, Professor, Department of Radiology, University of Colorado Health Sciences Center
Author and Editor Disclosure
Synonyms and related keywords:
usual interstitial pneumonia, desquamative interstitial pneumonia, Hamman-Rich syndrome, cryptogenic fibrosing alveolitis alveolocapillary block, diffuse fibrosing alveolitis, fibrosing alveolitis, interstitial diffuse pulmonary fibrosis, IPF, idiopathic interstitial pneumonitis, fibrosing alveolitis, familial pulmonary fibrosis
Background
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive pulmonary disease of unknown etiology and is primarily defined by using clinical, physiologic, and radiologic criteria. In its International Consensus statement, the American Thoracic Society defines IPF as a specific chronic interstitial pneumonia that is limited to the lung and is associated with the histologic appearance of usual interstitial pneumonia (UIP) on open or thoracoscopic biopsy. No specific pathognomonic clinical or pathologic findings are associated with IPF, and the diagnosis is made after other causes of interstitial lung disease are excluded.
The diagnosis is confirmed with a lung biopsy, but the histology shows striking variation from one region to the next (histologic temporal and spatial heterogeneity). It is not unusual to find areas of normal lung next to areas with severe thickening of alveolar walls. Therefore, findings on bronchoscopic or percutaneous lung biopsy are difficult to interpret. Open lung biopsy and video-assisted thorascopic lung biopsy are the preferred methods.
IPF usually affects patients aged 50-70 years. Most series report a male preponderance, with a male-to-female ratio of 2:1. Clinical features consist of progressive exertional dyspnea, interstitial infiltrates on chest radiographs, and physiological evidence of restriction and impaired gas exchange on pulmonary function tests.
Patients are generally treated with corticosteroids, other immunosuppressants, or both.
The prognosis of patients with IPF is poor, with most patients dying of respiratory failure. The mean survival is approximately 4 years.
Pathophysiology
The pathologic features of IPF are nonspecific, and the exact pathogenesis is not clear. However, the disease is thought to evolve from undefined alveolar injuries produced by infectious, oxidative, or immunologic agents resulting in a recurring cascade of injury, repair, and subsequent fibrosis.
The histologic findings show a striking variation from region to region, and it is not unusual to find areas of normal lung next to areas with severe thickening of alveolar walls. Biopsy findings with predominant fibrosis and scant inflammatory cells are required to confirm IPF.
A diagnosis of IPF is considered when a surgical biopsy sample shows changes of UIP including the following features: (1) the exclusion of other causes of interstitial lung disease, such as those related to drug-related lung disease, asbestosis, chronic hypersensitivity pneumonitis, and collagen-vascular disease; (2) abnormalities of lung function, which include restricted and/or impaired lung gas exchange at rest or exercise or decreased diffuse lung capacity for carbon dioxide; and (3) abnormalities on conventional chest radiographs or high-resolution CT (HRCT) scans.
The diagnosis of IPF remains elusive in the absence of a lung biopsy. However, major and minor criteria have been suggested for application in immunocompetent patient. These criteria may obviate biopsy. Major criteria are the exclusion of known causes of interstitial lung disease, abnormal results on lung function tests, bibasilar reticular abnormalities and honeycombing with a minimal ground-glass appearance on HRCT, and a transbronchial lung biopsy or bronchoalveolar lavage sample that shows no features of an alternate diagnosis. Minor criteria are age older than 50 years, insidious onset of unexplained dyspnea, duration of symptoms longer than 3 months, and bibasilar inspiratory crackles.
Potential risk factors include cigarette smoking, exposure to commonly prescribed drugs, chronic aspiration, environmental factors, infections, and genetic predisposing factors.
Frequency
United States
The incidence of IPF has been estimated at 10.7 cases per 100,000 men and 7.4 cases per 100,000 women. The incidence appears to be rising. The prevalence of IPF ranges from 13 cases per 100,000 women to 20 cases per 100,000 men, though these rates may underestimate the problem.
The incidence increases with age. The disease effects individuals aged 50-70 years, and it appears to be infrequent in young people and extremely rare in children.
International
No data suggest that the international prevalence of IPF differs from that in the United States. Reports from England and Wales describe a similar prevalence of 6 cases per 100,000 population.
Mortality/Morbidity
The overall prognosis of patients with IPF is not favorable, and death often ensues within 4-6 years.
