Restrictive lung disease

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Restrictive lung disease
Other namesRestrictive ventilatory defect [1]
Specialty Pulmonology

Restrictive lung diseases are a category of extrapulmonary, pleural, or parenchymal respiratory diseases that restrict lung expansion, [2] resulting in a decreased lung volume, an increased work of breathing, and inadequate ventilation and/or oxygenation. Pulmonary function test demonstrates a decrease in the forced vital capacity.

Contents

Presentation

Due to the chronic nature of this disease, the leading symptom of restrictive lung disease is progressive exertional dyspnea. [3]   For acute on chronic cases, shortness of breath, cough, and respiratory failure are some of the more common signs. [3]

Causes

Restrictive lung diseases may be due to specific causes which can be intrinsic to the parenchyma of the lung, or extrinsic to it. [3]

Intrinsic

Many cases of restrictive lung disease are idiopathic (have no known cause). Still, there is generally pulmonary fibrosis. [5] Examples are:

Conditions specifically affecting the interstitium are called interstitial lung diseases.

Extrinsic

Pathophysiology

In normal respiratory function, the air flows in through the upper airway, down through the bronchi and into the lung parenchyma (the bronchioles down to the alveoli) where gas exchange of carbon dioxide and oxygen occurs. [7] During inspiration, the lungs expand to allow airflow into the lungs and thereby increasing total volume. After inspiration follows expiration during which the lungs recoil and push air back out of the pulmonary pathway. Lung compliance is the difference of volume during inspiration and expiration. [7]

Restrictive lung disease is characterized by reduced lung volumes, and therefore reduced lung compliance, either due to an intrinsic reason, for example a change in the lung parenchyma, or due to an extrinsic reason, for example diseases of the chest wall, pleura, or respiratory muscles. [3] Generally, intrinsic causes are from lung parenchyma diseases that cause inflammation or scarring of the lung tissue, such as interstitial lung disease or pulmonary fibrosis, or from having the alveoli air spaces filled with external material such as debris or exudate in pneumonitis. [3] As some diseases of the lung parenchyma progress, the normal lung tissue can be gradually replaced with scar tissue that is interspersed with pockets of air. [5] This can lead to parts of the lung having a honeycomb-like appearance. The extrinsic causes result in lung restriction, impaired ventilatory function, and even respiratory failure due to the diseases that effect the lungs ability to create a change in lung volumes during respiration due to the diseases of the systems stated above. [3]

Diagnosis

In restrictive lung disease, both forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) are reduced, however, the decline in FVC is more than that of FEV1, resulting in a higher than 80% FEV1/FVC ratio. In obstructive lung disease however, the FEV1/FVC is less than 0.7, indicating that FEV1 is significantly reduced when compared to the total expired volume. This indicates that the FVC is also reduced, but not by the same ratio as FEV1. [8]

One definition requires a total lung capacity which is 80% or less of the expected value. [9]

Management

Medical treatment for restrictive lung disease is normally limited to supportive care since both the intrinsic and extrinsic causes can have irreversible effects on lung compliance. [10] The supportive therapies focus on maximizing pulmonary function and preserving activity tolerance through oxygen therapy, bronchodilators, inhaled beta-adrenergic agonists, and diuretics. [10] Because there is no effective treatment for restrictive lung disease, prevention is key. [10]

See also

Related Research Articles

<span class="mw-page-title-main">Lung volumes</span> Volume of air in the lungs

Lung volumes and lung capacities refer to the volume of air in the lungs at different phases of the respiratory cycle.

<span class="mw-page-title-main">Asbestosis</span> Pneumoconiosis caused by inhalation and retention of asbestos fibers

Asbestosis is long-term inflammation and scarring of the lungs due to asbestos fibers. Symptoms may include shortness of breath, cough, wheezing, and chest tightness. Complications may include lung cancer, mesothelioma, and pulmonary heart disease.

