16. • Interstitial lung
diseases is a broad
category of
pulmonary diseases
that includes
idiopathic interstitial
pneumonias
• Idiopathic interstitial
pneumonias is
another category of
diseases that
includes idiopathic
Interstitial Lung Disease
Idiopathic interstitial
pneumonia
Idiopathic
Pulmonary
Fibrosis
17. Interstitial Lung Disease
• The Idiopathic Interstitial Pneumonias (IIP) are
an important category of ILD
• Idiopathic pulmonary fibrosis (IPF) is clinically
the most important type of IIP
• Idiopathic pulmonary fibrosis (IPF) is a
devastating disease,with a 5-yr survival rate of
only 20–30% following diagnosis.
18. Question
What is the most important distinction to
make?
UIP/IPF Everything Else
Vs.
19. Why?
Complete recovery is possible with all
types of Idiopathic Interstitial
Pneumonitis
Except UIP/IPF !!!
23. • Major Criteria:
– Exclude other causes of ILD
– PFTs consistent with restrictive pattern and/or
impaired gas exchange
– HRCT with Bibasilar, reticular abnormalities and
minimal ground glass opacities
– Transbronchial lung biopsy or BAL showing
nothing to suggest other diagnosis
ATS/ERS CRITERIA FOR DIAGNOSIS OF IPF IN
ABSENCE OF SURGICAL LUNG BIOPSY
24. Diagnosis without Biopsy
• Minor Criteria:
– Age > 50 yo
– Gradual onset dyspnea on exertion , not
explained by another diagnosis
– Duration >3 months
– Bibasilar, inspiratory crackles (dry or “Velcro”-
type in quality)
ALL of the major criteria plus at least THREE minor
criteria
25. Clinical Evaluation: History, PE, CXR, PFTs,
6MWT
Not IIP Potential IIP
HRCT
Diagnostic of IPF or other
diffuse lung disease
Diagnosis uncertain
Surgical lung
biopsy
Transbronchial Bx or
BAL
Diagnostic Nondiagnostic
IPFNot IPF
Approach to Diagnosing IPF
Adapted from ATS/ERS Consensus Statement. Am J Respir Crit Care
Med. 2002.
28. Therapeutic Approaches to IPF
Antioxidant agents
Glutathione
N-acetylcysteine
Others
Antifibrotic agents
Colchicine
D-penicillamine
IFN-
IFN-
1950s 1990s 2005
Immunosuppressants
Azathioprine
Cyclophosphamide
Immunomodulator
s
Etanercept
Pirfenidone
IFN-
Adapted from ATS/ERS. Am J Respir Crit Care Med. 2000
29. Approach to treatment
Treatment should be started
1. At the first identification of clinical or physiological
evidence of impairment of lung function
2. Early in the course of the disease before
irreversible fibrosis has developed
• No data exist to document that any of the current
treatment approaches improves survival or the
quality of life for patients with IPF.
30. Approach to treatment
• Therapy is not indicated for all patients and the
risk of complications of treatment must be weighed
against the potential benefit
1. Age >70 years
2. Extreme obesity
3. Concomitant major illness
4. Severe impairment in pulmonary function
5. End stage honeycomb on radiology
31. TREATMENT
•ATS recommendation (2000):
Prednisone + Azathioprine or Cyclophosphamide
•Consensus recommendation (2008):
Prednisone + Azathioprine + N-acetylcysteine
There is still no effective therapy for IPF
32. American Thoracic Society
Consensus Statement
“. . . Conventional Treatment Options
Treatment options include
• Corticosteroids
• Immunosuppressive / cytotoxic agents (e.g.,
azathioprine, cyclophosphamide)
•Antifibrotic agents (e.g., colchicine or D-
penicillamine) alone or in combination. . .”
