management of acute interstitial lung diseases
TRANSCRIPT
Management of AcuteInterstitial Lung Diseases
Objectives: Summary of Interstitial Lung Disease Classification:
Reminder
Case based illustrations to cover ACUTE RespiratoryPresentations in Known ILD Unknown ILD
Common themes in Management
When to refer to ICU
ILD – Interstitial Lung disease
Classification of ILDs and DPLDs
*Desquamative interstitial pneumonia can occasionally occur in non-smokers. ATS, American Thoracic Society; DPLD, diffuse parenchymal lung disease, ERS, European Respiratory Society; ILD, interstitial lung disease. 1. ATS and ERS. Am J Respir Crit Care Med 2002;165:277–304; 2. Travis et al. Am J Respir Crit Care Med 2013;188:733–748.
Image adapted from ATS and ERS, 20021 and ATS
and ERS, 20132
Unknown Causes: Known Causes: Idiopathic Pulmonary
Fibrosis
Idiopathic Non specific interstitial pneumonia
Sarcoidosis
Cryptogenic organising pneumonia
Occupational: asbestosis/silicosis
Environmental: Hypersensitivity pneumonities(pigeons/birds)
Immune: Connective Tissue Disease (RA; systemic sclerosis)
Smoking: DIP and RB-ILD
Drugs: Any!
Is this disease inflammatory or fibrotic?
Inflammation Fibrosis
Idiopathic Pulmonary Fibrosis
SarcoidosisHypersensitivity PneumonitisConnective Tissue DiseaseCryptogenic Organising pneumoniaDrug ILDCellular NSIPSmoking related ILD
CTD – Connective Tissue Disease; NSIP – Non specific interstitial pneumonia; AIP – Acute interstitial pneumonia; DIP – Desquamatous interstitial penumonia; RB-ILD – Respiratory Bronchiolitis Interstitial lung Disease; COP – Cryptogenic organising pneumonia; IPF – Idiopathic Pulmonary Fibrosis; HSP –Hypersensitivity pneumonitis; f – fibrotic; c - chronic
History and Examination very important
Symptoms of Connective Tissue Disease
Reynauds, swollen/stiff joints, Dry eyes dry mouth, lethargy, mouth ulcers, photosensitive rash
Occupational/environmental exposures
Birds; Mould; Hot tub; asbestos
Drug history Any!
www.pneumotox.org
Soft signs of Connective Tissue Disease:
Mechanic hands;
periungal erythema
sclerodactyly
tight skin
telangectasia
Case 1: 67 year old male
3 week history of increasing breathlessness
Reduced exercise tolerance from 200 yards to breathlessness at rest
Cough with yellow sputum
Feeling hot
No chest pain No rigors
PMH:
Idiopathic Pulmonary Fibrosis diagnosed 12 months ago
Hypertension
Previous Myocardial Infarction 5 years ago
Angina well controlled
Medication:
Pirfenidone
Bisoprolol
Aspirin
Ramipril
GTN spray
Ex smoker 40 pack year history
Examination:
SOB at rest
Saturations 88% on air
RR 20/min
Pulse 120 BP 120/60
Apyrexial
Clubbed
Bilateral fine crackles
Investigations:
Hb 13 WCC 13 N 9.0
U+E, LFT normal
CRP 50
Sputum MCS requested
ABG pH 7.45 pCO2 5.5 PO2 9.0 Lactate 0.5
Chest x ray:
Diagnosis:
Community acquired pneumonia
Background of IPF
Management:
Oxygen therapy:
FiO2 28% Saturations 94%
Antibiotics: Augmentin and Clarithromicin
Clexane prophylaxis
Question often asked: Do you stop the Pirfenidone?
