restrictive lung disease

bronchiectasis - what it is & causes

- chronic, irreversible dilation of bronchi

- caused by recurrent infection leading to airway wall destruction

What is the "vicious cycle theory" in bronchiectasis?

Infection → inflammation → tissue damage → impaired clearance → recurrent infection → further destruction of bronchial walls

What are the three main mechanisms that lead to bronchiectasis?

(1) Bronchial wall injury or weakness, (2) Traction from adjacent fibrosis, (3) Bronchial lumen obstruction

What causes traction bronchiectasis?

Fibrotic lung diseases (e.g., sarcoidosis, tuberculosis, interstitial fibrosis) pulling airways open

What are common causes of bronchial wall injury?

Recurrent infections, genetic defects (e.g., cystic fibrosis), or impaired mucociliary clearance

What are typical causes of localized bronchiectasis?

Foreign body aspiration or airway tumor that prevents clearance of mucus distally

What is the "signet ring sign" on HRCT?

A dilated bronchus larger than its accompanying pulmonary artery (resembles a ring with a jewel)

What is the "tram-track sign" on HRCT?

Parallel thickened bronchial walls seen in dilated airways

Which lobes are commonly affected by cystic fibrosis-related bronchiectasis?

Upper lobes

Which lobes are typically affected in aspiration-related bronchiectasis?

Lower lobes

What are the hallmark symptoms of bronchiectasis?

- Chronic productive cough

- large amounts of purulent sputum

- recurrent lung infections

What are the three layers of sputum typically seen in bronchiectasis?

White frothy top, mucoid middle, and thick yellow-green purulent bottom layer

What is "dry bronchiectasis"?

A type of bronchiectasis with little or no sputum production

What causes hemoptysis in bronchiectasis?

Erosive damage to airway blood vessels during infection

What are common physical exam findings in bronchiectasis?

- Crackles

- wheezing

- diminished breath sounds

- accessory muscle use

- kyphosis

What is the gold standard for diagnosing bronchiectasis?

High-resolution computed tomography (HRCT)

What do pulmonary function tests (PFTs) typically show in bronchiectasis?

An obstructive or mixed pattern with ↓FVC, ↓FEV₁, ↓FEF 25-75%, and ↑RV

What can cause hypoxemia in bronchiectasis?

Ventilation-perfusion mismatch due to mucus plugging and airway damage

What are the key components of acute bronchiectasis management?

Antibiotics, bronchodilators, hydration, and secretion clearance techniques

When is surgery indicated for bronchiectasis?

Only for localized disease not responding to medical therapy or for controlling massive hemoptysis

Which group has the poorest prognosis?

Patients with cystic fibrosis-related bronchiectasis

What preventive strategies can reduce bronchiectasis risk?

Prompt treatment of lung infections, managing GERD, and genetic or immune screening in at-risk individuals

What are the main physical therapy goals for bronchiectasis?

Improve secretion clearance, breathing control, muscle strength, and endurance

How is a bronchoscopy usually performed?

with a flexible fiber-optic tube inserted through the patient's mouth or nose for direct visualization of the airways

What are inhaled corticosteroids used for?

decrease inflammation in airways in those with asthma (mostly)

Side effects of inhaled corticosteroids

hoarseness, fungal infection in the mouth

Does sympathetic or parasympathetic system cause bronchodilation?

sympathetic

Muscarinic receptors are involved with sympathetic or parasympathetic system?

parasympathetic

Medications that are muscarinic antagonists prevent _______

bronchoconstriction

Mucoactive agents are a class of chemical agents which aid in _________

the clearance of muscus

Mucoactive drugs - expectorants

increase airway water or the volume of airway secretions

Mucoactive drugs - mucolytics\Mucinex

thin the mucus

Mucoactive drugs - mucokinetics

increase the transportability of mucus by cough

Mucoactive drugs - mucoregulators

suppress underlying mechanisms of mucus hypersecretion

True or false: antibiotics are used for AECOPD (emphysema & chronic bronchitis)

true

Medications for chronic bronchitis

antibiotics, bronchodilators, anti-inflammatory

in RLD, lung expansion is restricted which means the volume or air or gas moving in and out of the lungs is (increased \ decreased)

decreased

restrictive lung disease is a problem getting air IN the lungs \ impairment of _________

volume

pulmonary function testing in restrictive lung disease will typically show a _________ in almost all volumes and capacities with fairly normal flow rates

decrease

true or false: in RLD, chest wall or lung compliance, or both, is increased

false - decreased

what are the FOUR causes of secondary restrictive lung disease?

- pleural

- chest wall

- disease

- abdomen/extrinsic

secondary causes of RLD - pleura

- pleural effusion

- pneumothorax

- pleural fibrosis

- pleural tumors

- pleural thickening

secondary causes of RLD - chest wall

- trauma

- kyphoscoliosis

- ankylosing spondylitis

- neuromuscular disease

- morbid obesity

- scleroderma

causes of primary restrictive lung disease

- idiopathic

- occupational

- collagenic

- granulomatous

- irradiation

- resection

- drug-induced

in restrictive lung disease, the FEV1/FVC ratio is >_____%

80

true or false: the most common cause of restrictive lung disease is idiopathic

true

besides idiopathic, what are FIVE risk factors of restrictive lung disease?

