Pulmonary Fibrosis

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Related to Pulmonary Fibrosis: Idiopathic pulmonary fibrosis

Pulmonary Fibrosis



Pulmonary fibrosis is scarring in the lungs.


Pulmonary fibrosis develops when the alveoli, tiny air sacs that transfer oxygen to the blood, become damaged and inflamed. The body tries to heal the damage with scars, but these scars collapse the alveoli and make the lungs less elastic. If the cycle of inflammation and scarring continues, the lungs become increasingly unable to deliver oxygen to the blood. Changes in the lungs can also increase the blood pressure in the pulmonary artery. This condition, called pulmonary hypertension, makes the heart work harder and it may fail.
Pulmonary fibrosis can result from many different lung diseases including sarcoidosis, drug reactions, autoimmune diseases, environmental allergies such as Farmer's lung, and exposure to toxic dusts and gases.
Pulmonary fibrosis that develops without a known cause is called idiopathic pulmonary fibrosis. This disease is equally common in men and women. It is usually diagnosed between the ages of 40 and 60.

Causes and symptoms

The causes and risk factors vary with the underlying disease. They may include genetics, environmental factors, and infections.
The first symptom of pulmonary fibrosis is usually shortness of breath—at first, during exercise, but later also while resting. Patients may also have a dry cough, a rapid heartbeat, or enlargement of the fingertips and ends of the toes. Some people feel tired or have a fever, weight loss, muscle or joint pains. In late stages of the disease, the lack of oxygen in the blood can give the skin and mucus membranes a blue tinge known as cyanosis.


Pulmonary fibrosis is often referred to a lung specialist. Several tests are usually needed to diagnose this disease and determine its cause. They include a physical examination, detailed history of the symptoms, chest x rays, lung function tests, and blood tests, including a measurement of the amount of oxygen in the blood. Computed tomography (CT scan) may give a more detailed picture of the lungs. Bronchoscopy may be done to examine the air passages and analyze the cells found deep in the lungs.
Lung biopsies are necessary to diagnose some diseases. Lung biopsies can be done through a needle inserted into the chest through the skin, during bronchoscopy, or as a surgical procedure under general anesthesia.


The treatment of pulmonary fibrosis depends on the underlying cause. Many diseases are treated by suppressing inflammation with corticosteroids. Stronger immune suppressants such as cyclophosphamide (Cytoxan) or azathioprine (Imuran) may also be tried. Some patients need supplemental oxygen. A lung transplant may be an option for incurable diseases. Approximately 60-80% of patients live for at least two years after the transplant.
There is no good treatment for idiopathic pulmonary fibrosis. Only 10-20% of patients with this disease respond to corticosteroids.

Alternative treatment

Anxiety and fear can make breathing difficulties worse. Some patients find that activities such as yoga, prayer or meditation, music therapy, or biofeedback help to relax them.


The prognosis depends on the specific disease. Some cases may stop progressing or improve, particularly if the cause can be identified and treated. Others may develop quickly or slowly into end-stage lung disease. The course of idiopathic pulmonary fibrosis is very difficult to predict; however, average survival is approximately five to seven years.


There is no known prevention for idiopathic pulmonary fibrosis.
Some ways to prevent other causes of pulmonary fibrosis are:
  • avoid exposure to particle dust such as asbestos, coal dust, and silica
  • avoid exposure to chemical fumes
  • do not smoke