- The course of the disease varies. Some patients have a rapidly progressive fatal course over several months, whereas others have deteriorating pulmonary function over a longer period.
- Indicators of an improved prognosis are a young age, female sex, short duration of symptoms, nonsevere physiologic or radiologic impairment, increased lymphocyte counts in bronchoalveolar lavage fluids, and ground-glass opacification on HRCT.
- Complications associated with IPF are common and include frequent chest infections, frequent drug intolerance or reactions, respiratory failure, cor pulmonale or right-sided heart failure, pneumothorax, pulmonary embolism, bronchial cancers, and coronary artery disease and strokes.
Race
IPF has no racial predilection and has been reported both in the rural and urban settings. However, age-adjusted mortality rates appear higher among white population than in black population (Mannino, 1996).
Sex
IPF affects more men than women at a ratio of 2:1. See also Frequency above.
Age
IPF affects people aged 40-70 years.
- Approximately two thirds of patients with IPF are older than 60 years at the time of diagnosis, with a mean age of 66 years.
- The prevalence is 2.7 cases per 100,000 adults aged 35-44 years and is approximately 17.5 cases per 100,000 adults older than 75 years (Coultas, 1994).
- Whether a syndrome similar to IPF occurs in children is uncertain.
- See also Frequency above.
Clinical Details
IPF has no pathognomonic clinical features. Gradual-onset exertional dyspnea, which progresses, is the most common symptom. Approximately one half of patients have a dry cough early in the disease, and a similar number have constitutional symptoms, such as tiredness, weight loss, and arthralgia. Approximately 5% have no presenting symptoms at diagnosis. Patients eventually become severely disabled and oxygen dependent. Physical examination Physical examination reveals end-inspiratory rales at the lung bases. Finger clubbing occurs in 20-50% of patients. Signs of pulmonary hypertension and right-sided heart failure may be observed with progressive disease. Differential diagnosis of IPF The differential diagnosis of IPF is wide and includes the entities listed in the Differentials section below.
Preferred Examination
The diagnosis of IPF is based on the patient's history, clinical findings, pulmonary physiology, and imaging results. The diagnosis is one of exclusion. Nonidiopathic causes must be excluded first because of the important therapeutic implications. After nonidiopathic causes are excluded, further investigation in patients with IPF typically reveals radiographic abnormalities and restrictive lung physiology with decreased diffusion capacity.
Plain chest radiography is usually the first investigation performed in patients with suspected interstitial lung disease. However, the findings on conventional radiography are highly nonspecific.
HRCT defines the underlying lung parenchymal abnormalities better than does plain radiography. Recent studies have shown that HRCT may obviate surgical lung biopsy in some patients. Raghu et al compared the diagnostic accuracy of clinical evaluation combined with HRCT with the accuracy of histology based on surgical lung-biopsy samples.
Clinical assessment plus careful review of HRCT scans was 60% sensitive and 97% specific for IPF. However, though HRCT may obviate tissue diagnosis in 60% of patients, surgical lung biopsy is still needed in 40%.
For diagnoses other than IPF, a combination of clinical assessment and HRCT is neither sensitive nor specific enough to be relied on without surgical biopsy. Open lung biopsy remains the criterion standard. In immunocompetent patients, the benefit is relatively low because corticosteroid therapy is frequently administered after biopsy. In immunocompromised patients, therapy changes substantially after tissue confirmation, but the mortality rate is high. Therefore, open biopsy should be performed only in patients in whom the diagnosis is likely to change therapy and in patients in whom the underlying condition has a reasonable prognosis.
Radionuclide scanning with gallium Ga 67 may depict interstitial fibrosis and can show changes early. This feature may be of therapeutic benefit, but the changes are nonspecific and do not obviate lung biopsy.
Limitations of Techniques
Neither the clinical findings nor imaging are diagnostic of IPF. The differential diagnosis is wide.
