Restrictive lung disease Flashcards by Eilidh Seaton

What is the pathology behind restrictive lung disease

Pathological material enters
Acute inflammation = diffuse alveolar damage (DAD)
Leads to massive pulmonary oedema
Cells are damaged
Vessels become more leaky
Macrophages and proteins form hyaline membranes
Interstitial inflammation and fibrosis = rapid
Can be fatal

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What can DAD also be

ARDS

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What are causes - ask in HX

Occupational exposure
Drugs -  amiadarone 
Hypersensitivity to allergens - EAA 
Infection - TB / fungi / pneumonia 
GORD associated
Systemic causes

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What are systemic causes - ask in Hx

Sarcoidosis
SLE
Systemic sclerosis
RA 
UC
Vasculitis 
Autoimmune thyroid

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What if unknown cause

Known as idiopathic pulmonary fibrosis

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What are symptoms of restrictive lung disease and what does it lead too

SOB
Dry cough
Crackles - end of inspiration 
Abnormal breath sounds
Shallow breath as reduced chest size 
Abnormal CXR / CT
Clubbing 
Type 1 resp failure
Pulmonary hypertension -> 
Cor pulmonale
HF

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What are RF for developing

Major trauma
Chemical injury
Circulatory shock
Drugs
Infection
Autoimmune
Radiation

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What is investigation of choice for DADs

CXR - show shaggy heart / diaphragm (diff oedema)
Always
HRCT

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What does histology show

Protein rich oedema
Hyaline membrane
Loss of fibrin
Epithelial fibroblast proliferation 
Scarring
Fibrosis + remodelling of interstitium
Chronic inflammation

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Is there reduced lung compliance

Yes as stiff

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What does spirometry show

Low FEV!
Low FVC
Ratio the same
Reduced gas transfer as diffusion abnormality 
V/Q imbalance

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What causes chronic

Sarcoidosis
UIP - usual interstitial pneumonia
Granulomatous
Hypersensitivity pneumonitis

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What is sarcoidosis

Multisystem granulomatous disorder
Granuloma = inflammatory cluster filled with mostly macrophages
Non-ceasaing = no necrosis and is buzzword for sarcoidosis

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What is histology in sarcoidosis

Epithelioid and giant cell granuloma's
Filled with MO 
Little lymphoid infiltrate
Variable fibrosis
Alveoli surrounding granuloma = normal

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What causes sarcoidosis

Unknown cause
Immune reaction to unknown antigen?
Can affect any organ

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What does acute sarcoid present like

Bilateral hilar lymphadenopathy - seen on CXR or CT
Swinging fever
Erythema nodosum
Polyarthralgia

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When is bilateral hilariously lymphadenopathy also common

TB
Malignancy - lymphoma 
Fungi
HIV 
Mycoplasma 
Hypersensitivty pneumonitis 
Histiocytosis

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What are insidious symptoms

SOB
Dry cough 
Crackles 
Chest pain
Malaise
Weight loss
Pink lesions containing granuloma - over scar tissue
Clubbing
Mediastinal LN

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What are skin symptoms

Lupus pernio

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Symptoms of non-pulmonary disease

Systemic - fever, fatigue, weight loss
Hypercalcaemia - macrophages in granuloma increase conversion from vitae's D
Lymphopenia
LN
Liver cirrhosis / cholestasis 
HSM
Uveitis
Conjunctivitis
Optic neuritis
Bells Palsy
Neuropathy
Cardiomyopathy 
Heart block 
Renal

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Who is affected by sarcoid

Young females

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How do you Dx

What is diagnostic

Blood - Elevated serum Ig / ACE / Ca / LFT / ESR
CXR
Spirometry = restrictive
Transbronchial biopsy / bronchoscopy = diagnostic
Mediastinoscopy + biopsy of hilar LN

Other organ involvement 
U+E / urine dip 
LFT
ECHO - if cardiac
ECG- BBB/. arrhythmia 
PET CT to see what lights up 
TB test
USS abdo
Examine eye for occular

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What does CXR show

Trachea
Nodes
Chunky light hilar
Pulmonary infitrates
Fibrosis

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What does biopsy show

Non-caesating granuloma

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How do you treat mild disease, normal PFT, no important organ

No treatment

What do you do if erythema nodosum / arthralgia

NSAID

What do you do if skin lesions / uveitis / cough

Topical Steroid

Cardia / neuro / hypercalcaemia / symptomatic stage 2/3 disease

Systemic steroid Biphosphonates for hypercalcaemia 2nd line = immunosuppression

What is stage 1 on CXR

Bilateral hilar lymphadenopathy

Stage 2

+ pulmonary infiltrate

Stage 3

Pulmonary infiltrates ONLY

Stage 4

``` Fibrosis Causes pulmonary hypertension Loss of lung function Impairment to lung function / gas exchange Benefit from lung transplant ```

What are complications of sarcoidosis

