Restrictive and mixed restrictive and obstructive lung diseases Flashcards

1
Q

What is Parenchymal lung disease (DPLD)?

A

DP is short for diffuse parenchymal and refers to a group of lung diseases that affect the lung tissue.

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2
Q

What are the types of Parenchymal lung disease?

A

Pulmonary fibrosis, sarcoidosis, and pneumoconiosis.

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3
Q

What is Pleural disease?

A

It refers to the diseases that affect the pleura, a thin layer of tissue that surrounds the lungs.

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4
Q

What are the types of Pleural disease?

A

pleural effusion, pneumothorax, haemothorax, pleural calcification, thickening, mesothelioma.

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5
Q

What is Obesity?

A

Obesity refers to having excess body fat.

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6
Q

What is Chest wall disease?

A

Chest wall diseases are the diseases that affect the chest wall, which supports the lungs and protects the heart.

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7
Q

What are the types of Chest wall disease?

A

neuromuscular disease, diaphragmatic palsy, kyphosis, scoliosis.

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8
Q

What is diffuse parenchymal lung disease?

A

A group of lung diseases characterized by damage to the lung parenchyma, leading to inflammation and fibrosis.

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9
Q

What is fibrosis in the lungs?

A

Scarring of the lungs which leads to thickening of the interstitium and stiffness of the lungs, reducing the transfer of oxygen and causing breathlessness.

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10
Q

What should be included in the patient’s history?

A

Occupation, pets, drugs, exposure to radiation and autoimmune disease, family history, and symptoms such as breathlessness, cough, fatigue, and weight loss.

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11
Q

What are the symptoms of DPLD?

A

Breathlessness, cough, fatigue, weight loss, and symptoms specific to autoimmune diseases, such as difficulty swallowing, cold hands, joint pain, weight loss, and skin rash.

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12
Q

What should be included in the clinical examination?

A

Increased respiratory rate, clubbing, fine crackles, cardiovascular examination, low oxygen saturation, and features of autoimmune diseases such as skin changes, joint signs, and eye signs.

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13
Q

What is IPF?

A

IPF is a type of chronic fibrosing interstitial pneumonia of unknown cause limited to the lungs.

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14
Q

Who is affected by IPF?

A

IPF is common, more common in males, usually over the age of 50, with no occupational or autoimmune disease history, but can be familial.

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15
Q

What are the symptoms of IPF?

A

Progressively worsening shortness of breath for over 2 years, dry cough, crackles, clubbing, weight loss, and hypoxia.

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16
Q

What is the prognosis for IPF?

A

The prognosis for IPF is gradual deterioration, with a median length of survival from diagnosis of 2.5-3.5 years, and can have exacerbations and sudden decline.

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17
Q

How does IPF affect lung function?

A

IPF affects lung function in a restrictive pattern, with a decrease in FVC and normal or slightly low FEV1, but an increase in FEV1/FVC. There is also a reduction in transfer factor/diffusing capacity (TLCO).

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18
Q

What is the management for IPF?

A

The management for IPF includes symptomatic treatment, antifibrotic therapy, lung transplantation, and palliative care.

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19
Q

What is non-specific interstitial pneumonia (NISP)?

A

NISP is a type of lung disease associated with autoimmune disease/collagen vascular disease (CVD)

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20
Q

Who does NISP affect

A

a median age of 40-50 years and equal incidence in males and females.

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21
Q

What are the symptoms of NISP

A

It presents with symptoms of progressive breathlessness, cough, crackles, clubbing, fatigue, and weight loss.

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22
Q

What are the associated autoimmune diseases with NISP?

A

What is the prognosis for NISP compared to IPF?

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23
Q

What is the prognosis for NISP compared to IPF?

A

The prognosis for NISP is better than IPF, with a better response to anti-inflammatory drugs.

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24
Q

What is the management for NISP?

A

The management for NISP includes treating the underlying autoimmune conditions, immunosuppression, long-term oxygen therapy, and palliative care.

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25
Q

What is Sarcoidosis?

A

Sarcoidosis is a multisystem granulomatous disease characterized by non-caseating granulomas, often in multiple organs, with an unknown cause.

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26
Q

What are the causes of sarcoidosis?