Acute Respiratory Distress Syndrome
Asbestos-Related Disease
Asbestosis
Aspergillosis, Thoracic
Bronchiectasis
Bronchiolitis Obliterans Organizing Pneumonia
Eosinophilic Granuloma, Thoracic
Lung, Drug-Induced Disease
Lymphangioleiomyomatosis
Sarcoidosis, Thoracic
Silicosis and Coal Worker Pneumoconiosis
Wegener Granulomatosis, Thoracic
Other Problems to be Considered
Other forms of idiopathic interstitial pneumonia
Nonspecific interstitial pneumonia
Respiratory bronchiolitis–associated interstitial lung disease
Desquamative interstitial pneumonitis
Collagen-vascular diseases
Chronic hypersensitivity pneumonitis
Cryptogenic organizing pneumonia
Lymphoid interstitial pneumonia
Acute interstitial pneumonitis
Pulmonary fibrosis related to abnormalities of the connective tissue
Progressive systemic sclerosis
Systemic lupus erythematosus
Rheumatoid arthritis
Drug- or radiation-induced conditions
Antibiotics (eg, nitrofurantoin)
Cardiovascular drugs (eg, amiodarone)
Antimitotic agents (eg, bleomycin)
Radiation exposure
Hypersensitivity pneumonitis (usually the chronic form)
Organic-dust related
Bacteria
Animal-protein related
Fungi related
Occupational exposures
Asbestosis
Silicosis and other pneumoconioses
Lymphocytic interstitial pneumonitis
Sjögren syndrome
Low-grade lymphoma
HIV infection
Other conditions
Chronic aspiration pneumonia
Sarcoidosis
Lymphangioleiomyomatosis
Eosinophilic granuloma
Findings
The radiographic pattern differs depending of the stage of the disease.
Early in the disease, the most common radiographic changes are an interstitial shadowing of small (1- to 2-mm) irregular opacities, which are seen in about three fourths of patients. Less common are small, round opacities, which are seen in one fifth. This finding is generally known as reticulonodular opacities. Septal lines are occasionally observed. The distribution is predominantly basal.
Peripheral accentuation is also a common feature, but this is more easily appreciated on CT than on plain chest radiography.
The pattern is usually symmetrical. Another common pattern is hazy, ground-glass opacification, which is either diffuse or patchy. Volume loss and a raised diaphragm are seen in up to 60% of patients. This may be accompanied by basal discoid atelectasis.
Pleural disease is not typical of IPF. If it is present, it should raise the possibility of other conditions, such asbestosis, rheumatoid pulmonary disease, or systemic lupus. Pneumothorax or/and pneumomediastinum have been reported in a few patients, particularly if bullae are present in the lung parenchyma.
With progression of the alveolitis to fibrosis, the initial fine lines become coarse and small (2-mm) cysts appear. These cysts coalesce and become 5-7 mm in diameter, with ring opacities of the honeycomb lung. As fibrosis worsens, the honeycombing becomes coarser with larger honeycomb cysts, and further volume loss occurs. The advanced stages show radiographic evidence of pulmonary arterial hypertension.
Degree of Confidence
The radiographic findings are not correlated with the stage of the disease, the histology, the respiratory symptoms, the respiratory function tests, or the prognosis.
The majority of patients with IPF have an abnormal chest radiograph at presentation, and previous radiographs often show reticular shadowing, even before symptoms developed (Johnston, 1997). Chest radiography is often the first investigation that may lead to physiologic investigation and HRCT in patients with IPF.
False Positives/Negatives
Symptomatic patients with an abnormal diffusion capacity may have a normal chest radiograph. In other patients, the radiographic appearances are abnormal before clinical symptoms appear. However, HRCT is abnormal in most patients with IPF.
Findings
HRCT findings can be used to predict outcomes and to guide the treatment of patients with IPF because the findings are well correlated with the histologic pattern of the IPF. Often, HRCT can used to confidently diagnose end-stage lung disease, which is characterized by honeycombing without ground-glass attenuation in typical distribution. This spares patient the risk of invasive diagnostic processes such as a lung biopsy. In the active stage, scans demonstrate ground-glass attenuations. The active stage of the disease, which is due to active alveolitis, is potentially reversible and potentially amenable to treatment, unlike the end-stage disease, which is irreversible.
On HRCT, IPF is commonly characterized by patchy and predominantly peripheral, subpleural, and bibasilar reticular opacities with predominantly posterior distribution and often with associated traction bronchiectasis and subpleural honeycombing.
Ground-glass attenuations are relatively uncommon and usually progress to the more common reticular attenuations and honeycombing. Some literature reports that HRCT scans show honeycombing in 90% of patients with IPF.
When a trained observer interprets HRCT, the accuracy of a confident diagnosis of IPF or cryptogenic fibrosing alveolitis (CFA) appears to be about 90%. Such a confident diagnosis is made in about two thirds of patients with histologic UIP.