33. Conventional therapy
• Corticosteroids alone- ineffective and associated
with significant side effects
• Corticosteroids + immunosuppressives
1. Limited efficacy in slowing the progressive
deterioration in lung function
2. Slightly superior to corticosteroids alone, when
initiated for new onset IPF
3. Low dose prednisone + azathioprine- frequently
used initial treatment in IPF
35. Corticosteroid (prednisone or equivalent)
• 0.5 mg.kg-lean body weight (LBW).day- orally for 4
weeks
• 0.25 mg.kg-day- for 8 weeks
• Taper to 0.125 mg.kg day- or 0.25 mg.kg- on alternate
days
Azathioprine
• 2–3 mg.kg- LBW.day
• Maximum dose 150 mg daily
• Dosing should begin at 25 – 50 mg. day
• Increasing by 25 mg increments every 1 – 2 weeks until
36. ATS recommendation 2000
• The current recommendation for therapy by the
committee is is a combined therapy (corticosteroids
and either azathioprine or cyclophosphamide)
• Regarding the length of therapy, combined therapy
should be continued for at least 6 months in
absence of complications.
• The patients should be evaluated every 3 – 6
months. The therapy should be
continued only in individuals with objective evidence
of continued improvement or stabilization
37. Monitoring of therapy
At 6 month
• If patient worse – therapy stop or changed
• If patient improved or stable - therapy continued at
same doses
At 12 months
• If patient worse - therapy stop or changed
• If patient improved or stable - therapy continued at
same doses
At 18 month or more
• Therapy continued only in individuals with evidence
of continued improvement or stabilization
38. Monitoring clinical course of IPF
Parameters used are
1. Assessment of dyspnea
2. Physiologic testing
A. Lung volumes
B. DLco
C. Resting ABG
D. Cardiopulmonary exercise testing with
measurement of gas exchange
4. HRCT
39. • Favorable (or improved) response to therapy is
defined by - 2 or more of the following , on 2
consecutive visits over a 3- to 6- month period
1. Clinically , symptoms decrease (SOB, cough)
2. Radiology improved Reduction of CXR/ HRCT
findings
3. Physiologic improvement by two or more
parameters
A. ≥ 10% increase in TLC or VC (or at least ≥200-ml
change)
B. ≥15% increase in single breath DLco
40. Favorable response to therapy
• Responses are usually partial and transient.
• Given the limitations of existing studies that are
based on defined quantitative assessment criteria,
10 to 30% of patients with IPF improve when
treated with corticosteroids
• If responses are to occur with corticosteroids,
improvement is usually noted within 3 months
41. Favorable response to therapy
• Even among responders, relapses or progression
of the disease after an initial response suggests
the need for prolonged treatment
• Since it is unlikely that corticosteroids completely
eradicate the disease, prolonged treatment for a
minimum of 1 to 2 yr is reasonable
• Cures (i.e., sustained, complete remissions) are
achieved in few patients.
42. • Failure to respond to therapy- (after 6 month
of therapy)
1. Increase in symptoms
2. Increase in opacities, honeycombing, PHT
3. Deterioration in lung function
– ≥10% decrease in TLC or VC (or ≥200-ml change)
– ≥15% decrease in single breath DLco
– Worsening of O2 saturation (>4 % decrease )
43. Monitoring for Adverse Effects of Treatment
• Cytotoxic therapy is associated with numerous
adverse effects. Cyclophosphamide use can lead to
hemorrhagic cystitis and poses a risk of
cardiotoxicity with high doses.
• Azathioprine is considered to be less toxic than
cyclophosphamide as it does not induce bladder
injury and has less oncogenic potential.
• Azathioprine can, however, induce hepatocellular
injury and rash.
• Both drugs are also associated with GI irritation and
alopecia.
44. • Since the use of either drug can lead to
leukopenia, thrombocytopenia, or bone marrow
suppression, the serial assessment of WBC and
platelet counts is recommended
• if the white blood cell count decreases to below
4,OOO/mm’ and the platelet counts fall below
100,000/mm” then the dose of azathioprine or
cyclo-phosphamide should be stopped or lowered
immediately by 50% of the current dose until these
hematologic abnormalities recover.