A

Causes of sarcoidosis are not fully understood, but may include infections, organic dusts, metals, minerals, solvents, pesticides, wood stoves, and beryllium.

27
Q

What is the clinical presentation of sarcoidosis?

A

Sarcoidosis affects people between the ages of 20-50, with a slightly higher incidence in women. It is more common in people of Scandinavian, Afro-Caribbean and African American descent. The incidence is 5/100,000, with a prevalence of being the most common ILD in the UK.

28
Q

What are the symptoms of acute sarcoidosis?

A

Symptoms of acute sarcoidosis can include fever, night sweats, arthralgia, myalgia, anterior uveitis, erythema nodosum, breathlessness, lymphadenopathy, reduced appetite, and weight loss. This form of sarcoidosis, called Loefgren’s syndrome, often responds to oral prednisolone and has a good prognosis.

29
Q

What are the symptoms of chronic pulmonary sarcoidosis?

A

Symptoms of chronic pulmonary sarcoidosis can include progressively worsening breathlessness, reduced exercise tolerance, cough, fatigue, anorexia, weight loss, neurological symptoms, bone pain, skin lesions such as erythema nodosum and lupus pernio (in 25%), renal stones, and others.

30
Q

What is Loefgren’s syndrome?

A

Loefgren’s syndrome is a type of acute sarcoidosis that includes symptoms of erythema nodosum and bilateral hilar lymphadenopathy.

31
Q

What organs can be involved in sarcoidosis?

A

Nearly every organ in the body can be involved in sarcoidosis.

32
Q

What is the clinical approach for diagnosing sarcoidosis?

A

The clinical approach for diagnosing sarcoidosis includes a:
patient history,
examination,
chest X-ray,
full lung function test,
blood tests,
urinalysis,
ECG,
ophthalmologic examination,
and a tuberculin skin test.
No definitive diagnostic test exists, and the diagnosis is made based on compatible clinical, radiological, and histopathological features.

33
Q

How is the stage of sarcoidosis determined radiologically?

A
  • Stage 0: Normal CXR (5-10%)
  • Stage 1: Bilateral Hilar LN (45-65%)
  • Stage 11: Nodes + upper zone parenchymal disease (25-30%)
  • Stage 111: Parenchymal disease upper zones
  • Stage 1V: End stage pulmonary fibrosis
34
Q

What is the significance of abnormal lung function tests in sarcoidosis?

A

Abnormal lung function tests in sarcoidosis can show either a restrictive or airflow obstruction pattern, or a combination of both.

35
Q

What are some other investigations for sarcoidosis?

A

Other investigations for sarcoidosis can include serum ACE level, hypercalcaemia, hypercalciuria, and others.

36
Q

What is the natural history of sarcoidosis?

A

The natural history of sarcoidosis varies, with about two-thirds of patients experiencing remission within 10 years, and about one-third progressing with significant organ damage. Approximately 1-5% of patients die secondary to respiratory failure, cardiac arrhythmia, or neurosarcoidosis.

37
Q

What is the management of acute sarcoidosis?

A

The management of acute sarcoidosis typically involves oral prednisolone. For asymptomatic or stage 1 sarcoidosis, observation may be recommended. Stage 2, 3, and 4 sarcoidosis may be treated with prednisolone, while progressive disease may require other immunosuppressive drugs such as methotrexate or mycophenolate mofetil.

38
Q

What is pleural effusion?

A

Pleural effusion is fluid in the pleural space.

39
Q

What is pneumothorax?

A

Pneumothorax is air in the pleural space. It can be spontaneous or due to procedural, penetrating trauma, rib fracture, or barotrauma.

40
Q

What is haemothorax?

A

Haemothorax is blood in the pleural space.

41
Q

What is pleural aspiration (thoracocentesis)?

A

Pleural aspiration (thoracocentesis) is the process of taking pleural fluid at the bedside with ultrasound guidance, local anaesthetic, and a needle.

42
Q

How is the diagnosis of pleural effusion made?

A

The diagnosis of pleural effusion is made through biochemistry (protein and lactate dehydrogenase (LDH)), microbiology (microscopy, culture and sensitivity, and acid and alcohol fast bacilli (AAFB)), and cytology (abnormal cells).

43
Q

What are the types of pleural effusion?