- age

- exposure to toxins

- smoking

- genetic

- GERD

decreased pulmonary compliances requires an increase in _______ just to maintain adequate lung expansion and ventilation

pressure

true or false: inspiratory reserve volume is diminished in RLD because the distensibility of the lung is decreased

true

true or false: tidal volume increases in RLD due to an increase in work of breathing

false - decreases

in restrictive lung disease, is the elastic recoil in the lung increased or decreased?

increased

what happens to the expiratory reserve volume (EDV) in restrictive lung disease?

Decreases

in RLD, residual volume is usually ________ which results in decreasing lung volumes

decreased

what are FOUR hallmark signs of restrictive lung disease?

- dyspnea

- dry cough

- difficulty maintaining weight

- decreased O2 sat

________ _______ = the accumulation of fluid in between the parietal and visceral pleura, called the pleural cavity

pleural effusion

____________ = collection of air outside the lung but within the pleural cavity which may apply pressure on the lung and make it collapse

Pneumothorax

true or false: pleural inflammation can cause the pleura to thicken and become stiff

true

________ _______ is an issue with the abdomen that can cause restrictive lung disease

severe distention

______-______ _______ is a radiological finding in CT scan consisting of a hazy opacity that represents restrictive lung disease

ground-glass opacity

diagnosis of restrictive disease:

- history & physical exam

- pulmonary function tests (PFTs)

- CXR

- CT scan

- ABGs

- bronchoscopy

- surgical biopsy

a V/Q mismatch or _______ is a classical presentation for restrictive disease

hypoxemia

what are signs of restrictive disease

- tachypnea

- hypoxemia

- dry inspiratory crackles

- decreased lung volume

- deceased diffusion capacity

- pulmonary HTN

- decreased chest wall expansion

- high WOB

_________ interstitial pulmonary fibrosis is due to exposures such as heavy metals, dust, farming and livestock

Occupational

_______ is due to inflammation of the lung alveoli from an infection where inflammatory fluid filled with WBC begin to fill up the alveoli which replaces air and interferes with gas exchange

Pneumonia

in pneumonia, fluid and pus -filled air space contains _______

bacteria

which medication is prescribed for patients with pneumonia to treat the infection?

Antibiotics

respiratory rate is usually ________ to overcome the decrease in pulmonary compliance

increased

in restrictive lung disease, the normal inspiratory muscles, especially the _______, work harder and accessory muscles are recruited to assist in expanding the thorax

diaphragm

in a normal person's, the body uses 5% of VO2 max but in restrictive disease, as much as ____% of VO2 max is used to support the work of breathing

40

pneumonia is an inflammatory process of the lung parenchyma that usually begins with an infection in the _______ respiratory tract

lower

in pneumonia, a substance denser than air lingers within the alveolar spaces causing an _______

infection

in ______ pneumonia, after antibiotic treatment, it is important to find the cause of the infection

aspiration

aspiration PnA is a _______ dysfunction

swallowing

aspiration pneumonia can take __-__ weeks to recover

1-4

what are common signs of aspiration pneumonia?

- fever

- choking

- cough

- SOB

- tachycardia

- decreased breath sounds

risk factors for aspiration PnA

- swallowing disorder

- lung transplant

- GERD

- age

can restrictive lung disease be cured?

No

in bacteria pneumonia, a ______ or ______ will be seen on a chest xray

infiltrate; consolidation

sxs of infiltrate PnA

- SOB

- low grade fever

- cough

- increased WBC count

- sputum

risk factors for infiltrate PnA

- community acquired

- cant fight infection

- immobilized

- antibiotics

true or false: possible pleural chest pain (pain with inspiration) can be symptom of pneumonia

true

_____ _____ occurs when there is excessive fluid within the pleural cavity which compresses the alveolar tissue

pleural effusion

a pleural effusion can occur by itself or can be due to infection, inflammation, or a history of ________

cancer

what is the main symptom of pleural effusion?

SOB

true or false: the larger the effusion, the greater the stress

true

signs/symptoms of pleural effusion

- dyspnea

- pleuritic chest pain

- dry, nonproductive cough

- fever

- hemoptysis

- weight loss

in _______ _______, there will be dullness to percussion and decreased breath sounds

pleural effusion

what are the THREE main causes of pleural effusion?

- Trauma

- history of cancer

- cardiac surgery

in restrictive lung disease, do symptoms of decreased O2 saturation occur at rest or with physical activity?

physical activity

treatment for pleural effusion

- wait for the body to reabsorb the fluid

- chest tube to remove the fluid

- needle aspiration

what abnormality would be seen on a chest X-ray for someone with a pleural effusion?

blurring of costophrenic angle

________ refers to a complete or partial collapse of a lung caused by blockage of the alveoli or by pressure on the lungs

atelectasis

the term _______ means "incomplete expansion" and describes a state where a region of the lung parenchyma is collapsed and nonaerated

atelectasis

causes of atelectasis

- hypoventilation

- compression

- airway obstruction

- adhesions

________ atelectasis = complete obstruction of an airway that interferes with ventilation (air in and out of the alveoli) to an area of the lungs

Resorption

which type of atelectasis is the most common and is often due to an obstruction such as tumor, foreign body, or mucus plug

resorption

what is the most common cause of atelectasis?

post surgical (also mucous/foreign body obstruction)

resorption atelectasis is also known as _______ atelectasis

obstructive

________ atelectasis results whenever the pleural cavity is partially or completely filled by fluid exudate, tumor, blood or air which increases the pressure exerted on the lungs

Compression

________ atelectasis occurs when local or generalized fibrotic changes/scarring in the lung or pleura prevent full expansion

Contraction