Restrictive, Mixed Restrictive and Obstructive Lung Disease Flashcards
AAFB
acid and alcohol fast bacilli
DPLD
Diffuse parenchymal lung disease
IPF
idiopathic pulmonary fibrosis
LDH
Lactate Dehydrogenase
NSIP
non-specific interstitial pneumonitis
SLE
systemic lupus erythematosus
Lung Function Test
Restrictive Lund disease diagram
Restriction diagram
Causes of restriction (4)(3)(5)(0)(4):
- Parenchymal lung disease (DPLD):
- Pulmonary fibrosis
- Sarcoidosis
-Pneumoconiosis - Pleural disease:
- pleural effusion
- pneumothorax
- haemothorax
- pleural calcification, thickening
- mesothelioma
- Obesity:
- Chest wall disease:
- neuromuscular disease
- diaphragmatic palsy
- kyphosis
- scoliosis
Diffuse Parenchymal Lung Disease diagram:
Damage to lung parenchyma leads to inflammation and fibrosis
Injury causes release of chemicals that damage epithelium leading to inflammation and fibrosis (go over inflammatiton)
Fibrosis of the Lungs:
- fibrosis of the lungs leads to scarring
- primary site of injury of the interstitium: the space between the epithelial cells and endothelial basement membrane
- lungs become thick and stiff
- there is reduction transfer of oxygen from the alveolar space into the lung capillaries
- patients become breathless
- oxygen saturation will drop
History in suspected diffuse parenchymal lung disease:
- Occupation: asbestos, pneumoconiosis
- Pets: birds (pigeons, budgies, parakeets),cats, dogs
- Drugs: amiodaron, nitrofurantoin, chemotherapy
- Exposure to Radiation, including radiotherapy
- Systemic enquiry: autoimmune diseases
- HIV
- family history
Symptoms in patients with Diffuse Parenchymal Lung Disease:
- breathlessness, progressively worsening
- cough
- fatigue
- weight loss
Symptoms specific to autoimmune disease/collagen vascular disease:
- difficulty swallowing
- cold hands
- joint pains
- weight loss
- skin rash
Clinical Examination in Diffuse Parenchymal Lung Disease:
- high respiratory rate at rest (tachypnoea, dyspnoea)
- clubbing
- fine bibasal crackles
- cardiovascular examination
- Increased JVP, peripheral oedema, loud P2 heart sound
- low oxygen saturation and desaturation on exertion
- Features of autoimmune disease:
- skin changes: telangiectasia, Raynaud’s phenomena
- joint signs
- eye signs
High respiratory rate, clubbing, fine bibasal crackles, breathless likely to be
pulmonary fibrosis (DPLD)
Idiopathic pulmonary fibrosis definition
IPF is a distinctive type of chronic fibrosing interstitial pneumonia of an unknown cause limited to the lungs
Prevalence of idiopathic pulmonary fibrosis
6-14.6/100,000
Prevalence of idiopathic pulmonary fibrosis in over 75 yrs.
175/100,000
Idiopathic Pulmonary Fibrosis usually presents in what age group?
Over 50 yrs
Which gender does idiopathic pulmonary fibrosis present in?
Male more than female
Does idiopathic pulmonary fibrosis have any occupational history of relevance?
No
Does idiopathic pulmonary fibrosis have any features of autoimmune disease?
No
Can idiopathic pulmonary fibrosis be famial?
Yes
Presentation of Idiopathic Pulmonary Fibrosis
- progressively worsenign shortness of breath over 2 years
- dry cough
- crackles (fine babasil)
- clubbing
- weight loss
- hypoxic
Prognosis of idiopathic pulmonary fibrosis
- gradual deterioration
- median length of survival from diagnosis: 2.5-3.5 years
- exacerbations and sudden decline
Idiopathic pulmonary fibrosis
HRCT of idiopathic pulmonary fibrosis
watch this part of lecture
Idiopathic pulmonary fibrosis: lung function:
- FVC decreases
- FEV1 normal or slightly low
- FEV1/FVC increases
- reduced transfer factor/diffusing capacity (TLCO)
Management of Idiopathic pulmonary fibrosis: 4 types of treatment:
- Symptomatic Treatment
- Antifibrotic Treatment
- Lung Transplantation under the age of 60
- Palliative Care
Is Idiopathic pulmonary fibrosis associated with smoking?