<span class="mw-page-title-main">Spirometry</span> Pulmonary function test

Spirometry is the most common of the pulmonary function tests (PFTs). It measures lung function, specifically the amount (volume) and/or speed (flow) of air that can be inhaled and exhaled. Spirometry is helpful in assessing breathing patterns that identify conditions such as asthma, pulmonary fibrosis, cystic fibrosis, and COPD. It is also helpful as part of a system of health surveillance, in which breathing patterns are measured over time.

<span class="mw-page-title-main">Interstitial lung disease</span> Group of diseases

Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of respiratory diseases affecting the interstitium and space around the alveoli of the lungs. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. Ordinarily, the body generates just the right amount of tissue to repair damage, but in interstitial lung disease, the repair process is disrupted, and the tissue around the air sacs (alveoli) becomes scarred and thickened. This makes it more difficult for oxygen to pass into the bloodstream. The disease presents itself with the following symptoms: shortness of breath, nonproductive coughing, fatigue, and weight loss, which tend to develop slowly, over several months. The average rate of survival for someone with this disease is between three and five years. The term ILD is used to distinguish these diseases from obstructive airways diseases.

<span class="mw-page-title-main">Pulmonary fibrosis</span> Disease that causes scarring of the lungs

Pulmonary fibrosis is a condition in which the lungs become scarred over time. Symptoms include shortness of breath, a dry cough, feeling tired, weight loss, and nail clubbing. Complications may include pulmonary hypertension, respiratory failure, pneumothorax, and lung cancer.

<span class="mw-page-title-main">Hypersensitivity pneumonitis</span> Medical condition

Hypersensitivity pneumonitis (HP) or extrinsic allergic alveolitis (EAA) is a syndrome caused by the repetitive inhalation of antigens from the environment in susceptible or sensitized people. Common antigens include molds, bacteria, bird droppings, bird feathers, agricultural dusts, bioaerosols and chemicals from paints or plastics. People affected by this type of lung inflammation (pneumonitis) are commonly exposed to the antigens by their occupations, hobbies, the environment and animals. The inhaled antigens produce a hypersensitivity immune reaction causing inflammation of the airspaces (alveoli) and small airways (bronchioles) within the lung. Hypersensitivity pneumonitis may eventually lead to interstitial lung disease.

<span class="mw-page-title-main">Pneumonitis</span> General inflammation of lung tissue

Pneumonitis describes general inflammation of lung tissue. Possible causative agents include radiation therapy of the chest, exposure to medications used during chemo-therapy, the inhalation of debris, aspiration, herbicides or fluorocarbons and some systemic diseases. If unresolved, continued inflammation can result in irreparable damage such as pulmonary fibrosis.

<span class="mw-page-title-main">Respiratory disease</span> Disease of the respiratory system

Respiratory diseases, or lung diseases, are pathological conditions affecting the organs and tissues that make gas exchange difficult in air-breathing animals. They include conditions of the respiratory tract including the trachea, bronchi, bronchioles, alveoli, pleurae, pleural cavity, the nerves and muscles of respiration. Respiratory diseases range from mild and self-limiting, such as the common cold, influenza, and pharyngitis to life-threatening diseases such as bacterial pneumonia, pulmonary embolism, tuberculosis, acute asthma, lung cancer, and severe acute respiratory syndromes, such as COVID-19. Respiratory diseases can be classified in many different ways, including by the organ or tissue involved, by the type and pattern of associated signs and symptoms, or by the cause of the disease.

Airway obstruction is a blockage of respiration in the airway that hinders the free flow of air. It can be broadly classified into being either in the upper airway (UPA) or lower airway (LOA).

Occupational lung diseases are work-related, lung conditions that have been caused or made worse by the materials a person is exposed to within the workplace. It includes a broad group of diseases, including occupational asthma, industrial bronchitis, chronic obstructive pulmonary disease (COPD), bronchiolitis obliterans, inhalation injury, interstitial lung diseases, infections, lung cancer and mesothelioma. These diseases can be caused directly or due to immunological response to an exposure to a variety of dusts, chemicals, proteins or organisms.