45. • Patients taking azathioprine should also undergo
monthly measurements of hepatocellular injury, and
the dose of the medication should be reduced or
stopped if abnormalities greater than three times the
normal level are found.
• Forced diuresis, 2- 8 glasses of water daily, and
monthly monitoring of the urine for red blood cells or
other abnormality is recommended in an attempt to
prevent clinically significant hemorrhagic cystitis in
patients treated with cyclophosphamide.
46. •Acetylcysteine is a precursor to the antioxidant
glutathione,which may be reduced in the lungs of
patients with IPF.
• Oral NAC augments lung glutathione GSH levels on
the respiratory epithelial surface to enhance the
antioxidant defenses and suppresses human lung
fibroblast proliferation
•Inexpensive
N-Acetylcysteine (NAC)
47. N-acetylcysteine (NAC)
Demedts et al, NEJM, 2005.
• 182 patients with UIP
• Prednisone + Azathioprine + High-dose NAC (600mg TID) vs. P/A
• Significant difference in the deterioration of VC and DLCO
at 12
months
Relative difference of 9% and 24% respectively
• Oxidant-antioxidant imbalance?
8/75 (11%)Pred+Aza+
Placebo
7/80 (9%)
NAC+Pred+Aza
Mortality, P =
NS
48. Colchicine
• Although substantive data affirming the efficacy of
colchicine as therapy for IPF are lacking
• Oral colchicine, 0.6 mg once or twice daily, may be
considered as first line therapy or for patients
refractory to corticosteroids, either alone or in
combination with immunosuppressive/cytotoxic
agents
50. Lung transplantation
• With regard to optimal medical management, there
remains no therapy that has been definitively shown to
alter the natural history and disease course in IPF
patients.
• Therefore, if patients appear to be appropriate
candidates, they should be referred for transplantation
consideration and possibly listed, rather than waiting
and assessing them for a response to therapy.
51. • Five‐year survival rates after lung transplantation in
IPF are estimated at 50 to 60% .
• The patients with IPF undergoing lung
transplantation have have favorable long‐term
survival compared with other disease
indications.
• The appropriate patients with IPF should undergo
lung transplantation.
Lung transplantation
52. •IPF is the most common ILD among referrals for
transplant and the 3rd leading indication for lung
transplantation
•Criteria: Evidence of UIP plus any of the following:
o DLCO < 39% predicted
o Decrement in FVC > 10% during 6 months
o Decrease in pulse ox below 88% during 6-
minute walk test
o Honeycombing on HRCT
Lung transplantation
53. Lung transplantation
• Considered for progressive physiologic
deterioration despite therapy and who meet
established criteria
1. Severe functional impairment
2. Oxygen dependency
3. Deteriorating course
4. <60 year age
54. Surgical Therapy for IPF
• Lung transplant is the only cure For end-stage
IPF
• lung transplantation Improves quality and
quantity of life
– 1 yr survival is 85%
– 5 yr survival is 60%
• Single lung transplantation is currently the
preferred surgical operation..
55. • Refer early for evaluation since disease can
progress rapidly to death!!
• Owing to limited donor availability, early listing is
important, as the waiting time for procuring a
suitable donor organ may exceed 2 yr.
• Unfortunately, patients with rapidly progressive or
severe IPF may die while awaiting transplantation.
Lung transplantation
57. • Idiopathic pulmonary fibrosis (IPF) is a
progressive, usually fatal disease
• Lung transplantation is the definitive treatment
for a variety of life-threatening lung diseases
including emphysema, idiopathic pulmonary
fibrosis (IPF), cystic fibrosis and other deadly
lung diseases.
59. • There is no known effective pharmacological
intervention to date for patients with IPF
• There is no known medical therapy proven to have
enhanced survival and improved outcomes for
patients with IPF
• Pharmacological treatment should be limited to the
minority of patients who are willing to accept
possible adverse consequences even if expected
benefits are small and this should be discussed
with the patient
60. • All patients should be monitored for disease
progression at 4–6 months or sooner as clinically
indicated and identify potential complications.