Key terms

Alveoli — Tiny air sacs in the lungs where oxygen and carbon dioxide are exchanged with the blood.
Autoimmune disease — A disease that develops when the immune system attacks normal cells or organs.
Bronchoscopy Scan — The examination of the air passages through a flexible or rigid tube inserted into the nostril (or mouth). Sometimes cells are collected by washing the lungs with a small amount of fluid.
Computed tomography(CT) — A special x-ray technique that produces a cross sectional image of the organs inside the body.
Corticosteroids — A class of drugs, related to hormones naturally found in the body, that suppress the immune system. One example is prednisone, sold under many brand names including Deltasone.
End-stage lung disease — The final stages of lung disease, when the lung can no longer keep the blood supplied with oxygen. End-stage lungs in pulmonary fibrosis have large air spaces separated by bands of inflammation and scarring.
Farmer's lung — An allergic reaction to moldy hay, most often seen in farmers, that results in lung disease.
Immune suppressant drug — Any drug that dampens immune responses and decreases inflammation.
Inflammation — The body's reaction to an irritant, characterized by the accumulation of immune cells, redness, and swelling.
Lung function tests — Tests of how much air the lungs can move in and out, and how quickly and efficiently this can be done. Lung function tests are usually done by breathing into a device that measures air flow.
Mucous membranes — The moist coverings that line the mouth, nose, intestines, and other internal organs.
Pulmonary artery — The blood vessel that delivers blood from the heart to the lungs.
Sarcoidosis — A disease of unknown origin that results in clumps of immune cells and inflammation in organs throughout the body.



Kobzik, Lester. "Diffuse Interstitial (Infiltrative, Restrictive) Diseases." In Robbins Pathologic Basis of Disease, edited by Ramzi S. Cotran, Vinay Kumar, and Tucker Collins, 6th ed. Philadelphia: W.B. Saunders, 2000, pp. 727-740.
Toews, Galen B. "Interstitial Lung Disease." In Cecil Textbook of Medicine, edited by Lee Goldman and J. Claude Bennett, 21st ed. Philadelphia: W.B. Saunders, 2000,pp. 409-419.


Mason, Robert J., Marvin I. Schwarz, Gary W. Hunninghake, and Robert A. Musson. "Pharmacologic Therapy for Idiopathic Pulmonary Fibrosis: Past, Present, and Future." American Journal of Respiratory and Critical CareMedicine 160 (1999): 1771-1777.
Michaelson, Jeffrey E., Samuel M. Aguayo, and Jesse Roman. "Idiopathic Pulmonary Fibrosis: A Practical Approach for Diagnosis and Management." Chest 118, no. 3 (September2000): 788-94.


The American Lung Association. 1740 Broadway, New York. NY 10019. (212) 315-8700. http://www.lungusa.org.
Pulmonary Fibrosis Association. P.O. Box 75004, Seattle, WA 98125-0004. (206) 417-0949. 〈http://pulmonaryfibrosisassn.com〉.
Pulmonary Fibrosis Foundation. 1075 Santa Fe Drive, Denver, Colorado 80204. (720) 932-7850. http://pulmonaryfibrosis.org.


"Bronchoscopy with Transtracheal Biopsy" Health Encyclopedia. 2001. [cited May 2, 2001]. http://www.merck-medco.com/medco/index.jsp.
Chronic Lung Disease Information Resource. The Cheshire Medical Center. May 5, 2001. http://www.cheshiremed.com/programs/pulrehab/rehinfof.html.
"Lung Needle Biopsy." Health Encyclopedia. 2001. [cited May 2, 2001]. http://www.merck-medco.com/medco/index.jsp.
"Open Lung Biopsy" Health Encyclopedia. 2001. [cited May 2, 2001]. http://www.merck-medco.com/medco/index.jsp.
Gale Encyclopedia of Medicine. Copyright 2008 The Gale Group, Inc. All rights reserved.


