Restrictive lung disease

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at November 1, 2016
StartDiseasesRestrictive lung disease

Restrictive lung disease is a type of respiratory disease. It inhibits a person's ability to expand their lungs when breathing. This causes a reduction in lung volume. A person with this condition will have to work harder to breathe. Their body will have significantly inadequate oxygenation. 

Contents

Definition & Facts

Restrictive lung disease can be caused by abnormalities of the lung parenchyma (the part of the lung that includes the alveoli and which is involved in gas exchange) or various diseases affecting other structures outside of the lungs such as the pleura and chest wall.

Diseases affecting the lung parenchyma are called intrinsic lung diseases. When this connective tissue becomes damaged, it can result in inflammation. Intrinsic lung diseases result when damage to the lung's parenchyma become chronic. Extrinsic lung diseases involve disorders of the chest wall and respiratory muscles.

Restrictive lung diseases result in a reduced amount of blood flowing to a person's lungs. This reduction will lower the amount of oxygen available for a person's body to use. This can result in shortness of breath, chronic fatigue, and more. The difference between restrictive lung disease and obstructive lung disease is that restrictive lung diseases cause reduced lung capacity, whereas obstructive lung diseases are not.

Symptoms & Complaints

When an individual is in the early stages of experiencing restrictive lung disease, they will have shortness of breath. This will only happen after periods of physical exertion. As the disease advances, a person may experience breathlessness after only minimal activity. It could occur even when they are at rest.

Uncontrollable coughing is also a common symptom. People could have a cough that is dry or involves white sputum. As the disease increases, people experience anxiety as well as depression. It will result in people having serious limitations with their lifestyle.

Many individuals also experience chest pain. This is often the case with people who have other illnesses such as systemic lupus, rheumatoid arthritis as well as disorders resulting from drug use.

People often experience wheezing. This occurs when individuals inhale and their lungs produce whistling sounds. This symptom will become worse as the damage to the lungs advances.

Another symptom is recurrent lung infections. It's also common for people to have significant unexplained weight loss. In its advanced stages, individuals could experience respiratory failure as well as pulmonary hypertension and more. 

Causes

There are a number of different reasons a person could develop restrictive lung disease. Many of the causes fall into known factors for developing it. This includes connective tissue diseases, primary diseases of the lung, sarcoidosis, fibrosiss, drug-induced lung disease, and more. There are also various pulmonary diseases that could cause this condition to occur. They can cause the respiratory muscles within the chest wall to fail and not be able to function properly. When these structures are affected, the ventilatory function will be impacted negatively. Possible causes include:

There are also a number of cases where restrictive lung disease is determined to be idiopathic. This means a person's condition has no known cause. 

Diagnosis & Tests

When a patient is suspected of having restrictive lung disease, the initial evaluation will involve a physician getting a complete medical history. It will include reviewing any past systemic conditions that may have occurred. A patient's habits, hobbies, occupation, travel destinations as well as HIV risk factors and more are important for identification of the condition.

Chest X-rays is used to identify types of lung disorders. A physician could also order a patient to undergo complete pulmonary function testing. This could involve lung volume testing as well as arterial blood gas measurements and more. A pulmonary function lung test can identify the level of severity of the restrictive lung disease.

A small piece of lung tissue could be removed from a patient in a procedure known as a lung biopsy. There are three different types utilized. During a bronchoscopic biopsy, a lighted instrument in placed through a patient's nose or mouth and down into their airway to take a sample of their lung tissue. With a fine-needle biopsy, a long needle is placed into the chest wall of a patient to retrieve a lung tissue sample. With an open biopsy, a surgeon makes an incision between a patient's ribs to obtain the sample. 

Treatment & Therapy

There is presently no known cure for restrictive lung disease. This type of condition usually results in damage to the lung tissue that is irreversible. The treatment a person receives will depend on their specific medical situation. If it is caught in time, the progression of the disease can be stopped and successfully managed. Treatment will be based on an individual's specific diagnosis.

Corticosteroids are often tried first, but they provide many negative side effects. Research has shown that up to 30 percent of patients experience improvement using this method. Chemotherapy is often used with individuals who don't respond to corticosteroids. It is also advised that patients with the disease avoid any type of lung irritants in their environment.

Some patients with restrictive lung diseases are also taught effective coughing techniques. This could be deep coughing designed to more effectively remove mucus from the lungs. Another method is the huff cough technique. This is where stomach muscles (the diaphragm) are activated for mucus removal.

Supplemental oxygen may be utilized when a patient's blood oxygen levels are low. It will help with their feelings of exhaustion and breathlessness. A bronchodilator may be used. This is a type of medication that is inhaled. It is able to smooth the muscles and increase the flow of air into the lungs.

Prevention & Prophylaxis

There are some things a person can do to decrease their risk of developing restrictive lung disease. Avoiding tobacco smoke is important. This includes smoking as well as secondhand smoke.

Obesity also has a negative impact on a person's respiratory physiology. Adopting a healthy diet and regularly exercising may be able to reduce the risk of restrictive lung disease.

Because occupations also play a role in the development of this disease, workers need to be vigilant in wearing safety gear. Welders, as well as coal miners, and others working in an environment with polluted air have a greater risk for developing restrictive lung disease.