Yes
Management of Idiopathic pulmonary fibrosis: Symptomatic Treatment:
- cough: mucolytics
- hypoxia treated with long term oxygen therapy
Management of Idiopathic pulmonary fibrosis: Antifibrotic Therapy:
- Pirfenidone
- Nintedanib
(not a cure but can stabilise)
(limiting factors are side effects)
What is Non - Specific Interstitial Pneumonia (NSIP) associated with?
- associated with autoimmune disease/ collagen vascular disease
Median age affected by non-specific interstitial pneumonia
40-50 yrs
Which gender does non - specific interstitial pneumonia affect more?
Affects both equally
Does non-specific interstitial pneumonia have an association with smoking?
No
Symptoms of non-specific interstitial pneumonia
- progressively worsening breathlessness over 1 year
- cough
- crackles
- clubbing
- fatigue
- weight loss
NSIP Associated with Collagen Vascular Disease
- Scleroderma (CREST/Systemic Sclerosis)
- Rheumatoid arthritis
- SLE
Non-specific interstitial pneumonia has a worse prognosis than IPF.
True or False? Why?
False
Better prognosis as can treat underlying autoimmune disease, which ease symptoms
Non-specific interstitial pneumonia responds better to anti-inflammatory drugs than IPF.
True or False?
True
Chest x-ray of NSIP
Small lungs with reticulo-nodular changes
CT NSIP
Ground glass changes
Management of NSIP
- treatment of underlying condition
- immunosuppression
- long term oxygen therapy
- palliative care
Prognosis of IPF and NSIP graph
Sarcoidosis (5) :
- multisystem granulomatous disease
- unknown cause/aetiology
- characterised by non-caseating granulomas, often in multiple organs
- sarcoidosis primarily affects the lungs
- may resolve spontaneously, can recur and may progress to pulmonary fibrosis
Aetiology of sarcoidosis:
- infection:
- remnants of microbial organisms
- organic dusts, metals, minerals, solvents, pesticides, wood stoves
- beryllium results in pulmonary granulomas
- can run in families
Clinical Presentation of Sarcoidosis: gender:
- slightly higher in women
Clinical Presentation of Sarcoidosis: age:
- 20-50 (smaller peak larger than 60 years??)
Clinical Presentation of Sarcoidosis: ethnicity:
- higher in Scandinavian, Afro-Caribbean and African American women
Clinical Presentation of sarcoidosis: incidence:
- overall, 5/100,000
Clinical Presentation of Sarcoidosis: Prevalence:
- most common ILD in the UK
Clinical Presentation of sarcoidosis: association:
- TB
- lymphoma
Symptoms of acute sarcoidosis are:
- duration
- self-limiting
- short duration
- self-limiting
Symptoms of chronic sarcoidosis:
- duration
- self-limiting
- changes
- longer duration
- not self-limiting
- persistent radiological changes
Asymptomatic sarcoidosis would be diagnosed by an
incidental finding
Symptoms and Signs of acute sarcoidosis:
- fever
- arthralgia: aching joints
- anterior uveitis: painful red eyes
- breathlessness
- reduced appetite
- night sweats
- myalgia
- erythema nodosum: painful red lumps on lower legs
- lymphadenopathy: swollen lymph nodes
- weight loss
- Loefgren’s syndrome: erythema nodosum and BHL (bilateral hilar lymphadenopathy)
- responds to oral prednisone
- good prognosis
Erythema Nodosum
painful red lumps on lower legs
Anterior Uveitis
painful red eyes
Chronic Pulmonary Sarcoidosis symptoms (10)
- progressively worsening breathlessness
- reduced exercise tolerance
- cough
- fatigue
- anorexia
- weight loss
Neurological Symptoms, bone pain, skin lesions (EN, lupus pernio in 25%), renal stones
Differential diagnoses: wide, cancer
Lung: any diffuse parenchymal disease
If a patient comes in with acute exacerbation of IPF, with an infection, can be given
- steroids
- obvs antibiotics
- steroids are not a long term solution
Organ involvement in Sarcoidosis: What % have pulmonary involvement? What % have evidence of only lung involvement?
