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Obstructive And Restrictive Lung Disease

Doctors may classify lung conditions as obstructive lung disease or restrictive lung disease. Obstructive lung diseases include conditions that make it hard to exhale all the air in your lungs. People with restrictive lung disease have trouble fully expanding their lungs with air.

Obstructive and restrictive lung disease share the same main symptom: shortness of breath when you exert yourself.

People with obstructive lung disease have shortness of breath because it's hard for them to exhale all the air from their lungs. Because of damage to the lungs or narrowing of the airways inside the lungs, the exhaled air comes out more slowly than it should. At the end of a full exhalation, too much air may linger in the lungs.

The most common causes of obstructive lung disease are:

Obstructive lung disease makes it harder to breathe, especially when you're exercising or otherwise being active. As your breathing rate increases, you have less time to breathe all the air out before you inhale again.

People with restrictive lung disease can't fully fill their lungs with air. Their lungs are restricted from fully expanding.

Restrictive lung disease most often results from a condition that causes stiffness in the lungs themselves. In other cases, stiffness of the chest wall, weak muscles, or damaged nerves may restrict how much your lungs can expand.

Some conditions causing restrictive lung disease are:

Most often, people with obstructive or restrictive lung disease see a doctor because they feel short of breath.

Doctors identify restrictive and obstructive lung diseases using pulmonary function tests. In pulmonary function testing, you blow air forcefully through a mouthpiece. As you go through various breathing exercises, a machine records the volume and flow of air through your lungs. Pulmonary function testing can show whether you have obstructive lung disease or restrictive lung disease, as well as how serious they are.

A doctor's interview (including smoking history), physical exam, and lab tests may provide additional clues to the cause of obstructive lung disease or restrictive lung disease.

Imaging tests are almost always part of the process of diagnosing restrictive and obstructive lung disease. These may include:

Chest X-rays

Computed tomography (CT scan) of the chest

Sometimes, your doctor may recommend a bronchoscopy to help them figure out what's causing obstructive or restrictive lung disease. In a bronchoscopy, your doctor uses an endoscope (a flexible tube with a camera and tools on its tip) to look inside your airways and take samples of lung tissue (biopsy).

Obstructive lung disease and restrictive lung disease cause shortness of breath. In their early stages, you get short of breath only when you exert yourself. If the underlying lung condition gets worse, it might happen with very little activity, or even when you're at rest.

Cough is a common symptom for both types of lung diseases. Usually, the cough is dry or brings up white sputum (mucus). People with chronic bronchitis, a type of obstructive lung disease, may cough up larger amounts of colored sputum.

 Many people with obstructive  or restrictive lung disease also have symptoms of depression and anxiety. These symptoms are more likely when lung disease seriously limits your activities and lifestyle.

Obstructive lung disease treatments work by helping to open narrowed airways. Your airways may be narrowed because you have spasms in the smooth muscles that line their walls. These are called bronchospasms.

Medicines that relax these smooth muscles and improve airflow are called bronchodilators and are inhaled. These include:

Theophylline (Theo-Dur and other brand names) is a rarely used bronchodilator taken as an oral tablet.

Inflammation also contributes to airway narrowing in obstructive lung disease. Inflamed airway walls may be swollen and filled with mucus, obstructing airflow. Various medicines help reduce inflammation in obstructive lung disease, including:

A program of regular exercise will improve symptoms of breathlessness in virtually all people with obstructive lung disease. Oxygen therapy may be necessary for some people.

In severe cases of end-stage, life-threatening obstructive lung disease, lung transplantation can be considered as a treatment option.

Few medicines are available to treat most causes of restrictive lung disease.

The FDA has approved two drugs, Esbriet (pirfenidone) and Ofev (nintedanib)to treat idiopathic pulmonary fibrosis. They act on several pathways that may be involved in causing scars to your lung tissue. Studies show both medications can slow down restrictive lung disease, as measured by pulmonary function tests.

If you have restrictive lung disease caused by long-lasting inflammation, your doctor may prescribe medicines that suppress your immune system, like:

You might also need oxygen therapy. Mechanical breathing assistance may help some people who have breathing problems due to restrictive lung disease. Non-invasive positive pressure ventilation (BiPAP) uses a tight-fitting mask and a pressure generator to help you breathe. BiPAP can help people with obesity hypoventilation syndrome and some nerve or muscle conditions that cause restrictive lung disease.

In cases of obesity-related lung disease, weight loss and exercise can help reduce breathing problems.