formation of fibrous tissue; see also fibroid degeneration. adj., adj fibrot´ic.
congenital hepatic fibrosis a developmental disorder of the liver, marked by formation of irregular broad bands of fibrous tissue containing multiple cysts formed by disordered terminal bile ducts, resulting in vascular constriction and portal hypertension.
cystic fibrosis (cystic fibrosis of pancreas) see cystic fibrosis.
diffuse idiopathic interstitial fibrosis (diffuse interstitial pulmonary fibrosis) idiopathic pulmonary fibrosis.
endomyocardial fibrosis an idiopathic type of myocardiopathy that is endemic in various parts of Africa and rarely in other areas, characterized by cardiomegaly, marked thickening of the endocardium with dense white fibrous tissue that may extend to involve the inner myocardium, and by congestive heart failure.
idiopathic pulmonary fibrosis chronic inflammatory progressive fibrosis of the pulmonary alveolar walls, with steadily progressive dyspnea, resulting in death from oxygen lack or right heart failure. Most cases are of unknown origin, although some are thought to result from pneumoconiosis, hypersensitivity pneumonitis, scleroderma, and other diseases.
mediastinal fibrosis development of hard white fibrous tissue in the upper portion of the mediastinum, sometimes obstructing the air passages and large blood vessels; called also fibrosing or fibrous mediastinitis.
periureteral fibrosis retroperitoneal fibrosis.
pleural fibrosis fibrosis of the visceral pleura so that part or all of a lung becomes covered with a plaque or a thick layer of nonexpansible fibrous tissue. The more extensive form is called fibrothorax.
postfibrinous fibrosis that occurring in tissues in which fibrin has been deposited.
proliferative fibrosis that in which the fibrous elements continue to proliferate after the original causative factor has ceased to operate.
pulmonary fibrosis idiopathic pulmonary fibrosis.
retroperitoneal fibrosis deposition of fibrous tissue in the retroperitoneal space, producing vague abdominal discomfort, and often causing blockage of the ureters, with resultant hydronephrosis and impaired renal function, which may result in renal failure. Called also Ormond disease.
fibrosis u´teri a morbid condition characterized by overgrowth of the smooth muscle and increase in the collagenous fibrous tissue of the uterus, producing a thickened, coarse, tough myometrium.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

pulmonary fibrosis

Scarring of lung tissue from any cause, such as PNEUMOCONIOSIS, previous PNEUMONIA or TUBERCULOSIS or repeated attacks of PULMONARY EMBOLISM. Fibrosis interferes with the efficient transfer of oxygen from the atmosphere to the blood and causes breathlessness. There is no specific treatment, short of a lung transplant, but progress can often be slowed.
Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005

Pulmonary Fibrosis

DRG Category:196
Mean LOS:6.9 days
Description:MEDICAL: Interstitial Lung Disease With Major CC

Pulmonary fibrosis is a restrictive lung disease in which alveolar inflation is reduced, thus impairing lung function. The alveoli are affected by fibrotic tissue, which may develop after inflammation, infection, or tissue damage. The resulting scarring and distortion of pulmonary tissue lead to serious compromise in gas exchange. Fibrosis leads to decreased lung compliance and increased elastic recoil, which increases the overall work of breathing and inefficient exchange of gases.


Idiopathic pulmonary fibrosis (IPF) is of unknown etiology and is characterized by a poor prognosis and no effective treatment. Some forms of pulmonary fibrosis can be attributed to exposure to radiation or inhalation of noxious materials such as silica, asbestos, and coal dust. Pulmonary conditions that can result in pulmonary fibrosis include pneumonia, atelectasis, alveolar cell cancer, pulmonary edema, and lung surgery or trauma. Nonpulmonary causes include neuromuscular disease such as Guillain-Barré syndrome, amyotrophic lateral sclerosis, myasthenia gravis, and muscular dystrophy. Approximately one-third of patients can trace their initial episodes of dyspnea to a viral respiratory illness. Deformities of the bones, such as ankylosing spondylitis and scoliosis, can result in pulmonary fibrosis.

Genetic considerations

Genetic contribution to pulmonary fibrosis is suggested by familial cases of the disease. Variants in the gene encoding pulmonary surfactant protein A1 (SFTPA1) have increased susceptibility to idiopathic pulmonary fibrosis in nonsmokers. Mutations in SFTPA2 can cause idiopathic pulmonary fibrosis. Mutations in the gene encoding surfactant protein C (SFTPC) have been identified in some families with idiopathic pulmonary fibrosis, but not in others.

