Restrictive lung disease - PowerPoint Presentation

Slide1

Restrictive lung disease

Dr Duncan

Powrie

Consultant Chest Physician

Southend

University Hospital

December

2018

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2

Simple spirometry

FEV

1

= Forced Expiratory Volume in 1 second

(

how much you can blow out in the first second of a forced blow

)

FVC = Forced Vital Capacity

(

how much you can breathe out altogether in a forced blow)

VC = Vital Capacity (

how much you can sigh out altogether in a full, steady blow)

FEV

1

/FVC ratio

= a calculation using the above measurements

(% of total that can be forced out in the first second

)

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3

Performing spirometry

1

Record the patient’s sex age and height to find their predicted normal values

Ask the patient to:

breathe in as deeply as possible

blow out forcibly as hard and fast as possible until there is nothing left to expel

*Repeat the procedure twiceThis should give 3 readings, with at least 2 within 100ml or 5% of each other

* severe patients may take up to 15 seconds

Consistent result Inconsistent result

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5

Obstructive vs.restrictive patterns

If the ratio FEV

1

/FVC <70%, obstruction is present

If this ratio is normal but FEV

1

and FVC are both reduced, restrictive pattern is presentA restrictive pattern should be referred to the doctor to check for lung fibrosis, pleural disease, chest wall disease. Obstructive disorder Restrictive disorder e.g COPD e.g. Fibrosing alveolitis, pleural diseaseFEV1

reduced (<80%) reduced (<80%)FVC normal or reduced reduced (<80%)FEV1/FVC ratio reduced (<70%) normal (>70%)

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Assessment of a patient with restrictive

spirometry

Pulm

fibrosis (severe), neuromuscular disease, obesity , chest wall disease

History

Examination

CXR

Full PFTs (inc tests of resp muscle function)OximetryBlood gasesSleep study

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Pulmonary fibrosis

Progressive exertional breathlessness

Dry cough

Arthralgia 20%

Weight loss

Finger clubbing in 50%

End inspiratory ‘velcro’ crackles

Cyanosis

Cor pulmonale

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Epidemiology

6-28/ 100 000yr

M:F 1.7:1

Median age of diagnosis 70

Uncommon before 50

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Risk factors

Exposure to metal or wood dust

Organic solvents

Mycotoxins

EBV, Hepatitis C

Cigarette smoking

Family history

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Management

No really effective evidence based treatment

Information provision and supportive management is key

Monitor lung function if minimal symptoms

If deteriorating lung function consider triple therapy

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Pirfenidone

Anti- inflammatory and anti-fibrotic action

Inhibits fibroblast proliferation

GI side effects

May reduce decline in lung function

Consider if FVC between 80 and 50% predicted

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Breathlessness

Hypoxia is common as is desaturation on exercise

LTOT if pO

2

< 7.3 kPa or <8 if signs of pulmonary hypertension

Ambulatory oxygen if desaturates on exertion

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Cough

Treat reflux

Consider simple linctus

Oral codeine

Consider oramorph or MST in end stage disease

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Pulmonary rehabilitation

No randomised controlled studies

But strong evidence base in COPD

Improves QoL, reduces breathlessness

Deconditioning, breathlessness, nutritional deficit, fatigue and social isolation

Oxygen may be required to allow exercise

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Other measures

Opioids

Anxiolytics

Relaxation and distraction techniques

Breathlessness clinic

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Causes of acute deterioration

Reflux

Infection

Pneumothorax

pulmonary embolism

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Lung transplantation

Patients <65

TLCO <40%

70-80% 1 year survival and 50% 5 yr survival

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Prognosis

Variable

Median survival 2.5-3.5 years

Improved survival associated with young age, female sex, less honeycombing and better lung function at diagnosis

Death from respiratory failure or infection

Lung cancer common

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Ventilatory pump failure

Myopathies- myotonic dystrophy

- muscular dystrophy

Neuropathy- MND

- bilateral diaphragm paralysis

- Guillain- Barr

é

NMJ abnormalities- myasthenia gravis

-anticholinesterase poisoningChest wall – obesity (often assoc obstructive sleep apnoea) - scoliosis - post thoracoplasty

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Respiratory consequences of obesity

