Edit concept Question Editor Create issue ticket

Pulmonary Fibrosis

Lung Fibrosis

In pulmonary fibrosis, excess fibrous connective tissue develops in the lungs. It presents initially with dyspnea, loss of appetite, fatigue and dry cough.


Presentation

The most pronounced clinical feature of pulmonary fibrosis is progressive exertional dyspnea associated with the production of dry cough.

On examination, there is gross clubbing of the fingernails and toenails. The movements of chest on the affected side are reduced. The trachea is shifted if there is fibrosis of the upper lobe. In fibrosis o the lower lobe, there is shifting of the heart and hence the apex beat. Percussion not is also dull over the affected area. Numerous end-inspiratory crepitations are also audible on auscultation.

In severe disease, there may be cyanosis eventually leading to respiratory failure, pulmonary hypertension and cor pulmonale.

Weight Loss
  • Herein we describe a patient who was diagnosed with MPA presenting with radiographic evidence of pulmonary interstitial fibrosis as an early clinical manifestation accompanied by constitutional symptoms such as fever and weight loss.[ncbi.nlm.nih.gov]
  • Symptoms include Shortness of breath A dry, hacking cough that doesn't get better Fatigue Weight loss for no known reason Aching muscles and joints Clubbing, which is the widening and rounding of the tips of the fingers or toes Your doctor may use your[web.archive.org]
  • Lung cancer is usually presented with cough, dyspnea, pain and weight loss, which is overlapping with symptoms of other lung diseases such as pulmonary fibrosis.[ncbi.nlm.nih.gov]
  • Signs and symptoms of pulmonary fibrosis include: Shortness of breath (dyspnea) A dry cough Fatigue Unexplained weight loss Aching muscles and joints The course of pulmonary fibrosis — and the severity of symptoms — can vary considerably from person to[web.archive.org]
  • Pulmonary Fibrosis Lungs Dry cough Shortness of breath Fatigue Weakness Chest pain Loss of appetite Weight loss Clubbed fingers or toes Environmental and occupational exposures Smoking Drug use Rheumatologic disorders Radiation therapy Overview Pulmonary[nm.org]
Hypoxemia
  • In acute conditions, oxygen is given to the patient to reduce hypoxemia and to reverse cyanosis. Treatment with corticosteroids is beneficial in around 30% of the patients.[symptoma.com]
  • He developed dyspnea after 65 days and presented with hypoxemia after 68 days. At presentation, chest computed tomography showed newly developed diffuse ground glass opacities with the pre-existing subpleural reticular shadows.[ncbi.nlm.nih.gov]
  • Arterial hypoxemia in these disorders is primarily caused by ventilation-perfusion mismatching, with further contribution from an intrapulmonary shunt.[web.archive.org]
  • Generally, oxygen therapy can help only people with very low blood-oxygen levels (hypoxemia). To find out if you need supplemental oxygen, speak to your doctor or certified respiratory educator.[lung.ca]
  • Because the lungs don't take in oxygen as well, low blood oxygen levels ( hypoxemia ) can develop. Lack of oxygen can affect the entire body.[bodyandhealth.canada.com]
Constitutional Symptom
  • Herein we describe a patient who was diagnosed with MPA presenting with radiographic evidence of pulmonary interstitial fibrosis as an early clinical manifestation accompanied by constitutional symptoms such as fever and weight loss.[ncbi.nlm.nih.gov]
Cough
  • Lung cancer is usually presented with cough, dyspnea, pain and weight loss, which is overlapping with symptoms of other lung diseases such as pulmonary fibrosis.[ncbi.nlm.nih.gov]
  • If reflux of stomach acid does contribute to cough in IPF, omeprazole might be expected to reduce cough. The purpose of this study is therefore to test whether omeprazole does reduce cough in patients with IPF.[clinicaltrials.gov]
  • Pulmonary fibrosis causes a dry cough and shortness of breath upon physical exertion. The condition can progress to respiratory failure.[britannica.com]