Gender, ethnic/racial, and life span considerations

Although it is possible for pulmonary fibrosis to occur at any age, the average age of patients who are diagnosed with pulmonary fibrosis is 50. Elderly patients or those exposed to risk factors for a prolonged period of time are at the greatest risk. Pulmonary fibrosis occurs in both men and women, and there is no known racial or ethnic patterns of prevalence or incidence.

Global health considerations

Around the world, the prevalence is reported as 10 to 20 cases per 100,000 individuals. The influences of geographical region, environmental exposure by region, and regional culture such as dietary habits and differences in smoking patterns are unknown.



Establish a history of work or lifestyle that may have caused the disease. Ask if the patient has worked as a coal miner or with materials such as asbestos or silica or whether he or she has lived near industrial plants that use such materials. Determine if the patient has had respiratory complications or conditions such as pneumonia, atelectasis, alveolar cell cancer, pulmonary edema, and lung surgery or trauma. Ask if the patient has experienced pain while breathing or shortness of breath. Ask if the patient smokes cigarettes. There is some suspicion that genetic factors may determine susceptibility to the disease, so take a family history of pulmonary conditions, including pulmonary fibrosis.

Physical examination

Although the symptoms of pulmonary fibrosis are nonspecific, the patient may have progressive dyspnea on exertion and cough. Observe the patient’s respiratory status, noting rate, depth, rhythm, and ease of breathing. In the initial phases of the disease, the physical examination may be normal and the lungs may be clear on auscultation. As the disease progresses, individuals with pulmonary fibrosis frequently develop shallow, rapid breathing patterns in an attempt to conserve energy. Auscultate the patient’s lungs; listen for diminished or absent breath sounds or coarse crackles, particularly at the lung bases on inspiration. Note chest excursion and symmetry. Check for digital clubbing. Some patients develop weight loss, fatigue, fever, and muscle pain. Because pulmonary fibrosis in its late stages can cause cor pulmonale, check for signs of cardiac failure, such as elevated neck veins, liver distention, and swelling of the lower extremities.


Patients may experience a lowering of self-esteem with increased dependence on others and changing roles. In addition, shortness of breath and difficulty in breathing usually cause increased anxiety. If the disease has an occupational source, financial concerns may play an important role if the patient is unable to return to work.

Diagnostic highlights

TestNormal ResultAbnormality With ConditionExplanation
Chest x-rayClear lung fieldsIdentification of interstitial infiltrates or ground glass patternFibrotic areas have a changed appearance
High-resolution computed tomographyNormal lung structuresReticular opacities, traction bronchiectasis, honeycombing, and alveolar distortion; ground-glass patternFibrotic areas have a changed appearance
Forced expiratory flow: Maximal flow rate attained during the middle (25%–75%) of forced vital capacity maneuverVaries by body size25% of the predicted valuePredicts obstruction of smaller airways
Residual volume (RV): Volume of air remaining in lungs at end of a maximal expiration1.2 LIncreased up to 400% normalIncreased RV indicates obstruction
Rheumatoid factor, antinuclear antibodiesNegativeRheumatoid factor > 30 IU/mL; antinuclear antibodies > 1:8Identifies autoimmune response and connective tissue disease

Other Tests: Hemoglobin may be elevated due to chronic hypoxemia; erythrosedimentation rate may be elevated; C-reactive protein, gallium scan, serological tests, histological analysis, high-resolution computed tomography

Primary nursing diagnosis


Ineffective breathing pattern related to shortness of breath and difficulty breathing


Respiratory status: Gas exchange; Respiratory status: Ventilation; Symptom control behavior; Treatment behavior: Illness or injury; Comfort level


Airway management; Anxiety reduction; Oxygen therapy; Airway suctioning; Airway insertion and stabilization; Cough enhancement; Mechanical ventilation; Positioning; Respiratory monitoring