- 90% have pulmonary involvement
- 50% have evidence of only lung involvement
Which organs can be involved in sarcoidosis? (
- Skin
- Joints and Muscles
- Heart
- Lacrimal glands
- GI system
- Lymph nodes
- Liver
- Bone
- Kidneys
- Pancreas
- Eyes
- Spleen
- Salivary Glands
- Nasal
- Brain
Clinical Approach in Sarcoidosis:
- History: occupational, family
- Examination
- Chest X-Ray
- Full lung function test
- Blood tests
- Urinalysis
- ECG
- Ophthalmologic examination
- Tuberculin skin test
- no definition diagnostic test for sarcoidosis
- diagnosis made with compatible clinical, radiological and histopathological features
Radiological Staging of Sarcoidosis:
- Stage 0: Normal chest X ray (5-0%)
- Stage I: Bilateral Hilar Lymph nodes (45%-65%) most common presentation, no treat lymph nodes
- Stage II: Nodes + upper zone parenchymal disease (25=30%)
- Stage III: parenchymal disease upper zones
- Stage IV: end stage pulmonary fibrosis
Treatment begins with steroids in stage 2 and other medication
Lung Function In Sarcoidosis:
- restrictive pattern
- airflow obstruction
- can have both mixed restrictive and obstructive lung function
Other Investigations in Sarcoidosis:
- Serum ACE: produced by activated macrophages in granulomas
- Hypercalcaemia due to increased Vitamin D by macrophages in granulomas
- Hypercalciuria (high calcium in urine)
- ACE = angiotensin converting enzyme
Natural History of Sarcoidosis:
- 2/3: remission within 10 years; stage 1 60% complete resolution within 2 years
- 1/3: progression with significant organ damage
- 1-5%: die secondary to respiratory failure, cardiac arrhythmia, neurosarcoidosis
Natural History of Sarcoidosis:
- 2/3: remission within 10 years; stage 1 60% complete resolution within 2 years
- 1/3: progression with significant organ damage
- 1-5%: die secondary to respiratory failure, cardiac arrhythmia, neurosarcoidosis
Management of Acute Sarcoidosis:
Oral Prednisolone
Management of Asymptomatic or Stage I Sarcoidosis:
observation
Management of Stage II, III, IV Sarcoidosis:
prednisolone (steroid)
Management of progressive Sarcoidosis:
- immunosuppressive drugs
- methotrexate
- mycophenolate mofetil
Main differential diagnosis for Stage I and Stage II Sarcoidosis is
differential diagnosis
Differential diagnoses for Stage III and Stage IV include
other DPLD
Pleural Disease (5):
- Pleural Effusion
- Pneumothorax
- Haemothorax
- Pleural Thickening
- Mesothelioma
Pleural Disease: Pleural Effusion:
- fluid in the pleural space
- exudate = thick pleural effusion
- transudate = thin pleural effusion
- sharp pain, which is worse on inspiration; pleural chest pain
Pleural Disease: Pneumothorax:
- air in pleural space, damage to parenchyma pops (anything could be emphysema)
- spontaneous
- traumatic: procedural, penetrating trauma, rib fracture, barotrauma
- tall, skinny male (white)
Pleural Disease: Haemothorax:
- blood in the pleural space
- traumatic injury
Pleural Disease: Pleural Thickening:
pleura thicken
Pleural Disease: Mesothelioma:
cancer of the pleura
Pleural Effusion diagram
Purpose of pleural fluid
lubricates pleural space, avoids friction when lungs move
What is the amount of pleural fluid produced and cycled through the pleural space?
1L
Pleural Aspiration (Thoracocentesis)
- pleural fluid can be taken at the bedside with ultrasound guidance, local anaesthetic and a needle.
- Pleural Drainage if patient is symptomatic
Pleural Effusion: when examining a patient, what would you see?