If you have very serious restrictive lung disease (such as idiopathic pulmonary fibrosis), you may need a lung transplant.

Regular exercise improves shortness of breath and quality of life in almost everyone with restrictive lung disease.

Rare Lung Disease: Genetics Reveals Cause Of PCD

These exciting and unexpected insights into ciliopathies were only possible due to the collaborative partnership between the patients in our studies, their clinicians and researchers with interdisciplinary expertise from across the world

Pleasantine Mill

Mutations in over 50 genes account for 70% of PCD cases, with the remainder of cases unexplained. Using next-generation DNA sequencing approaches, researchers were surprised to find recurrent mutations in the beta-tubulin gene TUBB4B, in twelve different PCD patients, that were not found in their parents. Moreover, where on the TUBB4B protein these recurrent mutations were found resulted in very different clinical presentations. 

TUBB4B is one of the building blocks of microtubules – scaffold-like structures involved in cell shape, movement, and cell division and critical for cilia formation. Each link of the tubulin 'chain' is composed of one alpha and one beta tubulin protein.  Humans have 10 types of beta tubulin, including TUBB4B. The different tubulin subunits seem to not be interchangeable and may have different roles as mutations in one, such as TUBB4B, cannot be always compensated for by the others. 

Researchers used both computational and laboratory-based approaches to resolve how mutations in different parts of the TUBB4B molecule differently affect how the tubulin building blocks assemble and disrupt cilia form or function. They found mutations at different interfaces between tubulin links result in different patient outcomes, such as early vision loss, lung disease, or both conditions simultaneously. The researchers were able to show that one mutant copy of TUBB4B was capable of over-riding the healthy copy to disrupt microtubule and cilia formation, a phenomenon called the 'dominant negative' effect, previously not observed in PCD. 

In addition to improving the diagnosis and genetic counselling of PCD patients and their families, this novel disease mechanism will require different therapeutic strategies to treat these patients. "These exciting and unexpected insights into ciliopathies were only possible due to the collaborative partnership between the patients in our studies, their clinicians and researchers with interdisciplinary expertise from across the world," said Professor Pleasantine Mill, Chair of Cilia Biology at the University of Edinburgh, who co-led the study. Study authors Mill, Shoemark and Mitchison are co-leads, with other UK researchers, for a newly funded £9.4M UK-wide LifeArc Centre for Rare Respiratory Diseases, which aims to improve molecular diagnoses as well accelerate molecular insights to develop new therapies for rare lung diseases, like PCD. 

"We know firsthand the unmet need from significant burden of disease for rare lung diseases like PCD. We hope this work and our newly funded collaborative centre will provide the foundation to raise awareness, develop much-needed treatments and use them to benefit these patients," added Prof. Amelia Shoemark. 

Source: University of Edinburgh

Lung Disease

Increasing level of pollution has made breathing in urban areas very difficult, raising the incidence of respiratory problems. Out of the world's top 20 cities with polluted air, 13 belong to India and according to the World health Organisation (WHO) report published in 2011, lung diseases rank 2ndin the list of top 20 causes of death. Naturally, lung disease is a big health issue that needs to be addressed. Here are some facts about lung disease you should know about.

To understand the condition of your lungs, you need to first understand how the lungs function. Lungs are soft organs present on both right and left side of the chest. They are protected by a had covering called the ribcage. Each lung is made of sections called lobes. The main function of the lungs is to make oxygen available for purifying blood and to and eliminate carbondioxide from impure blood or deoxygenated blood. So when you breathe, air travels down your windpipe (trachea). The windpipe bifurcates with each branch entering each of the lungs. Into the lungs, the windpipe is further branched to tubes called bronchioles that end in small sacs called alveoli, where the inhaled air reaches for delivering oxygen.

A lung disease or disorder occurs in the various structures of lungs and disrupts its functioning. It can vary from a mild common cold to life-threatening conditions like pneumonia and cancer. Lung disease can be caused by infections, inflammation, obstruction of airways, decreased lung function, problems related to pulmonary circulation, etc. Smoking, infections, air pollution, genetics, exposure to asbestos, etc. Can cause various lung diseases.