• Selected patients manifesting disease progression
during follow-up must undergo evaluation for
consideration of lung transplantation.
61. Non-Pharmacologic Therapy
– Oxygen therapy
– Pulmonary rehabilitation
– Immunizations
– Patient education
Do not under-estimate the importance
of non-pharmacologic therapy!!!
62.
63. LTOT
ATS/ERS/JRS/ALAT Recommendation
• The patients with IPF and clinically significant
resting hypoxemia should be treated with
long‐term oxygen therapy .
• Patients with hypoxemia (PaO2 < 55 mmHg or
oxygen saturation as measured using pulse
oximetry [SpO2] < 88%) at rest or with exercise
should be prescribed oxygen therapy to maintain a
saturation of at least 90% at rest, with sleep, and
with exertion.
64. • Supplemental O2 during exercise may markedly
improve exercise-induced hypoxemia and improve
exercise performance
• Higher flow rates than that frequently used in
chronic obstructive pulmonary disease may be
required
•End-stage disease is characterized by severe PH with
cor pulmonale that does not improve with oxygen
65. • Seasonal influenza Vaccine and
pneumococcal Vaccine should be
encouraged in all patients with idiopathic
pulmonary fibrosis
66.
67. In spite of potent anti-inflammatory therapy:
• progressive
• irreversible
• lethal disease
Idiopathic Pulmonary Fibrosis
68. Clinical Course – IPF
• Gradual deterioration despite treatment with
occasional periods of rapid clinical deterioration
• Median survival after diagnosis (as per Sharma
and Maycher): 2.5-3.5 years
• 5 year survival rate: 10%-50%
69. Clinical phenotypes of IPF
The heterogeneous natural history pattern in IPF
• The disease has a long (months to years)
asymptomatic period.
• Most patients follow a relatively slow clinical and
functional decline (slowly progressive) after
diagnosis.
• About 10% of these patients present with episodes of
acute clinical deterioration (acute exacerbations) that
precede and possibly initiate the terminal phase of
their disease
70. • A few patients have a short duration of illness ,
have an accelerated decline, with a rapidly
progressive clinical course.
• Some patients remain stable
• Heavy smokers might develop pulmonary fibrosis
combined with emphysema, with shorter survival
compared with patients with IPF alone.
Clinical phenotypes of IPF
75. Indicators of longer survival
among IPF patients
1) Younger age (< 50 yr)
2) Female sex
3) Shorter symptomatic period (1 yr) with less
dyspnoea, relatively preserved lung function
4) Presence of ground glass and reticular opacities on
HRCT
5) Increased proportion of lymphocytes (20 – 25 %) in
BAL
6) A beneficial response or stable disease ( 3 – 6
76. Risk Factors for Progressive Disease
at time of Presentation
o Age: >50 years
o Gender: male
o Dyspnea: moderate to severe
o History of cigarette smoking in absence of
emphysema
o Lung function: moderate to severe loss (especially
gas exchange with exercise)
o HRCT scan: extent of honeycomb changes
o 6 MWT: desaturation below 88%
o Pathology: more fibroblastic foci
o Response to anti-inflammatory therapy: none
77. Histopathologic Subsets in IIP
A Retrospective Analysis of Survival
Bjoraker JA, et al. Am J Respir Crit Care Med.1998;157:199-203.
