Idiopathic pulmonary fibrosis (IPF) –
The need for early recognition and referral
PRC-2128
It can be difficult to separate idiopathic pulmonary fibrosis
(IPF) from other conditions
•
IPF is a rare but fatal lung disease, with a lower survival rate than many
common malignancies1,2
•
Treatment is available,3 and early diagnosis can make a difference
•
It is important to identify patients presenting with the classical signs and
symptoms so that they can be referred to a pulmonologist
1.
2.
3.
Collard HR, et al. Am J Respir Crit Care Med 2003;168:538-42
Verdecchia A, et al. Lancet Oncol 2007;8:784-96
ATS/ERS/JRS/ALAT. Am J RespirCrit Care Med 2011;183:788-824
Overview
•
These slides provide information about:
– IPF pathophysiology, prevalence and prognosis
– Clinical features and diagnosis of IPF
– Recognising IPF in clinical practice
– Patient management
Idiopathic pulmonary fibrosis (IPF):
Pathophysiology, prevalence and prognosis
Idiopathic pulmonary fibrosis (IPF)
•
IPF is a fatal lung disease characterised by an unpredictable decline of lung
function due to lung fibrosis1,2
•
Progressive decline in lung function restricts routine physical activity1,2
•
Notable inter- and intra-patient variability in deterioration3,4
•
Prognosis is extremely poor
– Median survival 2–5 years2-5
•
1.
2.
3.
4.
5.
The cause of IPF is not known1,2
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
ATS/ERS. Am J Respir Crit Care Med 2002;165:277-304
Collard HR, et al. Am J Respir Crit Care Med 2003;168:538-542
Kim DS, et al. Proc Am Thorac Soc. 2006;3:285-292
Meltzer EB, Noble PW. Orphanet J Rare Dis. 2008;3:8
Definition of idiopathic pulmonary fibrosis (IPF)
•
A chronic, progressive, fibrosing type
of pneumonia affecting the tissue and
space around the air sacs of the
lungs (the interstitium)
•
Of unknown cause (‘idiopathic’)
•
Occurring primarily in older adults
(>45 years old)
•
Associated with specific radiologic
and/or histopathologic criteria1
1.
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
Incidence and prevalence of idiopathic pulmonary fibrosis
(IPF)
IPF is an orphan disease
•
Incidence ranges from 4.6 to 8.8 per 100,0001-4
– Up to 30,000–35,000 patients diagnosed with IPF in Europe every year5
•
Prevalence ranges from 14 to 28 per 100,0003,4
– Up to 80,000–110,000 patients with IPF in Europe5
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1.
2.
3.
4.
5.
6.
Incidence and prevalence increase with age, and are higher in males than
females6
Gribbin J, et al. Thorax. 2006;61(11):980-985
Navaratnam V et al. Thorax. 2011;66(6):462-7
Raghu G et al. Am J Respir Crit Care Med. 2006;174(7):810-816
Fernández Pérez ER, et al. Chest. 2010;137(1):129-137
Eurostat data, January 2012
Meltzer EB, Noble PW. Orphanet J Rare Dis. 2008;3:8
Idiopathic pulmonary fibrosis (IPF) pathophysiology
IPF is the result of an abnormal and persistent fibrotic response1,2
•
1.
2.
The chronic fibrotic process in IPF ultimately converts the lungs
into shrunken, firm, dense, and honeycombed structures
King TE Jr, et al. Lancet 2011;378:1949-1961
Selman M, et al. Ann Intern Med 2001;134:136-151
Prognosis
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Idiopathic pulmonary fibrosis (IPF) is an inevitably fatal disease,
with a median survival of 2 to 5 years following diagnosis1
•
IPF has a lower survival rate than many cancers2-4
Comparison of the 5-year survival rate for IPF and some common
malignancies
100
5-year survival rate, %
90
80
70
60
50
40
30
20
10
0
IPF
1.
2.
3.
4.