Types

The common as well as rare types of lung disease include -

Causes

'In any form of lung disease mainly the breathing gets affected,' says Dr Animesh Arya, Senior Consultant, Respiratory Medicine, Delhi based Sri Balaji Action Medical Institute. Here are some common forms of lung diseases he explains. 1. Asthma:It is the most common lung disease triggered by allergies, infections and pollutants that harm the airways and cause inflammation. 2. Acute bronchitis: Bronchitis is the inflammation of the air passage. Acute bronchitis is caused by viral or bacterial respiratory infections. 3. Chronic Obstructive Pulmonary Disease (COPD): It a group of diseases that causes breathing difficulties and interfere with the exhalation process of breathing. 4. Chronic bronchitis: It is a type of COPD caused mainly due to smoking. 5. Emphysema: It is a COPD caused by environmental factors and may also be triggered by allergens or organisms that cause diseases like pneumonia and tuberculosis. Apart from these common forms of lung diseases, other rare lung diseases include: 6. Cystic fibrosis: It is rare lung disease caused by genetic mutation that results in excess mucus production causing blockage of airways. 7. Acute respiratory distress syndrome (ARDS): It is a medical emergency caused as a result of lung injury due to a severe illness. 8. Lung cancer: It can affect any part of the lung. It is mainly caused due to smoking. Here are  10 common symptoms of lung cancer you need to know. 9. Interstitial lung disease: This is a group of diseases affecting the interstitium, a thin lining separating the air sacs.

Symptoms

'Bronchitis, asthma, COPD, lung infections and pulmonary fibrosis have similar symptoms even though they are very different in nature,' says Dr Arya.  He lists a few common symptoms of lung diseases:

Difficulty in breathing

Chronic cough

Being short of breath easily after any strenuous activity

Excess mucus production

Coughing up blood due to inflammation in the lining of the respiratory tract

Chest pain

Air hunger which indicates lack of oxygen and also can cause fatigue

Diagnosis

In most cases, chronic cough, chest pain and breathing difficulties necessitate a lung function test. But your doctor might first take a look at the results of your blood test that may indicate whether you have any kind of lung infection. Otherwise, based on your symptoms your doctor might recommend the following lung function tests for you, as explained by Dr Arya.

Imaging tests: A chest X ray, CT scan or nuclear lung scanning can be done to reveal any kind of structural abnormality of the lungs and the chest. With the help of an Xray, the doctor will be able to detect lung tumors, infections like pneumonia, emphysema, interstitial lung disease. It also helps identification of fluid accumulation around the lungs (pulmonary edema).

Lung Volume measurement or spirometry:  This test will determine how much air your lungs can hold. A device called spirometer is used in the test to record and analyse the volume of air inhaled and exhaled. The test is repeated after administering a bronchodilator which opens up the airways.

Diffusing capacity measurement: This test determines how well oxygen is transferred from the air sacs (alveoli) to the bloodstream.

Maximal Voluntary Ventilation: The maximum capacity a person can breathe is checked in this test.

Lung biopsy: In case interstitial lung disease is suspected, the doctor may suggest a lung biopsy where a tissue from the lung is obtained using procedures like bronchoscopy for investigation.

Treatment

'If any of the above mentioned symptoms persist for a long time, it is better to consult a doctor immediately for diagnosis,' says Dr Arya. The management of lung disease depends on the type of lung disease you're diagnosed with but largely medication is the main treatment option for lung disease patients.

Asthma and COPD patients may require bronchodialtors and inhalers for relieving asthma symptoms and opening up the airways. Read more about medicines for asthma

Expectorants may be prescribed in case of chronic productive cough to loosen the mucus and facilitate easy expulsion

Corticosteroids are given for reducing inflammation and swelling of the lung tissue

Patients with extreme shortness of breath are managed with oxygen therapy, where the prescribed level of oxygen is delivered through a mask

Antibiotics like azithromycin may be prescribed for patients diagnosed with interstitial lung disease. Lung Infections are also treated with antibiotics

There are some thoracic surgical interventions in which the lung volume or contraction and expansion of the diaphragm can be improved for better lung function

Lung transplantation is the last option for patients with end stage lung disease

'However additional measures like lifestyle management are also needed,' says Dr Arya. These measures not only help you protect lung function but also prevent lung disease. Here are some tips he offers for better management and prevention of lung disease.

If you are a smoker, quit smoking and if not, avoid smokers as far as possible.

Identify things that you are allergic to and avoid them. Some common allergens include animal dander, dust, and aerosols.

Contrary to popular belief, exercise helps with lung diseases. Exercising for even half an hour daily can increase your lung capacity.

Manage your weight as being obese puts more stress on your heart and lungs.

 It is important is to take your medication on time every day.

Lastly, get as much rest as possible.

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