A

The type of pleural effusion is determined by the content of the pleural fluid (protein, lactate dehydrogenase (LDH), and serum protein and serum LDH). Light’s Criteria is used to differentiate between exudate and transudate.

44
Q

What is an exudate

A

An exudate is a type of pleural effusion characterized by:
- Pleural fluid protein/serum protein > 0.5
- Pleural fluid LDH/serum LDH > 0.6
- Pleural fluid LDH> two thirds of upper limit of normal serum LDH

45
Q

What is a transudate

A
  • Pleural fluid protein/serum protein < 0.5
  • Pleural fluid LDH/serum LDH < 0.6
  • Pleural fluid LDH < two thirds of upper limit of normal serum LDH
46
Q

What are the causes of pleural effusion?

A

The causes of pleural effusion can be transudate (low protein, reduced oncotic pressure, normal pleura) such as congestive cardiac failure or low albumin due to nephrotic syndrome or liver failure, or exudate (unhealthy pleura) such as malignancy, infection, autoimmune disease, or chylothorax due to blocked lymphatics.

47
Q

What are the clinical signs of pleural effusion?

A

Clinical signs of pleural effusion can include reduced chest wall movement on the side of the effusion, reduced air entry, dullness on percussion, decreased tactile vocal fremitus and vocal resonance, bronchial breathing above the effusion, and tracheal deviation away from the side of a large effusion.

48
Q

What is pneumothorax?

A

Pneumothorax is a medical condition in which air accumulates in the pleural space, leading to sudden onset of chest pain and breathlessness.

49
Q

What are the symptoms of pneumothorax?

A

The symptoms of pneumothorax include sudden onset of chest pain and breathlessness.

50
Q

How is pneumothorax treated?

A

The treatment for pneumothorax involves aspiration of air using a needle under local anaesthetic, or chest drain insertion.

51
Q

What are the causes of pneumothorax?

A

Pneumothorax is caused by a rupture of alveoli near the pleural space.

52
Q

What is the classification of pneumothorax?

A

Pneumothorax is classified into two types: primary spontaneous pneumothorax and secondary pneumothorax due to underlying lung disease.

53
Q

Who is at risk for primary spontaneous pneumothorax?

A

People who are tall and thin, have asthma, or have collagen vascular disease (CVD) are at risk for primary spontaneous pneumothorax.

54
Q

What are some underlying lung diseases that can cause secondary pneumothorax?

A

Some underlying lung diseases that can cause secondary pneumothorax include COPD, pulmonary fibrosis, and cystic fibrosis.

55
Q

What is chest wall disease?

A

Chest wall disease refers to conditions that affect the muscles and bones of the chest, which can result in difficulty expanding the lungs and progressively worsening breathlessness.

56
Q

Does chest wall disease directly affect oxygenation?

A

No, chest wall disease does not directly affect oxygenation, but it can result in difficulty expanding the lungs, which can ultimately affect oxygenation.

56
Q

What are some conditions that fall under the category of neuromuscular chest wall disease?

A

Some examples of neuromuscular chest wall disease include motor neurone disease, muscular dystrophy, poliomyelitis, and diaphragmatic palsy.

57
Q

What are some examples of musculoskeletal chest wall disease?

A

Examples of musculoskeletal chest wall disease include kyphosis and scoliosis.

58
Q

What is a common factor that can contribute to chest wall disease?

A

Obesity is a common factor that can contribute to chest wall disease.

59
Q

What is a combination of obstructive and restrictive diseases?

A

A combination of obstructive and restrictive diseases is a condition where a patient has both obstructive and restrictive respiratory conditions.

60
Q

What is an example of a combination of obstructive and restrictive diseases that can result from smoking?

A

Emphysema and pulmonary fibrosis are examples of a combination of obstructive and restrictive diseases that can result from smoking.

61
Q

What is an example of a combination of obstructive and restrictive diseases that can result from pulmonary sarcoidosis?

A

Endobronchial sarcoidosis and fibrosis are examples of a combination of obstructive and restrictive diseases that can result from pulmonary sarcoidosis

62
Q

What is an example of a combination of obstructive and restrictive diseases that can result from obesity in a smoker?

A

A combination of obstruction and restriction can occur in an obese smoker, where the airways can become obstructed due to the effects of smoking, while the chest wall is restricted due to the added weight of excess body fat.