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.
Entire Body System
- 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]
Symptoms of pulmonary fibrosis include a chronic (ongoing) dry cough, shortness of breath, chest discomfort, and weight loss. [umiamihealth.org]
- Fever
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]
Loss of appetite and weight, fatigue, fever, weakness, and vague chest pains are common. Infections occur easily, and untreated individuals develop complications that include emphysema, pulmonary infections, or cardiac disease. [healthcentral.com]
Symptoms include: shortness of breath, especially during or after physical activity a persistent dry cough tiredness weight loss and loss of appetite rounded and swollen fingertips and nails (clubbing) fever chills night sweats In cases of IPF, scarring [medicalnewstoday.com]
[…] your provider right away if you have any of the following: Breathing that is harder, faster, or shallower (you are unable to take a deep breath) You have to lean forward when sitting to breathe comfortably Frequent headaches Sleepiness or confusion Fever [uihc.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]
The hypoxemia may also lead an increase in red blood cell (RBC) production (polycythemia). [rtmagazine.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]
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. [medbroadcast.com]
Respiratoric
- 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]
They are often symptomatic for shortness of breath and a dry cough. The diseases can be difficult to diagnose and vary from patient to patient. [academicdepartments.musc.edu]
Forced Coughing Forced coughing is a technique to remove excess mucus from the airways. According to the website Know COPD, mucus buildup is a common problem for those with respiratory illnesses, such as pulmonary fibrosis. [livestrong.com]
Symptoms and signs (eg, subacute dyspnea, nonproductive cough, and Velcro crackles) are nonspecific and usually caused by other, more common disorders. [merckmanuals.com]
- Dyspnea
[…] and rise in dyspnea over time predict timing of O 2 prescription. [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]
It presents initially with dyspnea, loss of appetite, fatigue and dry cough. The most pronounced clinical feature of pulmonary fibrosis is progressive exertional dyspnea associated with the production of dry cough. [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. [msdmanuals.com]
Combined pulmonary fibrosis and emphysema (CPFA) syndrome is characterized by exertional dyspnea, low carbon monoxide diffusion capacity (DLCO), preserved lung volumes, as well as radiological findings of emphysema in the upper lobes and fibrosis in the [dergipark.org.tr]
- Respiratory Insufficiency
Unfortunately, she died as a result of progressive respiratory insufficiency. At autopsy bilateral patchy, sharply circumscribed fibrotic areas in the upper and lower lobes of the lungs were seen with honeycombing. [ncbi.nlm.nih.gov]
Progressive and irreversible lung functional impairment leads to chronic respiratory insufficiency with a severely impaired quality of life. [dovepress.com]
Relentless progression of the disease with eventual respiratory insufficiency is the rule, and the average survival time is about four years. Lung transplantation for highly selected patients with end-stage pulmonary fibrosis has been reported. [healthcentral.com]
Pulmonary fibrosis is a general term used to describe the group of fibrosing interstitial lung diseases that are characterized by progressive scarring of the alveolar interstitium, often leading to hypoxemic respiratory insufficiency. [ucdenver.edu]
- Clubbed Finger
Symptoms can include: shortness of breath a persistent dry cough tiredness loss of appetite and weight loss rounded and swollen fingertips (clubbed fingers) Many people ignore their breathlessness at first and blame it on getting old or being out of shape [nhs.uk]
Symptoms can include: shortness of breath a persistent dry cough tiredness loss of appetite and weight loss rounded and swollen fingertips ( clubbed fingers ) Many people ignore their breathlessness at first and blame it on getting old or being out of [nhsdirect.wales.nhs.uk]
Gastrointestinal
- Failure to Thrive
Failure to thrive was a common finding at presentation and occurred despite a normal birth weight in most cases. Weight gain can be achieved if high energy supplements are provided. [adc.bmj.com]
Cardiovascular
- Chest Pain
Loss of appetite and weight, fatigue, fever, weakness, and vague chest pains are common. Infections occur easily, and untreated individuals develop complications that include emphysema, pulmonary infections, or cardiac disease. [healthcentral.com]
Symptoms may include any of the following: Occasional chest pain Cough, usually dry Shortness of breath during activity—this symptom lasts for months or years, and over time may also occur when at rest Tests and examinations for IPF Your provider will [uihc.org]
Chest pain is uncommon in most instances of the disease, but pleuritic chest pain can occur in patients with rheumatoid arthritis, systemic lupus erythematosus, and some drug-induced disorders. [web.archive.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 [medbroadcast.com]
- 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]
The physical exam may find that you have: Abnormal breath sounds called crackles Bluish skin (cyanosis) around the mouth or fingernails due to low oxygen (with advanced disease) Enlargement and curving of the fingernail bases, called clubbing (with advanced [uihc.org]
[…] fibrosis, but others may be present : Loss of endurance during physical or daily activities Loss of appetite Tiredness Weight Loss A diffuse pain in the chest Thereafter : A shortness of breath without physical effort, eating, talking or simply resting Cyanosis [pq.lung.ca]
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 [medbroadcast.com]
Skin
- Skin Disease
In addition, they examined tissue samples of diseased and healthy areas of skin from six patients with fibrotic skin diseases. [helmholtz-muenchen.de]
But data from Northwestern’s extensive research program in scleroderma — an autoimmune disease closely tied to pulmonary fibrosis that results in hardening of the skin — suggested immune cells may actually play an important role. [news.feinberg.northwestern.edu]
Musculoskeletal
- Osteoporosis
Erbagci AB, Araz M, Erbagci A, Tarakcioglu M, Namiduru ES: Serum prolidase activity as a marker of osteoporosis in type 2 diabetes mellitus. Clin Biochem 2002, 35, 263–268. [advances.umed.wroc.pl]
Taking steroid medication, especially for a prolonged period of time, can cause side effects including: weight gain osteoporosis (brittle bones, which especially affects women after the menopause) cataracts (cloudy patches in the lens in the eye) glaucoma [hse.ie]
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.