Any patient with suspected IPF and more than 30% ground-glass attenuation on lung HRCT should prompt a consideration of another diagnosis, such as desquamative interstitial pneumonitis, idiopathic bronchiolitis obliterans organizing pneumonia, respiratory bronchiolitis–associated interstitial lung disease, hypersensitivity pneumonitis, or nonspecific interstitial pneumonia.
Degree of Confidence
HRCT significantly increases the accuracy of the diagnosis of IPF compared with chest radiography. The accuracy of a confident diagnosis when a trained observer performs HRCT is reported to be about 90% (Grenier, 1991).
False Positives/Negatives
One third of all cases of IPF are missed as on HRCT, and a confident diagnosis of IPF is made in about two thirds (Grenier, 1991).
Findings
Perfusion lung scintigraphy shows nonspecific, subsegmental mismatched perfusion defects. These are not correlated with the clinical severity.
Gallium Ga 67 imaging has not proven to be of value in established IPF (Primack, 1994).
Technetium Tc 99m diethylenetriamine penta-acetic acid (DTPA) is cleared more rapidly when capillary permeability is increased than when it is not, and the findings may provide an index of lung inflammation (Labrune, 1994). [18-Fluorine]-fluorodeoxyglucose (FDG) positron emission tomography (PET) may show FDG accumulation in the lung bases, which is correlated with the honeycomb fibrosis on high-resolution HRCT.
Degree of Confidence
Nuclear medicine study currently has little if any role in the management of IPF.
Lung transplantation is an alternative to drug treatment for patients in the severe, final stages of IPF. It is most often performed in patients younger than 60 years whose condition does not respond to any form of treatment. The survival rate is approximately 60%.
| Media file 1:
Courtesy of Sat Sharma, MD, FRCPC, FACP, FCCP, DABSM. |
 |  View Full Size Image | |
Media type: Photo
|
| Media file 2:
Courtesy of Sat Sharma, MD, FRCPC, FACP, FCCP, DABSM. |
 | View Full Size Image | |
Media type: Photo
|
| Media file 3:
Bilateral lower lobe opacities and possible mild decrease in lung volumes. Courtesy of Sat Sharma, MD, FRCPC, FACP, FCCP, DABSM. |
 | View Full Size Image | |
Media type: X-RAY
|
| Media file 4:
High-resolution CT (HRCT) shows increased pulmonary attenuation with distortion of the pulmonary architecture. Courtesy of Sat Sharma, MD, FRCPC, FACP, FCCP, DABSM. |
 | View Full Size Image | |
Media type: CT
|
| Media file 5:
High-resolution CT (HRCT) shows distortion of the pulmonary architecture with thickening of pulmonary interstitium and some areas of ground-glass attenuation. No obvious honeycombing is present. Courtesy of Sat Sharma, MD, FRCPC, FACP, FCCP, DABSM. |
 | View Full Size Image | |
Media type: CT
|
| Media file 6:
HRCT of advanced stage of pulmonary fibrosis demonstrating reticular opacities with honeycombing, with predominant subpleural distribution. |
 | View Full Size Image | |
Media type: CT
|
- Ambrosini V, Cancellieri A, Chilosi M, et al. Acute exacerbation of idiopathic pulmonary fibrosis: report of a series. Eur Respir J. Nov 2003;22(5):821-6. [Medline].
- Antonio GE, Wong KT, Hui DS, et al. Thin-section CT in patients with severe acute respiratory syndrome following hospital discharge: preliminary experience. Radiology. Sep 2003;228(3):810-5. [Medline].
- Baughman RP, Fernandez M. Radionuclide imaging in interstitial lung disease. Curr Opin Pulm Med. Sep 1996;2(5):376-9. [Medline].
- Bekerman C, Hoffer PB, Bitran JD, Gupta RG. Gallium-67 citrate imaging studies of the lung. Semin Nucl Med. Jul 1980;10(3):286-301. [Medline].
- Belzunegui J, Intxausti JJ, De Dios JR, et al. Absence of pulmonary fibrosis in patients with psoriatic arthritis treated with weekly low-dose methotrexate. Clin Exp Rheumatol. Nov-Dec 2001;19(6):727-30. [Medline].
- Bourke SJ, Hawkins T, Keavey PM, et al. Ventilation perfusion radionuclide imaging in cryptogenic fibrosing alveolitis. Nucl Med Commun. Jun 1993;14(6):454-64. [Medline].
- Boxt LM. MR imaging of pulmonary hypertension and right ventricular dysfunction. Magn Reson Imaging Clin N Am. May 1996;4(2):307-25. [Medline].
