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