X-Ray
- Pulmonary Infiltrate
pulmonary infiltrates, and varying. .. [doi.org]
AE was defined as RD with newly acquired bilateral pulmonary infiltrates without evidence of pulmonary infection or other known causes. [ncbi.nlm.nih.gov]
[…] tool=bestpractice.com 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 [bestpractice.bmj.com]
Acute exacerbation of IPF was defined using the following criteria proposed by Kondoh et al. 7 and Akira et al. 8. 1) Acute worsening of dyspnoea within 1 month of presentation. 2) New pulmonary infiltrates on a chest radiograph or computed tomography [erj.ersjournals.com]
- Pulmonary Infiltrates
pulmonary infiltrates, and varying. .. [doi.org]
AE was defined as RD with newly acquired bilateral pulmonary infiltrates without evidence of pulmonary infection or other known causes. [ncbi.nlm.nih.gov]
[…] tool=bestpractice.com 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 [bestpractice.bmj.com]
Acute exacerbation of IPF was defined using the following criteria proposed by Kondoh et al. 7 and Akira et al. 8. 1) Acute worsening of dyspnoea within 1 month of presentation. 2) New pulmonary infiltrates on a chest radiograph or computed tomography [erj.ersjournals.com]
- Bilateral Pulmonary Infiltrates
AE was defined as RD with newly acquired bilateral pulmonary infiltrates without evidence of pulmonary infection or other known causes. [ncbi.nlm.nih.gov]
Pulmonary Function Test
- Decreased Lung Compliance
Vital capacity, functional residual capacity, total lung capacity, and forced vital capacity (FVC) all are reduced. 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]
- Restrictive Pulmonary Function
All patients who were assessed with respiratory function testing showed a restrictive pulmonary defect. [adc.bmj.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].
- Exposure to inorganic dusts causes fibrosing lung diseases that are referred to as pneumoconiosis. These include silicosis, anthracosis, asbestosis and berylliosis and are caused by silica dust, coal dust, asbestosis and beryllium respectively [2].
- The use of certain drugs such as methotrexate, amiodarone, bleomycin, busuphan and hydralazine is also associated with the development of pulmonary fibrosis [3] [4].
- Aspiration of gastric fluid into the lungs can also lead to the development of pulmonary fibrosis.
- Overdose of radiation to the chest can also cause pulmonary fibrosis [5].
Other less important causes of pulmonary fibrosis include the following.
- Pulmonary fibrosis may be idiopathic, in which case it is also known as cryptogenic fibrosing alveolitis.
- Pulmonary fibrosis may be present along with certain connective tissue disorders such as rheumatoid arthritis, systemic lupus erythematosis, ankylosing spondylitis and polymyositis.
- Pulmonary fibrosis is also a rare feature of with inflammatory bowel diseases, sarcoidosis, amyloidosis and biliary cirrhosis.
- Eosinophilic pneumonias such as allergic bronchopulmonary aspergillosis may also lead to the development of pulmonary fibrosis.
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.
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:
- Thickening of the alveolar walls by fibrosis and edema
- Increased stiffness of the lungs associated with exertional dyspnea
- Maldistribution of pulmonary ventilation and perfusion leading to hypoxemia
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
- Kronenberger H. [Etiology of pulmonary fibrosis]. Pneumologie. Jun 1991;45(6):439-446.
- 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.
- Fuzesi K, Karolyi A. [Amiodarone-induced pulmonary fibrosis]. Orvosi hetilap. Jan 5 1997;138(1):43.
- 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.
- 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.
- Crestani B. [Pathophysiology of idiopathic pulmonary fibrosis]. Bulletin de l'Academie nationale de medecine. Feb 2010;194(2):343-351; discussion 351-342.
- 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.
- 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.
- Honma Y, Ogasawara H. [Pathophysiology and treatment of acute exacerbation of pulmonary fibrosis]. Kokyu to junkan. Respiration & circulation. Apr 1986;34(4):377-383.
- 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.