  • An 83-year-old man, who was a former smoker, with anti-ribonucleoprotein (RNP) antibody-positive combined pulmonary fibrosis and emphysema presented with a cough and dyspnea. A chest radiograph showed bilateral pleural effusions.[ncbi.nlm.nih.gov]
  • A 65-year-old man, welder by occupation and an ex-smoker, presented with progressive exertional dyspnoea associated with dry cough noticed for the last four months.[ncbi.nlm.nih.gov]
Dyspnea
  • […] and rise in dyspnea over time predict timing of O 2 prescription.[ncbi.nlm.nih.gov]
  • Dyspnea on exertion (DOE) is a major symptom of CPFE. We report a patient with DOE due to CPFE who was successfully treated with acupuncture. Case report.[ncbi.nlm.nih.gov]
  • A young rural man referred to us with profound icterus and dyspnea after he had eaten three grapes almost 10 days earlier.[ncbi.nlm.nih.gov]
  • Lung cancer is usually presented with cough, dyspnea, pain and weight loss, which is overlapping with symptoms of other lung diseases such as pulmonary fibrosis.[ncbi.nlm.nih.gov]
  • Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive dyspnea.[icd9data.com]
Exertional Dyspnea
  • A 71-year-old man developed exertional dyspnea and was diagnosed with IPF.[ncbi.nlm.nih.gov]
  • The most pronounced clinical feature of pulmonary fibrosis is progressive exertional dyspnea associated with the production of dry cough. On examination, there is gross clubbing of the fingernails and toenails.[symptoma.com]
  • Symptoms and signs develop over months to years and include exertional dyspnea, cough, and fine (Velcro) crackles. Diagnosis is based on history, physical examination, high-resolution CT, and/or lung biopsy, if necessary.[merckmanuals.com]
  • Symptoms of intrinsic diseases Progressive exertional dyspnea is the predominant symptom. Grading the level of dyspnea is useful as a method to gauge the severity of the disease and to follow its course.[web.archive.org]
Cyanosis
  • In acute conditions, oxygen is given to the patient to reduce hypoxemia and to reverse cyanosis. Treatment with corticosteroids is beneficial in around 30% of the patients.[symptoma.com]
  • Other signs on examination may include tachypnoea, cyanosis and also signs of cor pulmonale and right heart failure in the later stages.[patient.info]
  • […] fibrosis, but you might also feel one or more of the following symptoms: loss of stamina loss of appetite fatigue weight loss diffuse chest pain Later on, symptoms can include: shortness of breath without exercise - eating, talking, or just resting cyanosis[bodyandhealth.canada.com]
Chest Pain
  • Pulmonary Fibrosis Lungs Dry cough Shortness of breath Fatigue Weakness Chest pain Loss of appetite Weight loss Clubbed fingers or toes Environmental and occupational exposures Smoking Drug use Rheumatologic disorders Radiation therapy Overview Pulmonary[nm.org]
  • Chest pain. Palpitations. Weight loss. Fatigue. Muscle and joint aches. Clubbing - This is a condition in which the end of the fingers take on the look of an inverted spoon.[verywellhealth.com]
  • Symptoms of pulmonary fibrosis are mainly: Shortness of breath, particularly with exertion Chronic dry, hacking coughing Fatigue and weakness Chest discomfort including chest pain Loss of appetite and rapid weight lossPulmonary fibrosis is suggested by[en.wikipedia.org]
  • These are often the only symptoms of early pulmonary fibrosis, but you might also feel one or more of the following symptoms: loss of stamina loss of appetite fatigue weight loss diffuse chest pain Later on, symptoms can include: shortness of breath without[bodyandhealth.canada.com]
Onset in Infancy
  • All three subjects had a mutation in the previously described autoinflammatory disease called SAVI (stimulator of interferon genes [STING]-associated vasculopathy with onset in infancy).[ncbi.nlm.nih.gov]

Workup

The diagnosis of pulmonary fibrosis is assisted by the use of the following investigations.

