Restrictive lung disease -

Restrictive lung disease -

Restrictive lung disease Dr Duncan Powrie Consultant Chest Physician Southend University Hospital December 2018 Simple spirometry FEV1 = 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) FEV1/FVC ratio = a calculation using the above measurements (% of total that can be forced out in the first second ) 2 Performing spirometry

1 Record the patients 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 * severe patients

may take up to twice 15 seconds the procedure This should give 3 readings, with at least 2 within 100ml or 5% of each other Consistent result Inconsistent result 3

Obstructive vs.restrictive patterns If the ratio FEV1/FVC <70%, obstruction is present If this ratio is normal but FEV1 and FVC are both reduced, restrictive pattern is present A 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 disease FEV1 reduced (<80%) reduced (<80%) FVC normal or reduced reduced (<80%) FEV1/FVC ratio reduced (<70%) normal (>70%) 5

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) Oximetry Blood gases

Sleep study Pulmonary fibrosis Progressive exertional breathlessness Dry cough Arthralgia 20% Weight loss

Finger clubbing in 50% End inspiratory velcro crackles Cyanosis Cor pulmonale Epidemiology 6-28/ 100 000yr M:F 1.7:1

Median age of diagnosis 70 Uncommon before 50 Risk factors

Exposure to metal or wood dust Organic solvents Mycotoxins EBV, Hepatitis C Cigarette smoking Family history 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 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 Breathlessness Hypoxia is common as is desaturation on exercise

LTOT if pO2 < 7.3 kPa or <8 if signs of pulmonary hypertension Ambulatory oxygen if desaturates on exertion Cough

Treat reflux Consider simple linctus Oral codeine Consider oramorph or MST in end stage disease 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 Other measures

Opioids Anxiolytics Relaxation and distraction techniques Breathlessness clinic Causes of acute

deterioration Reflux Infection Pneumothorax pulmonary embolism

Lung transplantation Patients <65 TLCO <40% 70-80% 1 year survival and 50% 5 yr survival 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 Ventilatory pump failure Myopathies- myotonic dystrophy

- muscular dystrophy Neuropathy- MND - bilateral diaphragm paralysis - Guillain- Barr NMJ abnormalities- myasthenia gravis -anticholinesterase poisoning Chest wall obesity (often assoc obstructive sleep apnoea) - scoliosis - post thoracoplasty

Respiratory consequences of obesity Obstructive sleep apnoea Obesity hypoventilation syndrome Acute hypercapnic respiratory failure

Postsurgical complications Pulmonary hypertension Mrs MC 54 Asthma 38 years No ITU admissions Salbutamol prn only Never smoked

Alcohol bottle whisky day Obese 127kg Admission Presented with 4/7 SOB and wheeze No cough Given chlordiazepoxide, beclomethasone and atrovent inhalers by GP No better so called ambulance

Examination PEFR 150 (450) L/min RR 22

Sats 95% on 2 L/min P 130 regular Diminished breath sounds throughout No wheeze ABG pH 7.217 pCO2 9.57

pO2 10.23 HCO3- 28.5 BE -1.3 Admission bloods

ALT 1230 Trop T 0.198 K+ Bilirubin

5.239 Urea246 GGT 20.9 Creat38336 Alb Hb 130 ALP 15.0 WCC

C. Ca2+14.3 2.02 Neut1.711.9 INR Plt 255 Immediate treatment

Nebulised salbutamol and atrovent Steroids Pabrinex and vitamin B Regular chlordiazepoxide stopped BiPAP commenced

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 Sleep study

Low sats throughout- down to 70% Multiple hypopnoeas Some apnoeas Lots of paradox AHI- 29 Compatible with OSAHS Discharge ABG pH 7.417

pCO2 5.61 pO2 8.30 HCO3- 26.3 BE 1.5 Follow up Weight loss 106.5kg No alcohol since discharge No daytime sleepiness

Epworth score 0/24 ABG continue to improve BiPAP stopped Obesity hypoventilation syndrome Definition Severe obesity BMI > 30 kg/m2 and diurnal

PaCO2 > 45 mmHg (6 kPa) In the absence of other known cause of hypoventilation Olson et al Am J Med 2005 Obesity hypoventilation syndrome Clinical presentation

Prez de Llano Chest 2005 Morbid obesity OSA dyspnoea daytime hypersomnolence

PREVALENCE Increases with BMI; Prevalence >25% for BMI>40 kg/m2 and >50% for BMI>50 kg/m2 Stable state OSA Hospitalised patients 15% in the general population of ambulatory obese patients?

Mokhlesi B, CHEST 2007 Nowbar, Am J Med 2004 Mechanisms underlying hypercapnia in obesity Neurohormonal 3 abnormalities 2

1 4 Mokhlesi et al. Proc Am Thorac Soc Obesity hypoventilation syndrome igh prevalence of associated cardiovascular morbidity in observational cohorts

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, 2.335) Angina pectoris (OR, 9; 95% CI, 1.457.1) Cor pulmonale (OR, 9; 95% CI, 1.457.1) Berg Chest 2001 Mokhlesi Proceedings ATS 2008

Treatment Weight loss CPAP BiPAP besity hypoventilation syndrome home message 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 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 No wheeze, no crackles Sats 84% on air Nil to find on examination CXR small volume lungs

pH 7.24 pCO2 13.41 pO2 18.31 HCO3 42.5 BE 9.8

Confused Minimal history available Dysarthric Generally wasted Poor respiratory effort Thoraco-abdominal paradox Multiple fasciculations

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

Mrs JT 45 female Kyphoscoliosis from birth

Spinal fusion aged 14 Married 2 teenage chidren Nil else in PMH 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 Wheezy JVP raised, oedema to knees WCC 12 CRP 50 Na 115 pH 7.307 pCO2 14.75 pO2 11.7 HCO3 54.1 Commenced on BiPAP

Deteriorating conscious level and worsening acidosis Intubated Echo- pulm hypertension, PAP 65mm Hg Weaned to BiPAP 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 pCO2 8.16 pO2 7.32 HCO3 38.4 Commenced on LTOT

IPAP increased 18 2 years Unlimited ET BiPAP 24:5 pH 7.45 pCO2 5.33 pO2 9.77 HCO3 27.2 Echo PAP 35mm Hg Differential diagnosis of restrictive spirometry

How to assess for respiratory muscle weakness The importance of OHS

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