Lung cancer
(limited SCLC)
Costabel U. Eur Respir Rev 2012;21:140
Collard HR, et al. Am J Respir Crit Care Med 2003;168:538-542
Sørensen M, et al. Ann Oncol. 2010;21 Suppl 5:v120-125
Verdecchia A, et al. Lancet Oncol 2007;8:784-796
Chronic myeloid
leukaemia
Prostate cancer
Breast cancer
Clinical course of idiopathic pulmonary fibrosis (IPF)
•
IPF disease progression is variable, irreversible, and inevitably fatal1,2
•
At the time of diagnosis it is not possible to predict whether patients will
have a slower or faster rate of decline1
1.
2.
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
Kim DS, et al. Proc Am Thorac Soc. 2006;3:285-292
The importance of early recognition and referral
Early identification of idiopathic pulmonary fibrosis (IPF) is important:
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To confirm diagnosis and ensure timely referral1
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Because effective treatment to slow disease progression is available2
– Early intervention may improve outcomes
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To list for lung transplant2
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Because there are several ongoing trials for potential new therapies3
•
To give patients the opportunity to make the most of their remaining time
1.
2.
3.
ATS/ERS. Am J Respir Crit Care Med 2002;165:277-304
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
Available at www.clinicaltrials.gov [Accessed May 2013]
Clinical features and diagnosis of idiopathic
pulmonary fibrosis (IPF)
Clinical features of idiopathic pulmonary fibrosis (IPF)
Key presenting features include:1-5
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Increasing breathlessness on exertion
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Dry, non-productive cough
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Over 45 years old
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Basal inspiratory crackles on auscultation
1.
2.
3.
4.
5.
ATS/ERS. Am J Respir Crit Care Med 2002;165:277-304
Meltzer EB, Noble PW. Orphanet J Rare Dis. 2008;3:8
Kim DS, et al. Proc Am Thorac Soc. 2006;3:285-292
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
Cottin V, Cordier J-F. Eur Respir J. 2012;40:519-21
Bibasilar inspiratory crackles may indicate idiopathic
pulmonary fibrosis (IPF)
Fine crackles on inspiration are
characteristic of IPF
•
Similar to the sound heard when
gently separating a strip of Velcro
•
Predominantly located in the lower,
posterior (basal) areas of the lung
Bilateral fine crackles should raise the suspicion of IPF and prompt referral for
pulmonary function tests prior to a thin-slice HRCT scan without contrast agent
1.
Cottin V, Cordier J-F. Eur Respir J. 2012;40:519-21
Diagnostic criteria for idiopathic pulmonary fibrosis (IPF)
The diagnosis requires exclusion of known causes, plus radiological and/or histological
criteria1
1.
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
The need for a multi-disciplinary approach in idiopathic
pulmonary fibrosis (IPF)
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Diagnosis of IPF is based on high-resolution computed tomography (HRCT)
and, when needed, histopathologic criteria, requiring a multi-disciplinary
approach1
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Multi-disciplinary discussion increases the accuracy of diagnosis1,2
1.
2.
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
Flaherty KR, et al. Am J Respir Crit Care Med. 2004;170:904-910
HRCT has a critical role in the diagnosis of idiopathic
pulmonary fibrosis (IPF)
•
A pulmonary function test may
warrant further investigation to
analyse lung structure
HRCT scan of lungs with UIP pattern
•
High-resolution computed
tomography (HRCT) is an essential
component of the diagnostic
pathway in IPF1
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HRCT has greatly increased the
accuracy of IPF diagnosis1
– The positive predictive value of a
HRCT diagnosis is 90 to 100%
•
1.
2.
A thin slice HRCT scan without
contrast agent is required2
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
ACR practice guideline for HRCT in adults 2009. Available at www.acr.org
A normal chest x-ray cannot exclude idiopathic pulmonary
fibrosis (IPF)
Terms in radiology reports that
warrant attention:
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Interstitial markings/changes
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Fibrosis
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Lower lobe predominance
Abnormal chest x-ray
1.
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
Recognising idiopathic pulmonary fibrosis
(IPF) in clinical practice
The typical IPF patient
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Over 45 years old
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Smoker or ex-smoker
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Male > female
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Exertional dyspnoea usually present
for more than 6 months
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Dry, non-productive cough
•
Comorbidities1,2
– May include
• Pulmonary hypertension
• Gastro-oesophageal reflux
• Emphysema
– These may be sub-clinical (asymptomatic)
1.
2.