- complaining of chest pain maybe
- breathless
- not moving equally or symmetrically; eg left PE, left side of chest will not rise as much as the right side
- reduced chest wall movement on the side of the effusion
- dullness on percussion on the side of the effusion: this is the most reliable clinical finding
- decreased tactile vocal fremitus and vocal resonance on the side of the effusion
- bronchial breathing above the effusion
- tracheal deviation away from side of a large effusion
Name procedure
Pleural aspiration
Diagnostic sample of pleural effusion:
- Biochemistry: protein and lactate dehydrogenase (LDH)
- Microbiology: microscopy, culture and sensitivity, acid and alcohol fast bacilli (AAFB) looking for infections (TB)
- Cytology: abnormal cells (cancer)
Types of pleural effusion:
- exudate
- transudate
Type of pleural effusion is determined by:
- protein
- lactate dehydrogenase (LDH)
- serum protein (ACE)
- serum LDH
What is used to differentiate between an exudate PE and transudate PE?
Light’s Criteria
Exudate Pleural Effusion:
- pleural fluid protein/serum protein
- pleural fluid LDH/serum LDH
- pleural fluid LDH
- > 0.5
- > 0.6
- > 2/3 of upper limit of normal serum LDH
- more protein
Transudate pleural effusion:
- pleural fluid protein/serum protein
- pleural fluid LDH/serum LDH
- pleural fluid LDH
- < 0.5
- < 0.6
- < 2/3 of upper limit of normal serum LDH
- less protein
Causes of Pleural Effusion: Transudate:
- low protein, reduced oncotic pressure, normal pleura
- Congestive cardiac failure
- Low albumin caused by nephrotic syndrome or liver failure
Causes of Pleural Effusion: Exudate:
- unhealthy pleura
- malignancy
- infection (pneumonia, tuberculosis, emphysema)
- autoimmune disease
- chylothorax (due to blocked lymphatics)
Clinical Signs of Pleural Effusion
- reduced chest wall movement on the side of the effusioni
Pneumothorax
Pneumothorax classification:
- Primary: spontaneous
- Secondary: due to underlying disease
Large pneumothorax can cause mediastinum to be deviated.
True or False?
True
Tension pneumothorax
- tension builds up
- can cause cardiac arrest
Primary Pneumothorax Classification: Risk Factors:
- tall and thin: to do with pressure gradients
- asthma
- collagen vascular disease
Secondary Pneumothorax Classification: Risk Factors:
- COPD
- Pulmonary Fibrosis
- Cystic Fibrosis
Pleural Disease
Chest Wall Disease
- results in difficulty expanding the lungs
- does not directly affect oxygenation, lung structure is normal but can’t ventilate
- presents with progressively worsening breathlessness
Chest Wall Disease Risk Factors:
- obesity
- neuromuscular: motor neuron disease, muscular dystrophy, poliomyelitis, diaphragmatic palsy
- musculoskeletal: kyphosis, scoliosis
Combination of obstructive and restrictive diseases: smoking:
- emphysema
- pulmonary fibrosis
Combination of obstructive and restrictive diseases: pulmonary sarcoidosis:
- endobronchial sarcoidosis
- fibrosis
Combination of obstructive and restrictive diseases: Obesity in a smoker:
- obstruction
- restriction
What will spirometry show in a restrictive lung disease?
1 = Increased FEV1
2 = Reduced FEV1
3 = Increased FVC
4 = Decreased FVC
5 = Decreased FEV1/FVC ratio
4
Which of these statements about idiopathic pulmonary fibrosis is true?
1 = is a restrictive lung disease
2 = is a type of malignancy
3 = has an excellent prognosis
4 = can be treated with antibiotics
5 = is common in young females
1
Which organ is most commonly affected by sarcoidosis?
1 = eyes
2 = kidneys
3 = lungs
4 = skin
5 = spleen
3
Which of these statements about pleural effusion is true?
1 = can cause an obstructive lung disease
2 = can be caused by heart failure
3 = there is no pleural fluid in healthy lungs
4 = important to measure calcium in the fluid
5 = is always associated with a bad outcome
2
Which of these conditions can cause a restrictive lung disease?
1 = kyphosis
2 = obesity
3 = pulmonary fibrosis
4 = sarcoidosis
5 = all of these
5