Respiratory - restrictive disease Flashcards
What are the types of restrictive diseases (2)
interstitial
Extra-pulmonary
What are the symptoms of interstitial lung disease (5)
progressive dyspnoea
Dry cough
Headache
Confusion
Lethargy
What causes headache, confusion, and lethargy in interstitial disease
Carbon dioxide retention
What are the signs of CO2 retention due to interstitial disease (6)
flushed skin
Bounding pulse
Rapid breathing
Premature heartbeats
Muscle twitches
Flapping tremor
What investigations are used for restrictive lung disease (4)
pulmonary function tests
Imaging (CXR, CT, and USS)
ABG
Bloods
What results would be obtained in PFTs in restrictive lung disease
reduced FEV1 and FVC
Normal ratio
What lifestyle treatments can be used for restrictive lung disease (2)
weight loss
Pulmonary rehab
What medical therapies can be used to treat restrictive lung disease (3)
Anti-inflammatory drugs/Immunosuppressive drugs
Anti-fibrotic drugs
Treating neuromuscular disease
What procedures can be used to treat restrictive lung disease (2)
Intercostal drainage
Ascitic drainage
What surgeries can be used to treat restrictive lung disease (4)
corrective spinal surgery
Decorticating
Diaphragmatic privation
Lung transplant
What methods of supportive treatment can be used in restrictive lung disease (3)
CPAP
Non-invasive ventilation
Oxygen
Describe pulmonary interstitium (2)
connective tissue making up alveolar walls
Contains collagen, elastin, and capillaries
General description of interstitial disease
disorders associated with inflammation and fibrosis of the interstitium of the lungs
What are the stages of interstitial lung disease associated with (2)
early - alveolitis
Later - fibrosis
What functional abnormalities are caused by interstitial lung disease (3)
reduction in diffusion capacity
Reduction in lung volume
Reduction in lung compliance
What are the categories of interstitial disease (5)
fibrosing
Granulomatous
Eosinophilic
Smoking related
Other
What are examples of fibrosing interstitial lung disease (3)
usual interstitial pneumonia
Connective tissue associated
Pneumoconiosis
What are examples of granulomatous interstitial diseases (2)
Sarcoiditis
Hypersensitivty pneumonitis
What are the causes of interstitial disease (3)
Environmental
Hypersensitivity
Idiopathic
What are the symptoms of interstitial disease (2)
dry cough
Breathlessness
Describe usually interstitial pneumonia (3)
progressive pulmonary fibrosis
Associated with patchy interstitial fibrosis, early lesions, and late lesions
Leads to respiratory failure
What regions of the lobe are affected in usual interstitial pneumonia
basal regions of lower lobes
What connective tissue disease are associated with interstitial disease (3)
rheumatoid arthritis
SLE
Systemic sclerosis
Describe components of connective tissue disease associated interstitial disease (3)
Interstitial fibrosis
Pleural effusion
Rheumatoid nodules (in rheumatoid arthritis)
What is pneumoconiosis caused by
reaction to inhaled particles
What are examples of pneumoconiosis (3)
coal worker’s pneumoconiosis
Silicosis
Asbestosis
Describe Pathophysiology of silicosis (4/5)
particles are inhaled then phagocytosed by macrophages
An inflammatory response is induced
Hard collagenous scars form
(Progressive massive fibrosis may develop)
Egg-shell calcification hilar nodes occurs
Describe Pathophysiology of asbestosis (3)
inhaled particles are phagocytosed
An inflammatory response is stimulated
Parenchymal interstitial fibrosis occurs
What complications is asbestosis linked to (5)
pleural plaques
Recurrent pleural effusions
Interstitial fibrosis
Lung carcinoma
Mesothelioma
Describe sarcoidosis (3)
A systemic granulomatous disease
Linked to type 4 Hypersensitivity
With granulomas distributed around bronchi and blood vessels along lymphatics
Describe granulomas in sarcoidosis (2)
well formed
Non-caseating
How can sarcoidosis be diagnosed (3)
CXR and CT showing peripheral nodular infiltrate
Tissue biopsy showing non-caseating granulomas
Raised ACE, calcium, and inflammatory markers in blood test
how is chronic sarcoidosis treated (3)
monitoring
Oral steroids
Immunosuppression
When would acute sarcoidosis be treated
if a vital organ is affected
What type of Hypersensitivity is involved in hypersensitivity pneumonitis
type 3
What are the signs of acute hypersensitivity pneumonitis (7)
cough
Breathlessness
Fever
Myalgia
Pyrexia
Crackles
hypoxia
What are the signs of chronic hypersensitivity pneumonitis (4)
crackles
Finger clubbing
Pulmonary fibrosis
Restrictive defect
which part of the lungs is affected by hypersensitivity pneumonitis
Upper zones
Describe histology of hypersensitivity pneumonitis (4)
Airway entry disease
Interstitial inflammatory infiltrate
Loosely formed, non-caseating granulomas
Interstitial fibrosis
How is hypersensitivity pneumonitis diagnosed (3)
history of exposure
Precipitants
Lung biopsies
How is hypersensitivity pneumonitis treated (5)
oxygen
Steroids
Antigen avoidance
Immunosuppression (if chronic)
Anti-fibrotic therapy (if progressive fibrosis)
Describe idiopathic pulmonary fibrosis (2)
a non-inflammatory condition
Linked to an imbalance in the fibrotic repair system
Describe what happens in idiopathic pulmonary fibrosis (2)
fibrosis of alveolar walls occurs (fibroblastic foci)
Destruction of architecture (honeycombing)
Describe the clinical presentation of idiopathic pulmonary fibrosis (6)
Progressive breathlessness
Dry cough
Clubbing
Bilateral fine inspiratory crackles
Restrictive defect
Bilateral infiltrates
What shows on a CT scan in cases of idiopathic pulmonary fibrosis (3)
reticulonodular fibrotic shadowing
Traction bronchiectasis
Honeycombing cystic changes
How can idiopathic pulmonary fibrosis be treated (3)
anti-fibrotic drugs
Oxygen (if hypoxic)
Lung transplant