3.
4.
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
Meltzer EB, Noble PW. Orphanet J Rare Dis. 2008;3:8
Kim DS, et al. Proc Am Thorac Soc. 2006;3:285-292
ATS/ERS. Am J Respir Crit Care Med 2002;165:277-304
Bibasilar inspiratory crackles may indicate
idiopathic pulmonary fibrosis (IPF)
•
Fine crackles on inspiration are
characteristic of IPF
•
Similar to the sound heard when gently
separating a strip of Velcro
•
Predominantly located in the lower,
posterior (basal) areas of the lung
•
Best detected during slow, deep breaths
•
Present throughout the inspiratory time
Bilateral fine crackles should raise the suspicion of IPF and prompt referral for
pulmonary function tests prior to a thin-slice HRCT scan without contrast agent
1.
Cottin V, Cordier J-F. Eur Respir J. 2012;40:519-21
When and where to refer
The patient should be referred to a pulmonologist
if the following are noted:
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Progressive breathlessness on exertion ≥3 months
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Dry cough ≥3 months
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Basal inspiratory crackles on auscultation
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Over 45 years old
1.
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
Idiopathic pulmonary fibrosis (IPF) patient
management
Pharmacological therapy for idiopathic pulmonary fibrosis
(IPF)
Approved
•
There is approved pharmacological therapy for the treatment of IPF
available1
Under investigation
•
1.
2.
A number of therapies are in clinical trials for the treatment of IPF2
ATS/ERS/JRS/ALAT. Am J RespirCrit Care Med 2011;183:788-824
Available at www.clinicaltrials.gov [Accessed May 2013]
Non-pharmacological therapy for
idiopathic pulmonary fibrosis (IPF)
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Oxygen therapy
– Recommended for patients with IPF and clinically
significant resting hypoxaemia1
•
Lung transplantation
– Only available for a small number of patients with IPF1,2
1.
2.
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
Meltzer EB, Noble PW. Orphanet J Rare Dis. 2008;3:8
Providing additional support
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Pulmonary rehabilitation
– Pulmonary rehabilitation programs involve aerobic conditioning, strength and
flexibility training, educational lectures, nutritional interventions and psychosocial
support, and may provide benefits to the majority of patients with IPF1
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Education and support
– Patients and caregivers should be provided with appropriate information and
support
– Specific goals for palliative care include relief from physical and emotional
suffering and consideration for psychological and spiritual support1
1.
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824
Summary
•
IPF is a progressive, irreversible and ultimately fatal fibrotic lung disease
with a lower survival rate than many common cancers1-3
•
Key presenting clinical features include:1
– Increasing breathlessness on exertion
– Dry, non-productive cough
– Over 45 years old
– Basal inspiratory crackles on auscultation
•
1.
2.
3.
Timely diagnosis and referral to a pulmonologist is key to prompt initiation of
treatment, which may improve outcomes
ATS/ERS/JRS/ALAT. Am J RespirCrit Care Med 2011;183:788-824
Collard HR, et al. Am J Respir Crit Care Med 2003;168:538-42
Verdecchia A, et al. Lancet Oncol 2007;8:784-96
Back-up slides
IPF is the most common type of idiopathic interstitial
pneumonia
1.
ATS/ERS. Am J Respir Crit Care Med 2002;165:277-304
Common IPF misdiagnoses
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IPF is often diagnosed at an advanced stage of the disease1,2
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It is commonly misdiagnosed as other conditions which have some
symptoms that mimic IPF1,2
– Chronic obstructive pulmonary disease (COPD)
– Congestive heart failure (CHF)
– Other lung diseases
– Connective tissue diseases
1.
2.
Meltzer EB, Noble PW. Orphanet J Rare Dis. 2008;3:8
Collard HR, et al. Respir Med. 2007;101(6):1350-4
Taking a patient history
A thorough history should aim to eliminate other causes of ILDs,
including medication and environmental exposures1
Assess:
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1.
Onset of symptoms
Family history
Smoking
Comorbidities
Occupational/environmental exposures
Allergies
Pets
Medications
Previous malignancy and treatment
ATS/ERS/JRS/ALAT. Am J Respir Crit Care Med 2011;183:788-824