  • Chest radiography: Radiography of the chest will show diffuse pulmonary opacities most obvious in the lower zones. The hemidiaphragms are also high due to the pulling effect of fibrosis and the lungs appear small. In advanced cases, the lungs have a honeycomb appearance with diffuse pulmonary shadowing interspersed with small cystic translucencies.
  • High resolution Computerized Tomogaphy (CT): Computerized Tomography is much more helpful than radiographs in the early stages of the disease. It has enhanced the diagnostic accuracy as it can detect lesions that are absent or only slight on chest x-rays [7].
  • Pulmonary function tests: Pulmonary function tests indicate the presence of a restrictive lung disease. There is a proportionate reduction in forced expiratory volume and vital capacity.
  • Arterial blood gases: In pulmonary fibrosis, evaluation of the arterial blood gases demonstrates hypoxemia with normal partial pressure of carbon dioxide.
  • Bronchoalveolar lavage: This shows an increased cellularity in the lavage fluid. Neutrophils, in particular, are significantly high [8].
  • Lung biopsy: Transbronchial lung biopsy is of no use in diagnosing pulmonary fibrosis; however, open lung biopsy may be helpful.
Pulmonary Infiltrate
  • AE was defined as RD with newly acquired bilateral pulmonary infiltrates without evidence of pulmonary infection or other known causes.[ncbi.nlm.nih.gov]
  • infiltrates, and varying . . .[doi.org]
  • It is the most common form of the group of interstitial lung diseases also known as idiopathic interstitial pneumonias, which share clinical features of shortness of breath; diffuse pulmonary infiltrates on imaging; and varying degrees of inflammation[bestpractice.bmj.com]
  • Acute exacerbations were defined as events meeting all of the following criteria: unexplained worsening or development of dyspnea within the previous 30 days; new diffuse pulmonary infiltrates visualized on chest radiography, HRCT, or both, or the development[doi.org]
Bilateral Pulmonary Infiltrate
  • AE was defined as RD with newly acquired bilateral pulmonary infiltrates without evidence of pulmonary infection or other known causes.[ncbi.nlm.nih.gov]
Decreased Lung Compliance
  • Additionally, the static pressure-volume curve is shifted downward and to the right as a result of decreased lung compliance.[physio-pedia.com]
  • Fibrosis leads to decreased lung compliance and increased elastic recoil, which increases the overall work of breathing and inefficient exchange of gases.[medical-dictionary.thefreedictionary.com]

Treatment

In acute conditions, oxygen is given to the patient to reduce hypoxemia and to reverse cyanosis [9].

Treatment with corticosteroids is beneficial in around 30% of the patients. A trial of prednisolone with 40 to 60 mg per day is started in the patients in the patients with progressive disease. The response to treatment is evaluated over a period of 6 to 8 weeks. If the response is good, the dose is reduced to 5 or 10 mg per day. If there is no response, the dosage is tapered and stopped.

Azathioprine or cyclophosphamide may be added if there is no response to prednisolone therapy. They are also used if there is a relapse on reducing the dose of prednisolone [10].

In severe disease, lung transplantation should be considered as the definitive cure.

Prognosis

Once fibrosis has developed, it is not reversible. The prognosis is good if the disease is detected early and its progression stopped through adequate treatment. If fibrosis is extensive, lung transplantation may be the only option.

Etiology

Pulmonary fibrosis can occur due to a number of well-established causes that are listed below [1].

Other less important causes of pulmonary fibrosis include the following.

Epidemiology

The epidemiology of pulmonary fibrosis is not very well documented. It is estimated that up to 5 million people are affected worldwide. The incidence of idiopathic pulmonary fibrosis is higher in people over the age of 50 years.