Years
0 2 4 6 8 10 12 14 16 18
0
20
40
60
80
100
UIP (n=63)
(P<0.001)
NSIP
(n=14)
Others
(n=20)
Alive(%)
78. Variability in the clinical
course of IPF
From Flaherty et al, Thorax, 2003
LUNG FUNCTION /
SYMPTOMS
TIME
NSIP, CTD-ILD
Sarcoidosis
Diagnosis = prognosis
IPF
82. Cause of Death
IPF
[N=543]
1-7 year FU
60% Died
[N=326]
Respiratory
failure
39%
Lung
cancer
10%
Pulmonary
embolism
3%
Pulmonary
infection
3%
Cardiovascular
disease
27%
Other
18%
Panos RJ, Mortenson RL, Niccoli SA, King TE Jr. Clinical deterioration in patients with idiopathic
pulmonary fibrosis: causes and assessment, 88:396-404, 1990.
83.
84. Causes of acute deterioration in
patients with previously stable UIP
• Accelerated decline of UIP , biopsy often shows
diffuse alveolar damage
• Pneumothorax , collapsed lung is often relatively
resistant to re-expansion, making treatment difficult
• Infection (especially if immunocompromised), e.g.
PCP
• Pulmonary embolism
• Acute deterioration may also occur post-operatively
following major surgery, e.g. cardiac or orthopaedic
85. • Acute exacerbations may occur in absence of
infection
1. Fulminant decline in oxygenation
2. Rapidly progressive respiratory failure
Acute Exacerbation of IPF
86. Progression of IPF
Acute Exacerbation Vs Slow Decline
50%
Years
Respiratory
function/symptoms
1 2 3 4
FVC
Traditional view of UIP/IPF progression
FVC=forced vital capacity
88. Acute Exacerbation of IPF
Proposed diagnostic criteria
1. Previous or concurrent diagnosis of IPF
2. Unexplained worsening or development of
dyspnea within 30 days or less
3. New or increased Bilateral ground glass and/or
consolidation superimposed on a background
reticular or honeycomb pattern consistent with
“UIP pattern” on HRCT
89. Proposed diagnostic criteria
4. No microbiological evidence of respiratory
infection by endotracheal aspirate or BAL
5. Exclusion of other known causes of acute
worsening e.g. LVF, PE
• Patients with idiopathic clinical worsening who fail
to meet all five criteria due to missing data should
be termed “suspected acute exacerbations.”
Acute Exacerbation of IPF
90. • The prognosis for acute exacerbation-IPF
requiring admission to I.C.U for respiratory failure
is very poor with reported mortality rates of 60-
100%
• Intiation of NIV is not an effective means to avoid
mechanical ventilation in patients with pulmonary
fibrosis
• NIV may delay the need for intubation but it would
not improve patient's poor prognosis .
91.
92.
93.
94. • BAL is the diagnostic procedure to exclude
opportunistic infection in an acute
exacerbation of IPF
• Routine sputum evaluation for Gm stain &
culture not sensitive enough to detect
opportunistic infectious organism
95. • As a first step, we test for the presence of d-dimer
and clinical probability of pulmonary embolism.
• If the test is positive, a specific computed
tomography (CT) scan is performed.
• The CT scan is an accurate modality with which to
detect pulmonary embolism; moreover, images can
be compared with previous CT scans to evaluate
reticular shadowing, honeycombing, and ground-
glass appearances.
2008 The American Thoracic Society
96. • Echocardiography is performed to rule out left heart
failure , at the same time, (increasing) pulmonary
hypertension can be ruled out.
• If diffusion capacity is greater than 30%, and if
hypoxemia can be corrected to a Po2 of 75 mm Hg
with supplemental oxygen , then bronchoalveolar
lavage (BAL) is performed to rule out infection
(bacterial, viral, opportunistic infections, and fungi).
2008 The American Thoracic Society
97. • BAL must be performed soon after admission, and
broad-spectrum antibiotics are started immediately
after bronchoscopy.
• We usually start broad-spectrum antibiotics (such as
piperacillin-tazobactam or third-generation
cephalosporins intravenously). However, atypical bacilli
(such as Legionella and Mycoplasma) must be covered
by adding quinolones.
• As most of these patients are immunocompromised,
Pneumocystis jiroveci pneumonia needs to be covered
empirically with sulphametoxazole.