Obstructive sleep apnoea

Obesity hypoventilation syndrome

Acute hypercapnic respiratory failure

Postsurgical complications

Pulmonary hypertension

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Mrs MC

54

♀

Asthma 38 years

No ITU admissions

Salbutamol prn only

Never smoked

Alcohol ½ bottle whisky dayObese 127kg

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Admission

Presented with 4/7 SOB and wheeze

No cough

Given chlordiazepoxide, beclomethasone and atrovent inhalers by GP

No better so called ambulance

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Examination

PEFR 150 (450) L/min

RR 22

Sats 95% on 2 L/min

P 130 regular

Diminished breath sounds throughout

No wheeze

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ABG

pH 7.217

pCO

2

9.57

pO

2

10.23HCO3- 28.5BE -1.3

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Admission bloods

Trop T 0.198

K

+

5.2

Urea 20.9

Creat 336

Hb 15.0WCC 14.3Neut 11.9Plt 255ALT 1230Bilirubin 39GGT 246Alb 38

ALP 130C. Ca2+ 2.02INR 1.7

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Immediate treatment

Nebulised salbutamol and atrovent

Steroids

Pabrinex and vitamin B

Regular chlordiazepoxide stopped

BiPAP commenced

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Respiratory review

Recent increase in alcohol consumption

Recent rapid weight gain

Daytime somnolence, falling asleep at work

Epworth score 14/24

Continue nocturnal BiPAP

Sleep study as inpatient

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Sleep study

Low sats throughout- down to 70%

Multiple hypopnoeas

Some apnoeas

Lots of paradox

AHI- 29

Compatible with OSAHS

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Discharge ABG

pH 7.417

pCO

2

5.61

pO

2

8.30HCO3- 26.3BE 1.5

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Follow up

Weight loss 106.5kg

No alcohol since discharge

No daytime sleepiness

Epworth score 0/24

ABG continue to improve

BiPAP stopped

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Severe obesity BMI > 30 kg/m

2

and

diurnal PaCO

2

> 45 mmHg (6 kPa)

In the absence of other known cause of hypoventilation

Olson et al

Am J Med 2005

Obesity hypoventilation syndrome

Definition

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Morbid obesity

OSA

dyspnoea daytime hypersomnolence

Pèrez de Llano

Chest 2005

Obesity hypoventilation syndrome

Clinical presentation

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PREVALENCE

Nowbar, Am J Med 2004

Mokhlesi B, CHEST 2007

Hospitalised patients

Stable state OSA

Increases

with

BMI;

Prevalence

>25%

for

BMI>40

kg/m

2

and

>50%

for

BMI>50

kg/m

2

15% in the

general

population of ambulatory obese patients?

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Mechanisms underlying hypercapnia in obesity

Mokhlesi et al. Proc Am Thorac Soc 2008

Neurohormonal abnormalities

1

2

3

4

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Berg Chest 2001

Mokhlesi Proceedings ATS 2008

Compared with obese control subjects, patients with OHS were statistically much more likely to have been diagnosed with:

Congestive heart failure (OR 9; 95% CI, 2.3–35)

Angina pectoris (OR, 9; 95% CI, 1.4–57.1)

Cor pulmonale (OR, 9; 95% CI, 1.4–57.1)

Obesity hypoventilation syndrome

High prevalence of associated cardiovascular morbidity in observational cohorts

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Treatment

Weight loss

CPAP

BiPAP

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Highly prevalent and easy to diagnose but underdiagnosed

Non invasive ventilation (NIV) improves blood gases, sleep, daytime sleepiness and mortality

Impact of NIV on cardiovascular morbidity?

Assessment and treatment of cardiovascular and metabolic risk recommended in OHS patients in association with NIV

Obesity hypoventilation syndrome

Take home message

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Mr

CB

75 male

Raised PSA – normal bone scan, CT CAP unremarkable

General deterioration

Wgt loss, lethargy,

poor appetite

2 weeks dyspnoea – unable to sleep

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No wheeze, no crackles

Sats 84% on air

Nil to find on examination

CXR small volume lungs

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pH 7.24

pCO

2

13.41

pO

2

18.31

HCO3 42.5BE 9.8

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Confused

Minimal history available

Dysarthric

Generally wasted

Poor respiratory effort

Thoraco-abdominal paradox

Multiple fasciculations

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MND and respiratory failure

Respiratory failure in MND is common and a frequent cause of death

It may be the cause of presentation

Deterioration may be rapid

Multidisciplinary involvement is key

There is some evidence that NIV improves survival and quality of life

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Mrs JT

45 female

Kyphoscoliosis from birth

Spinal fusion aged 14

Married

2 teenage chidren

Nil else in PMH

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4-6 week history of dyspnoea on exertion

1 week history of ankle oedema and new onset confusion

Started on salbutamol and frusemide by GP

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Wheezy

JVP raised, oedema to knees

WCC 12 CRP 50

Na 115

pH 7.307 pCO

2

14.75

pO2 11.7 HCO3 54.1

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Commenced on BiPAP

Deteriorating conscious level and worsening acidosis

Intubated

Echo- pulm hypertension, PAP 65mm Hg

Weaned to BiPAP

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4/52 post discharge

Using BiPAP all night 15:5

Exercise tolerance improved to half a mile

Oedema resolved

No daytime somnolence

pH 7.42 pCO

2

8.16 pO2 7.32 HCO3 38.4Commenced on LTOT IPAP increased 18

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2 years

Unlimited ET

BiPAP 24:5

pH 7.45 pCO

2

5.33 pO

2

9.77 HCO3 27.2Echo PAP 35mm Hg

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Differential diagnosis of restrictive

spirometry

How to assess for respiratory muscle weakness

The importance of OHS