<span class="mw-page-title-main">Idiopathic pulmonary fibrosis</span> Medical condition

Idiopathic pulmonary fibrosis (IPF), or (formerly) fibrosing alveolitis, is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue. It is a type of chronic scarring lung disease characterized by a progressive and irreversible decline in lung function. The tissue in the lungs becomes thick and stiff, which affects the tissue that surrounds the air sacs in the lungs. Symptoms typically include gradual onset of shortness of breath and a dry cough. Other changes may include feeling tired, and abnormally large and dome shaped finger and toenails. Complications may include pulmonary hypertension, heart failure, pneumonia or pulmonary embolism.

<span class="mw-page-title-main">Usual interstitial pneumonia</span> Medical condition

Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. The scarring (fibrosis) involves the pulmonary interstitium. UIP is thus classified as a form of interstitial lung disease.

<span class="mw-page-title-main">Obstructive lung disease</span> Category of respiratory disease characterized by airway obstruction

Obstructive lung disease is a category of respiratory disease characterized by airway obstruction. Many obstructive diseases of the lung result from narrowing (obstruction) of the smaller bronchi and larger bronchioles, often because of excessive contraction of the smooth muscle itself. It is generally characterized by inflamed and easily collapsible airways, obstruction to airflow, problems exhaling, and frequent medical clinic visits and hospitalizations. Types of obstructive lung disease include; asthma, bronchiectasis, bronchitis and chronic obstructive pulmonary disease (COPD). Although COPD shares similar characteristics with all other obstructive lung diseases, such as the signs of coughing and wheezing, they are distinct conditions in terms of disease onset, frequency of symptoms, and reversibility of airway obstruction. Cystic fibrosis is also sometimes included in obstructive pulmonary disease.

<span class="mw-page-title-main">Pulmonary function testing</span> Test to evaluate respiratory system

Pulmonary function testing (PFT) is a complete evaluation of the respiratory system including patient history, physical examinations, and tests of pulmonary function. The primary purpose of pulmonary function testing is to identify the severity of pulmonary impairment. Pulmonary function testing has diagnostic and therapeutic roles and helps clinicians answer some general questions about patients with lung disease. PFTs are normally performed by a pulmonary function technician, respiratory therapist, respiratory physiologist, physiotherapist, pulmonologist, or general practitioner.

<span class="mw-page-title-main">FEV1/FVC ratio</span> Ratio used in the diagnosis of lung disease

The FEV1/FVC ratio, also called modified Tiffeneau-Pinelli index, is a calculated ratio used in the diagnosis of obstructive and restrictive lung disease. It represents the proportion of a person's vital capacity that they are able to expire in the first second of forced expiration (FEV1) to the full, forced vital capacity (FVC). FEV1/FVC ratio first proposed by E.A. Haensler in 1950. The FEV1/FVC index should not be confused with the FEV1/VC index as they are different, although both are intended for diagnosing airway obstruction. Current recommendations for diagnosing pulmonary function recommend using the modified Tiffeneau-Pinelli index. This index is recommended to be represented as a decimal fraction with two digits after the decimal point.