POLL OPEN
Vote Trigger
1 Yes0%
2 No0%
3 Don’t know0%
24 hours later: Feeling more short of breath RR 30/min
Working hard; using accessory muscles
Vague chest discomfort/tightness/sharp pain centre of chest – worse on breathing in
Oxygen requirements increased to FiO2 60% to maintain Sats 93%
CRP 130 WCC 18
What would you do next?POLL OPEN
Vote Trigger
1 Intubate and ventilate0%
2 Perform CTPA0%
3 Escalate antibiotics to second line0%
4 Perform Echocardiogram0%
5 Perform HRCT0%
6 High flow nasal oxygen0%
Management: High Flow nasal oxygen
FiO2 50%
Escalated antibiotics Tazocin
Additional tests: Urine leigonella and
pneumoccocal antigen
PCR Respiratory viruses
Sputum MCS
Performed HRCT to assess for ground glass (could request HRCT/CTPA if PE in differential)
Too unwell for bronchoscopy and lavage
Need more clinical information:
Known IPF –
What is baseline disease severity – FVC 65% DLCO 45%
Baseline exercise tolerance 100-200yards; Progressive decline over 12months Previously 500 yards a year ago
Differential Diagnosis in patients with Acute Worsening of ILD.
Heart Failure – Prior history, CxR (kerley B lines, Cardiomegaly, pleural effusions) ECG, ECHO, raised BNP
Pulmonary Embolism – Typical history; normal CxR, Raised D dimer
Pneumothorax – CxR
Infection – History, CxR, Raised inflammatory markers, Positive cultures
Acute Exacerbation of IPF/ILD
Revised Definition of AE-IPF Revised definition:
An acute, clinically significant respiratory deterioration characterised by evidence of new widespread alveolar abnormality
Revised Diagnostic criteria:
Previous or concurrent diagnosis of IPF
Acute worsening or development of dyspnoea typically less than 1 month duration
New ground glass +/- consolidation on CT on background of UIP pattern
Exclude cardiac failure or fluid overload
Unpredictable course
5-10% AE rate per year in clinical trials
As high as 15% in retrospective studies
Frequency increased in more advanced disease
Accountable for 40% of IPF deaths
Risk Factors:
More severe disease Lower FVC DLCO Oxygen use More extensive CT
High BMI
Cardiovascular disease
Pulmonary Hypertension
Winter-Spring months
Potential Triggers:
Infection
Drugs eg chemotherapy 8-30%, biologics, radiotherapy
Aspiration
Post procedural: Lung bx 2-4% Resection up to 30%
Management: High Flow nasal oxygen
Maintain Saturations above 90%
Broad spectrum iv antibiotics
If no improvement after 3 days (my practise – not evidence based) as everything pointing to infection
Start iv methylprednisolone 500mg-1g of for 3 consecutive days to treat AE-IPF (diffuse alveolar damage)
ATS/ERS Majority of patients with acute exacerbation of IPF
should be treated with corticosteroids, but corticosteroids may not be reasonable in the minority (weak recommendation, very low quality evidence)
Uncontrolled studies only: 4: Steroids ; 4: Cyclophosphomide 5: Polymyxin B
What others may do:
Cyclophosphomide : 4 studies
Tacrolimus: Horita et al 2011 Median survival>92daysvs 38days
Rituximab, plasma exchange and iv ImmunoglobulinDonahoe et al 2015 n=11 vs historical n=20 1 yearsurvival 46% vs 0%
Thrombomodulin
Do you stop antifibrotics? Omit if cant maintain oral intake
Ideally continue as shown to prevent AE-IPF
Case 2: 37 year old female
Insurance sales
Non smoker
PMH: Pancreatitis and cholecystectomy 3 years ago
Oct 15 developed Upper resp tract viral infection
Chronic cough and breathlessness ETT 100yards
Associated wheeze
No benefit from inhalers or PPI
Much better after a course of steroids for presumed asthma
May 16
Developed severe breathlessness, cough and fever
CRP 50 WCC 13 N9.0