- Camus P, Fanton A, Bonniaud P, Foucher P. Interstitial lung disease induced by drugs and radiation. Respiration. Jul-Aug 2004;71(4):301-26.
- Coultas DB, Zumwalt RE, Black WC, Sobonya RE. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med. Oct 1994;150(4):967-72.
- Desai SR, Ryan SM, Colby TV. Smoking-related interstitial lung diseases: histopathological and imaging perspectives. Clin Radiol. Apr 2003;58(4):259-68. [Medline].
- Erbes R, Schaberg T, Loddenkemper R. Lung function tests in patients with idiopathic pulmonary fibrosis. Are they helpful for predicting outcome?. Chest. Jan 1997;111(1):51-7. [Medline].
- Franquet T, Giménez A, Torrubia S, et al. Spontaneous pneumothorax and pneumomediastinum in IPF. Eur Radiol. 2000;10(1):108-13. [Medline].
- Gast KK, Puderbach MU, Rodriguez I, et al. Distribution of ventilation in lung transplant recipients: evaluation by dynamic 3He-MRI with lung motion correction. Invest Radiol. Jun 2003;38(6):341-8. [Medline].
- Gay SE, Kazerooni EA, Toews GB, et al. Idiopathic pulmonary fibrosis: predicting response to therapy and survival. Am J Respir Crit Care Med. Apr 1998;157(4 Pt 1):1063-72. [Medline].
- Grenier P, Valeyre D, Cluzel P, et al. Chronic diffuse interstitial lung disease: diagnostic value of chest radiography and high-resolution CT. Radiology. Apr 1991;179(1):123-32. [Medline].
- Hansell, DM, Armstrong, P, Lynch DA, H. Page McAdams. Imaging of Diseases of the Chest. 4th ed. St. Louis, Mo:. Mosby;2005.
- Hunninghake GW, Kalica AR. Approaches to the treatment of pulmonary fibrosis. Am J Respir Crit Care Med. Mar 1995;151(3 Pt 1):915-8. [Medline].
- James JM, Lloyd JJ, Leahy BC, et al. 99Tcm-Technegas and krypton-81m ventilation scintigraphy: a comparison in known respiratory disease. Br J Radiol. Dec 1992;65(780):1075-82. [Medline].
- Johnston ID, Prescott RJ, Chalmers JC, Rudd RM. British Thoracic Society study of cryptogenic fibrosing alveolitis: current presentation and initial management. Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society. Thorax. Jan 1997;52(1):38-44. [Medline].
- Jung JI, Park SH, Lee JM, et al. MR characteristics of progressive massive fibrosis. J Thorac Imaging. Apr 2000;15(2):144-50. [Medline].
- Kataoka M, Kawamura M, Ueda N, et al. Diffuse gallium-67 uptake in radiation pneumonitis. Clin Nucl Med. Oct 1990;15(10):707-11. [Medline].
- Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med. Apr 1998;157(4 Pt 1):1301-15. [Medline].
- Kauczor HU, Kreitner KF. MRI of the pulmonary parenchyma. Eur Radiol. 1999;9(9):1755-64. [Medline].
- King TE Jr. Idiopathic pulmonary fibrosis. In: Schwarz MI, King TE Jr, eds. Interstitial Lung Disease. St. Louis, MO:. Mosby-Year Book;1993: 367-403.
- Konen E, Weisbrod GL, Pakhale S, et al. Fibrosis of the upper lobes: a newly identified late-onset complication after lung transplantation?. AJR Am J Roentgenol. Dec 2003;181(6):1539-43.
- Kroft LJ, Simons P, van Laar JM, de Roos A. Patients with pulmonary fibrosis: cardiac function assessed with MR imaging. Radiology. Aug 2000;216(2):464-71. [Medline].
- Labrune S, Chinet T, Collignon MA, et al. Mechanisms of increased epithelial lung clearance of DTPA in diffuse fibrosing alveolitis. Eur Respir J. Apr 1994;7(4):651-6. [Medline].
- Müller NL, Mayo JR, Zwirewich CV. Value of MR imaging in the evaluation of chronic infiltrative lung diseases: comparison with CT. AJR Am J Roentgenol. Jun 1992;158(6):1205-9. [Medline].
- MacDonald SL, Rubens MB, Hansell DM, et al. Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparative appearances at and diagnostic accuracy of thin-section CT. Radiology. Dec 2001;221(3):600-5. [Medline].