``` Respiratory failure Bronchiectasis Aspergilloma Haemoptysis Pneumothorax ```

DDX of sarcoidosis

Infection Autoimmune Vasculitis EAA

What is hypersensitivity pneumonitis also known as

Extrinsic allergic alveolitis

What is hypersensitivity pneumonitis

Group of diseases caused by inhalation of allergen | Immune mediated

What type of hypersensitivity and histology

``` Type 3 = immune complex Granuloma's - centracinar (type 4) Foamy histiocytes Intersitital pneumoinitis Bronchiolitis obliterans can occur ```

What happens in acute

Alveoli infiltrated by inflammatory cells | If remove allergen process can be halted

What happens in chronic

Granulomas + lymphocytes = chronic inflammation Fibrosis Bronchiectasis

What are acute symptoms 4-8 hours after exposure

``` Recurrent SOB Dry cough Pyrexia Myalgia Bibasal crackles Wheeze Tachypnoea Flu symptoms Reduction in lung volume and diffusion ```

What are chronic symptoms

``` Fibrosis Increasing SOB Weight loss Cough Crackles Wheeze Malaise / lethargy Clubbing Type 1 resp failure Cor pulmonale ```

How do you investigate

CXR BAL Biopsy FBC - increased WCC, neutrophil, ESR

What does CXR show

Fibrosis of upper and middle lobe | Diffuse shadowing

What does biopsy show

Fibroses Alveolitis Granuloma

What does BAL show

Lymphocytosis and mast cells

How do you treat

Remove allergen Steroids in severe Oxygen may be used in acute attacks

Often misDx as

Pneumonia

What is Ddx of fibrosis

Infection Lymphoma Carcinoma Haemorrhage

What is idiopathic pulmonary fibrosis

Most common cause of fibrosis in the lung

Pathology of IPF

``` Patchy chronic inflammation Intra-alveolar fibrosis Obliteration of bronchioles Smooth muscle and vascular proliferation Turns into collagen ```

What causes IPF

Connective tissue - ANCA / RF +Ve Drugs Asbestos After viral infection

Symptoms of IPF

Progressive exertional dyspnoea Dry cough Basal fine crackles at end of inspiration Clubbing ``` Malaise Fever Weight loss Arthralgia Elevated ESR Cyanosis Failed Rx of LVF / pneumonia ```

How do you investigate

``` ABG - decreased O2 Bloods - increased CRP, Ig, ANCS, RF CT = diagnostic CXR Spirometry - restrictive BAL PFT - reduced gas transfer ```

What does CXR show

``` Fleeting shadows Small irregular opacities Peripheral opacities -> honeycomb late Diaphragm and fuzzy heart border Smaller lung volume Often misdiagnosed as pneumonia ```

What does BAL show

Lymphocytosis Neutrophils Eosinophilia suggest worse prognsosi

How do you treat

``` Often poor response to Rx Pulmonary rehab Oral anti fibrotic Supplementary O2 Opiates for SOB Palliative Surgical transplant ```

What are the complications

``` Lung cancer Fibrosis Honeycomb End stage lung disease Cor pulmonale and res failure = death ```

What are most common restrictive lung disease

Idiopathic Drugs LL tend to be affected

What affects upper lobe

``` CHARTS Coal work - hypersnesitivity Histiocytosis / hypersnesitivity / EAA AS Radiation TB SArcoidosis ```

What affects LL

``` Idiopathic Connective tissue Drugs Asbestos a!A! ```

What is the advanced stage of fibrosis

Honeycomb lung

What drugs cause fibrosis

Amiadarone Cyclophosphomdie Methotrexate Nitrafuratoin

What is hypoxia at rest an indication for

Oxygen therapy

What can be given to slow down progression

Anti-fibrinolytic

To get drug what is required

Non-smoker | FVC <80%

What lifestyle

Smoking cessation | Pulmonary rehab

What do yo follow up patients with

CXR | Spirometry and PFT

What is important in history

``` Childhood - TB / pneumonia Occupational exposure Dust / asbestos Immune - scleroderma / RA / sarcoid Smoking Drugs Pets Feather duvet FH ```

When should you think if ILD

Desaturations | Chronic SOB / chronic cough

What is general management of ILD

``` Remove cause Home O2 if hypoxic at rest Stop smoking Physio and pulmonary rehab Pneumococcal and flu Lung transplant is an option ACP and palliation ```

What type of crackles

Fine end inspiratory

In pneumonia

Coarse inspiratory