Sex distribution
Age distribution

Pathophysiology

The initial insult in the development of pulmonary fibrosis is injury to the epithelial surface. This leads to the development of inflammation in the air spaces and the walls of the alveoli, leading to an acute phase of alveolitis. The disease then spreads to the adjacent portions of the interstitium and vasculature, ultimately leading to fibrosis.

It has been suggested that fibrosis is caused by the release of fibronectin and growth factors from alveolar macrophages following tissue injury caused by the release of oxidants from neutrophils, eosinophils and macrophages [6].

Histologically, pulmonary fibrosis is characterized by cellular infiltration along with thickening and fibrosis of the walls of the alveoli. The degree of fibrosis is variable and is often progressive.

Prevention

Idiopathic pulmonary fibrosis can not be prevented. However, pulmonary fibrosis of known etiology can be prevented by avoiding exposure to silica dust, asbestos, coal dust and beryllium. The people who come in contact with these dusts in their occupation should use proper masks.
The overuse of methotrexate, amiodarone, hydralazine, bleomycin and busuphan should be avoided.

Summary

Pulmonary fibrosis is not a single disease entity. It is a part of a group of disorders that are known as interstitial lung diseases. The common features in these disease include:

Patient Information

Pulmonary fibrosis refers to the thickening and hardening of the walls of the alveoli of the lungs. This condition causes a reduction in the amount of expansion and capacity of the lungs. As a result, the function of the lungs is rendered short and the patient becomes breathless upon any kind of exertion. The disease is progressive if not treated properly. Lung transplantation may be the only treatment option in advanced cases.

References

Article

  1. Kronenberger H. [Etiology of pulmonary fibrosis]. Pneumologie. Jun 1991;45(6):439-446.
  2. Kolev K, Burkova T. [Histochemical mechanisms in etiology of pulmonary fibrosis after short-term exposure to a mixture of dust and quartz from copper mine]. Problemi na khigienata. 1982;7:88-97.
  3. Fuzesi K, Karolyi A. [Amiodarone-induced pulmonary fibrosis]. Orvosi hetilap. Jan 5 1997;138(1):43.
  4. Hesterberg TW, Gerriets JE, Reiser KM, Jackson AC, Cross CE, Last JA. Bleomycin-induced pulmonary fibrosis: correlation of biochemical, physiological, and histological changes. Toxicology and applied pharmacology. Sep 15 1981;60(2):360-367.
  5. Isobe K, Hata Y, Sakamoto S, Takai Y, Shibuya K, Homma S. Clinical characteristics of acute respiratory deterioration in pulmonary fibrosis associated with lung cancer following anti-cancer therapy. Respirology. Jan 2010;15(1):88-92.
  6. Crestani B. [Pathophysiology of idiopathic pulmonary fibrosis]. Bulletin de l'Academie nationale de medecine. Feb 2010;194(2):343-351; discussion 351-342.
  7. Oda K, Ishimoto H, Yatera K, et al. High-resolution CT scoring system-based grading scale predicts the clinical outcomes in patients with idiopathic pulmonary fibrosis. Respiratory research. 2014;15:10.
  8. Watters LC, Schwarz MI, Cherniack RM, et al. Idiopathic pulmonary fibrosis. Pretreatment bronchoalveolar lavage cellular constituents and their relationships with lung histopathology and clinical response to therapy. The American review of respiratory disease. Mar 1987;135(3):696-704.
  9. Honma Y, Ogasawara H. [Pathophysiology and treatment of acute exacerbation of pulmonary fibrosis]. Kokyu to junkan. Respiration & circulation. Apr 1986;34(4):377-383.
  10. Kondoh Y, Taniguchi H, Yokoi T, et al. Cyclophosphamide and low-dose prednisolone in idiopathic pulmonary fibrosis and fibrosing nonspecific interstitial pneumonia. The European respiratory journal. Mar 2005;25(3):528-533.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2019-07-11 21:22