Planning and implementation


To relieve breathing difficulties and correct hypoxia, most physicians prescribe low-flow oxygen therapy (2 to 4 L/minute). Corticosteroid therapy is the treatment of choice. If the patient does not respond to the corticosteroid therapy, immunosuppression with cyclophosphamide (Cytoxan) is considered. Cyclophosphamide reduces the white blood cell count, causing a distinct drop in the total blood lymphocyte count. Patients who do not respond to conventional therapy and whose life expectancy is less than 18 months may be candidates for lung transplantation. If cor pulmonale develops, the patient may be placed on diuretics and digitalis. Bronchodilators may improve wheezing and airway obstruction. Infections need to be identified and treated promptly. Pneumococcal and influenza vaccines are important.

Pharmacologic highlights

Medication or Drug ClassDosageDescriptionRationale
Prednisone5–60 mg/day PO; dose is individualizedCorticosteroidDecreases the inflammatory process
Cyclophosphamide (Cytoxan)1–5 mg/kg per day; dose is individualizedAntineoplasticReduces the white blood cell count, causing a distinct drop in the total blood lymphocyte count

Other Drugs: Immunosuppressive agents azathioprine (Imuran), antifibrotic agents (colchicine), and antiviral cytokines (interferon gamma-1b). Experimental Drug: Pirfenidone (antifibrotic agent)


Focus on relieving respiratory difficulties and caring for the patient’s emotional condition. To assist with breathing, assist the patient to attain an upright, supported position to enhance respiratory excursion. Assist the patient into a Fowler’s or semi-Fowler’s position. Provide assistance with the activities of daily living as appropriate and help the patient conserve energy by alternating rest periods with periods of activity. Plan rest time of at least 1 hour after meals before engaging in activities. Teach the patient deep-breathing and coughing exercises. Use humidified air. Provide regular oral hygiene to combat dry mouth.

Because there is no cure for pulmonary fibrosis, dealing with a chronic debilitating disease requires many psychosocial adjustments for the patient and family members. Encourage the patient to verbalize concerns and fears. Encourage the patient to identify actions and care measures that help make her or him comfortable and relaxed. As much as possible, try to include the patient and family in decisionmaking about care measures. Lifestyle changes for the patient may be necessary. A well-balanced diet with adequate fluid intake is important. If the patient smokes cigarettes, cigars, or pipes, smoking cessation is an essential intervention for the patient’s survival. A job counseling session may be helpful if the patient needs to change occupations. If the patient is having trouble coping with role changes, counseling may be helpful.

Evidence-Based Practice and Health Policy

Lamas, D.L., Kawut, S.M., Bagiella, E., Philip, N., Arcasoy, S.M., & Lederer, D.J. (2011). Delayed access and survival in idiopathic pulmonary fibrosis. American Journal of Respiratory and Critical Care Medicine, 184(7), 842–847.

  • Investigators conducted prospective study among 129 patients diagnosed with pulmonary fibrosis and found that increased delays in the time from dyspnea onset to initial evaluation for pulmonary fibrosis was associated with an increased mortality rate. The median delay was 2.2 years (IQR, 1 to 3.8 years).
  • Compared to patients with less than a 1-year delay in evaluation, mortality was twice as likely among patients with a 1- to 2-year delay, 2.5 times more likely among patients with a 2- to 4-year delay, and 3.4 times more likely among patients with more than a 4-year delay (p = 0.04). Thirty-one percent of patients underwent lung transplantation, 27% died without undergoing lung transplantation, and 3% died after lung transplantation.
  • Patients with diabetes mellitus and gastroesophageal reflux disease experienced significantly longer delays than patients without these comorbidities (p = 0.049 and p = 0.01, respectively).