- Mannino DM, Etzel RA, Parrish RG. Pulmonary fibrosis deaths in the United States, 1979-1991. An analysis of multiple-cause mortality data. Am J Respir Crit Care Med. May 1996;153(5):1548-52. [Medline].
- Paltiel HJ, Azouz EM, Esseltine DW, Bernstein M. Computed tomography in interstitial lung disease. J Comput Tomogr. Oct 1986;10(4):349-56. [Medline].
- Pipavath S, Godwin JD. Imaging of the chest: idiopathic interstitial pneumonia. Clin Chest Med. Dec 2004;25(4):651-6, v-vi.
- Pochis WT, Krasnow AZ, Collier BD, et al. Idiopathic pulmonary fibrosis. A rare cause of scintigraphic ventilation-perfusion mismatch. Clin Nucl Med. May 1990;15(5):321-3. [Medline].
- Potente G, Bellelli A, Nardis P. Specific diagnosis by CT and HRCT in six chronic lung diseases. Comput Med Imaging Graph. Jul-Aug 1992;16(4):277-82. [Medline].
- Primack SL, Mayo JR, Hartman TE, et al. MRI of infiltrative lung disease: comparison with pathologic findings. J Comput Assist Tomogr. Mar-Apr 1994;18(2):233-8. [Medline].
- Raghu G. Idiopathic pulmonary fibrosis. A rational clinical approach. Chest. Jul 1987;92(1):148-54. [Medline].
- Raghu G. Interstitial lung disease: a diagnostic approach. Are CT scan and lung biopsy indicated in every patient?. Am J Respir Crit Care Med. Mar 1995;151(3 Pt 1):909-14. [Medline].
- Rizzato G. Is nuclear imaging of any value in managing interstitial fibrosis?. Curr Opin Pulm Med. Sep 1997;3(5):372-7. [Medline].
- Ryu JH, Colby TV, Hartman TE. Idiopathic pulmonary fibrosis: current concepts. Mayo Clin Proc. Nov 1998;73(11):1085-101. [Medline].
- Shah RM, Miller W. Widespread ground-glass opacity of the lung in consecutive patients undergoing CT: Does lobular distribution assist diagnosis?. AJR Am J Roentgenol. Apr 2003;180(4):965-8. [Medline].
- Shaw RJ, Djukanovic R, Tashkin DP, et al. The role of small airways in lung disease. Respir Med. Feb 2002;96(2):67-80. [Medline].
- Siemsen JK, Grebe SF, Waxman AD. The use of gallium-67 in pulmonary disorders. Semin Nucl Med. Jul 1978;8(3):235-49. [Medline].
- Simkin PH, Licho R, Brill AB. Pulmonary nuclear medicine. Curr Opin Radiol. Dec 1991;3(6):859-70. [Medline].
- Strickland B. Imaging fibrosing alveolitis. Postgrad Med J. 1988;64 Suppl 4:35-40. [Medline].
- Strollo DC. Imaging of idiopathic interstitial lung diseases. Concepts and conundrums. Am J Respir Cell Mol Biol. Sep 2003;29(3 Suppl):S10-8. [Medline].
- Wells A. Clinical usefulness of high resolution computed tomography in cryptogenic fibrosing alveolitis. Thorax. Dec 1998;53(12):1080-7. [Medline].
- Wittram C, Mark EJ, McLoud TC. CT-histologic correlation of the ATS/ERS 2002 classification of idiopathic interstitial pneumonias. Radiographics. Sep-Oct 2003;23(5):1057-71. [Medline].
- Ziesche R, Hofbauer E, Wittmann K, et al. A preliminary study of long-term treatment with interferon gamma-1b and low-dose prednisolone in patients with idiopathic pulmonary fibrosis. N Engl J Med. Oct 21 1999;341(17):1264-9. [Medline].
- Zompatori M, Calabrò E, Chetta A, et al. [Chronic hypersensitivity pneumonitis or idiopathic pulmonary fibrosis? Diagnostic role of high resolution Computed Tomography (HRCT)]. Radiol Med (Torino). Sep 2003;106(3):135-46. [Medline].
- du Bois RM. Idiopathic pulmonary fibrosis. Annu Rev Med. 1993;44:441-50. [Medline].
Idiopathic Pulmonary Fibrosis excerpt Article Last Updated: Jan 19, 2007
|