Treated with oxygen therapy and antibiotics for Community acquired pneumonia
Sep 17:
Not quite right since discharge
Progressive breathlessness and cough for a few month
Muscle aches all over 4 weeks
Fever
Generalised muscle weakness
Examination:
Flushed
P120 BP 120/50
RR 20/min Sats 88% on air
Temp 37.5
Generalised muscle pain
Dry cracked hands
Bibasal reduced air entry and basal creps
Investigations:
Hb 13 WCC 13 N 9.0
U+E, LFT normal
CRP 100
Sputum MCS requested
ABG pH 7.45 pCO2 5.5 PO2 9.0 Lactate 0.5
Chest x ray:
Diagnosis:
Community acquired pneumonia
?Viral
Management:
Oxygen therapy:
FiO2 60% Saturations 94%
Antibiotics: Augmentin and Clarithromicin
Clexane prophylaxis
What would you do next?POLL OPEN
Vote Trigger
1 Start antiviral therapy0%
2 Perform HRCT0%
3 High flow nasal oxygen0%
4 Perform Viral PCR0%
5 Perform CK0%
Additional tests: Urine leigonella and
pneumoccocal antigen
PCR Respiratory viruses
Sputum MCS
CK>1000
Autoimmune screen
Performed HRCT
Awaited
Awaited
Jo-1 positive
Anti-synthetase Syndrome Idiopathic Inflammatory
myopathies
Antibodies to tRNAacetylating enzymes
Jo1; PL7; EJ; OJ; KS
ILD>60% often predates systemic symptoms
60-85%% survival
Myositis elevated CK
Relatively acute onset disease
Constitutional symptoms
Reynauds
Mechanical hands
Inflammatory polyarthropathy
Can have DM skin lesions
Treatment: Prednisolone 0.5(-1mg)/kg taper dependent on
response
If acute high dose 1mg/kg or Methylprednisolone 1g od for 3 days
Second line: Cyclophosphomide IV vs Rituximab
Rituximab for anti-synthetase syndrome- ILD
Norwegian Retrospective case series n=11
Severe and progressive ILD
Failed Cycloph
Ritux stabilised or improved lung function in 7/11 over 6 months
Well tolerated
1 fatal infection
Retrospective
N=50 (33 CTD; 6 HSP; 11 Other)
Severe disease baseline FVC 44% DLCO 24.5%
Unresponsive to conventional Rx
6% improvement in FVC; stabilisation of DLCO
4% serious infection
Do you escalate to ICU? Not all ILD is IPF
IPF 30% of all ILDs
How convert complicated nomenclature to predicting prognosis hence support
decision making for ICU
INFLAMMATION.REVERSIBLETREATABLEBETTER PROGNOSIS
FIBROSIS.IRREVERSIBLEPOOR TREATMENTPOORER PROGNOSIS
CTD NSIPSSc NSIPDM/PM
HSP - Acute
Drug Toxicity
SarcoidStage 1,2,3
AIP
COP
EO Pneum
DIP/RB-ILD
IPF Fibrotic NSIP
Stage 4 Sarcoid
CTD – UIPRA
CONSIDER ICU ADMISSIONREVERSIBLETREATABLEBETTER PROGNOSISIF COMORBITIES ALLOWCASE BY CASE BASISPATIENT FACTORS
RISK ASSESSMENT vsPROGNOSISIRREVERSIBLEPOOR TREATMENTPOORER PROGNOSISCOMORBIDITIES
INFLAMMATION.REVERSIBLETREATABLEBETTER PROGNOSIS
FIBROSIS.IRREVERSIBLEPOOR TREATMENTPOORER PROGNOSIS
Ground Glass Traction Bronchiectasis
Architectural Distortion
FibrosisHoney Combing
Risk Assessment in Fibrotic Lung Disease
No validated prognostic system
Prognosis is dependent on the type of Interstitial Lung
Disease
Staging: No ideal score Age
Dyspnoea scale
FVC
DLCO
Desaturation during 6MWT
Extent of fibrosis on HRCT
PH
What is the extent of irreversibility? – Fibrosis
Fibrosis/Traction bronchiectasis/UIP
Difficult to quantify – Automated CALIPER
Different patterns: UIP worse than NSIP
Severity of Lung Function – Low FVC Low DLCO at baseline
What is the prior history? Prior history of decline FVC decline >10% DLCO>15%
Age
Impact of Comorbidities in ILD on Risk of death
Are there any models in ILD? YES for IPF;
Risk Assessment in Fibrotic Lung Disease.
Conclusion: Wide differential in acute deterioration of ILD including
infection, PE or acute exacerbation
Consider treating acute exacerbation of ILD with methylprednisolone
I+V not recommended for IPF but consider for all non-IPF