Documentation guidelines

  • Physical changes: Skin color, respiratory patterns, breath sounds, breathing difficulties, chest symmetry and excursion, pulse oximetry
  • Reaction to diagnosis and coping strategies
  • Frequency of oxygen use, noting liter flow and type of delivery device, activity tolerance

Discharge and home healthcare guidelines

Teach energy conservation methods and relaxation, breathing, and coughing techniques. Explain the importance of pacing activities, avoiding strenuous activity, and providing rest periods. Teach the patient positions that can provide relief during acute episodes of dyspnea. To prevent infection, encourage the patient to receive flu and pneumococcal vaccines and to avoid crowds and people with known respiratory infections. Be sure the patient understands all medications, including the dosage, route, action, and adverse effects. If the patient is using oxygen therapy at home, teach the patient and family appropriate safety precautions. Help the patient understand the equipment and liter flow and provide information on how to obtain all the necessary equipment. Work with social services to provide for equipment in the home.

Diseases and Disorders, © 2011 Farlex and Partners

Patient discussion about Pulmonary Fibrosis

Q. my uncle was diagnosed with pulmonary fibrosis. can anyone help?

A. Pulmonary Fibrosis involves scarring of the lung. Gradually, the air sacs of the lungs become replaced by fibrotic tissue. When the scar forms, the tissue becomes thicker causing an irreversible loss of the tissue’s ability to transfer oxygen into the bloodstream. Traditional theories have postulated that it might be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a growing body of evidence which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of Pulmonary Fibrosis. The most current thinking is that the fibrotic process is a reaction to microscopic injury to the lung. While the exact cause remains unknown, associations have been made with the following:*Inhaled environmental and occupational pollutants, *Cigarette smoking, *Diseases such as Scleroderma, Rheumatoid Arthritis, Lupus,*Therapeutic radiation. For full: http://www.pulmonaryfibrosis.org/ipf.htm Hope this helps.

More discussions about Pulmonary Fibrosis
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References in periodicals archive ?
In all patients with idiopathic pulmonary fibrosis and in all patients with pulmonary fibrosis and COPD, inhalation of the nasal spray demonstrated a significant reduction of coughing and a significant improvement in nasal irritation/erythema, inflammation and congestion, with most patients free of irritation by day 22.
Idiopathic Pulmonary Fibrosis Treatment is a life-threatening condition of the lungs in which tissues becomes thickened, stiff, and scarred over a long period.
"Action for Pulmonary Fibrosis is calling on NHS England to prioritise improvement in the rate of diagnosis for this condition."
Gingko biloba and carnitine were also studied in rats exposed to a substance that causes pulmonary fibrosis. (57) Researchers induced pulmonary fibrosis in the animals then administered ginkgo or carnitine.
(2.) Raghu G, Collard HR, Egan JJ, et al; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis--evidence-based guidelines for diagnosis and management.
Familial pulmonary fibrosis was defined by the presence of two or more cases of definitive or probable idiopathic interstitial pneumonia within three generations of a family, with at least one case of idiopathic interstitial pneumonia established as a definitive or probable case of IPF.
One of the lead drug candidates emerging from this platform, PBI-4050, is expected to enter pivotal phase 3 clinical trials for the treatment of Idiopathic Pulmonary Fibrosis (IPF).
Recommendations for the management of idiopathic pulmonary fibrosis in South Africa: A position statement of the South African Thoracic Society.
Interesting data supporting its utility and validity in the assessment of the lung to detect and quantify interstitial pulmonary fibrosis in rheumatic diseases, even in subclinical phases, have been reported.
An observational study focusing on pulmonary fibrosis in MPA showed that myalgia was freguently observed in MPA cases with pulmonary fibrosis, compared with cases without pulmonary fibrosis (46% vs.
[U.S.A.], May 27 ( ANI ): Developing new medicines to treat pulmonary fibrosis, one of the most common and serious forms of lung disease, is not easy.
Approximately two hundred entities are included in this group of diseases, the most common being idiopathic pulmonary fibrosis (IPF).2

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