Pulmonary rehabilitation, also known as respiratory rehabilitation, is an important part of the management and health maintenance of people with chronic respiratory disease who remain symptomatic or continue to have decreased function despite standard medical treatment. It is a broad therapeutic concept. It is defined by the American Thoracic Society and the European Respiratory Society as an evidence-based, multidisciplinary, and comprehensive intervention for patients with chronic respiratory diseases who are symptomatic and often have decreased daily life activities. In general, pulmonary rehabilitation refers to a series of services that are administered to patients of respiratory disease and their families, typically to attempt to improve the quality of life for the patient. Pulmonary rehabilitation may be carried out in a variety of settings, depending on the patient's needs, and may or may not include pharmacologic intervention.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a diffuse parenchymal lung disease which often presents with symptoms of cough and shortness of breath. The pathological definition published by the World Health Organization is “a generalized proliferation of scattered single cells, small nodules, or linear proliferations of pulmonary neuroendocrine (PNE) cells that may be confined to the bronchial and bronchiolar epithelium.” The true prevalence of this disease is not known. To date, just under 200 cases have been reported in the literature. However, with an increase in recognition of this disease by radiologists and pulmonologists, the number of cases has been increasing. DIPNECH predominantly affects middle-aged women with slowly progressive lung obstruction. DIPNECH is usually discovered in one of two ways: 1) as an unexpected finding following a lung surgery; or 2) by evaluation of a patient in a pulmonary clinic with longstanding, unexplained symptoms.

<span class="mw-page-title-main">Emphysema</span> Medical condition

Emphysema is any air-filled enlargement in the body's tissues. Most commonly emphysema refers to the enlargement of air spaces (alveoli) in the lungs, and is also known as pulmonary emphysema.

References

  1. ↑ Johns Hopkins School of Medicine's Interactive Respiratory Physiology > Restrictive Ventilatory Defect Retrieved on February 25, 2010
  2. ↑ Sharma, Sat. "Restrictive Lung Disease" . Retrieved 2008-04-19.
  3. 1 2 3 4 5 6 "eMedicine - Restrictive Lung Disease : Article by Sat Sharma" . Retrieved 2008-11-21.
  4. ↑ Amaral, André F.S.; Coton, Sonia; Kato, Bernet; Tan, Wan C.; Studnicka, Michael; Janson, Christer; Gislason, Thorarinn; Mannino, David; Bateman, Eric D.; Buist, Sonia; Burney, Peter G.J. (October 2015). "Tuberculosis associates with both airflow obstruction and low lung function: BOLD results". European Respiratory Journal. 46 (4): 1104–1112. doi:10.1183/13993003.02325-2014. PMC   4594762 . PMID   26113680.
  5. 1 2 PULMONARY FUNCTION TESTS A Workshop on Simple Spirometry & Flow Volume Loops. Archived 2010-03-31 at the Wayback Machine Dr. S. Osborne, Dept. Cellular & Physiological Sciences. Mars 2009
  6. 1 2 eMedicine Specialties > Pulmonology > Interstitial Lung Diseases > Restrictive Lung Disease Author: Lalit K Kanaparthi, MD, Klaus-Dieter Lessnau, MD, Sat Sharma, MD. Updated: Jul 27, 2009
  7. 1 2 Capriotti, Theresa (2016). Pathophysiology : introductory concepts and clinical perspectives. Frizzell, Joan Parker. Philadelphia. ISBN   978-0-8036-1571-7. OCLC   900626405.{{cite book}}: CS1 maint: location missing publisher (link)
  8. ↑ Lee, H., Lim, S., Kim, J., Ha, H., & Park, H. (2015). Comparison Of Various Pulmonary Function Parameters In The Diagnosis Of Obstructive Lung Disease In Patients With Normal Fev1/FVC And Low FVC. American Journal of Respiratory and Critical Care Medicine, 191, American Journal of Respiratory and Critical Care Medicine, 2015, Vol.191.
  9. ↑ Brack T, Jubran A, Tobin MJ (May 2002). "Dyspnea and decreased variability of breathing in patients with restrictive lung disease". Am. J. Respir. Crit. Care Med. 165 (9): 1260–4. doi:10.1164/rccm.2201018. PMID   11991875.
  10. 1 2 3 Focus on adult health : medical-surgical nursing. Honan, Linda, 1955- (1st ed.). Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins. 2013. ISBN   978-1-58255-877-6. OCLC   756895022.{{cite book}}: CS1 maint: others (link)
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