98. • If infection cannot be proven, corticosteroids are
added to the treatment in a dose of 500–1,000 mg
for 3 consecutive days.
• Antiviral agents should be considered when
herpesvirus or cytomegalovirus (CMV) is found in
BAL, and antifungal agents should be initiated
especially when Aspergillus is found in an
immunocompromised patient or if the Aspergillus
antigen test in BAL is clearly positive
99. • If there is no effect from these therapies, and in the
absence of generalized infection, lung
transplantation should be considered as a treatment
for acute exacerbation in IPF. It is the only treatment
that improves survival in patients with interstitial lung
disease .
• The patient can be put on the high-urgency list
• This is another strong reason to refer patients with
IPF in an early stage to a transplant center for
transplant evaluation
100. • Corticosteroids are an appropriate treatment
option for acute exacerbation.
• Mechanical ventilation is not recommended in the
majority of patients with respiratory failure due to
the progression of their disease.
101. Mechanical Ventilation
• There is high hospital mortality rate in mechanical
ventilation patients with IPF and respiratory failure.
• The majority of patients with respiratory failure due
to IPF should not receive mechanical ventilation, b
ut mechanical ventilation may be a reasonable
intervention in a minority
102. • Symptom control (palliative care) focuses on
reducing symptoms (e.g. cough, dyspnoea)
providing comfort to patients, rather than
treating disease.
103.
104. Diseases That Mimic IPF
• IPF is often misdiagnosed, or diagnosed at an
advanced stage of the disease
• Symptoms of other diseases that mimic IPF:
– COPD
– CHF
– Connective tissue diseases (eg, RA, Sjögren’s,
SLE)
– Other lung diseases (asbestosis, hypersensitivity
pneumonitis)ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000
ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165:277-304.
105. KEY POINTS
Wide spectrum of ILD
oIPF is predominantly imbalance in fibrosing repair
system
oOther ILDs are predominantly inflammatory
oAim of therapy in IPF is slow down future
progression
oAim of therapy in other ILD is to control
inflammation
oPrognosis of IPF remains poor IPF , Worst
•Realisation that IPF is not an inflammatory
disease
106. 1. Disease course is variable
2. Median survival after diagnosis is less than 3 years
3. 5-year survival rate is 30-50%
4. 40% IPF patients die of respiratory failure
5. Others die of complicating illnesses, mainly CAD
and infections
6. Incidence of bronchogenic carcinoma is increased
in patients with IPF
KEY POINTS
107. A quick recap……..
• Approximately 5 million people worldwide suffer from
IPF
• Tragically, IPF frequently leads to death, with an
average life expectancy from the time of diagnosis of
about 3 years.
• While medical research is ongoing and many
treatments have been tried, there is still no proven
effective treatment for IPF.
• Supportive treatments such as pulmonary
rehabilitation and the use of oxygen are commonly
used.
• A lung transplant is the only therapy which has been
108.
109.
110.
111. • UIP is NOT a clinical entity but a specific radiological
and pathological pattern.
• IPF (Idiopathic Pulmonary Fibrosis) is its clinical
counterpart; defined as a specific form of chronic,
progressive fibrosing interstitial pneumonia of
unknown cause, occurring primarily in older adults,
limited to the lungs, and associated with the
histopathologic and/or radiologic pattern of UIP.
(UIP)2011
112. • The major and minor criteria established by the
previous panel of IPF experts and proposed in the
2000 ATS/ERS IPF statement are no longer
required.
• Patients may have normal pulmonary function tests
and yet have IPF.
• In the appropriate clinical setting, TBX or BAL
cellular analysis are no longer required to make a
diagnosis of IPF in patients manifesting HRCT
ATS/ERS/JRS/ALAT International IPF
guidelines 2011
113. ATS/ERS/JRS/ALAT International IPF
guidelines 2011
• Diagnosis
1. Exclusion of other known causes of ILD
Environmental exposure, CTD, Drug toxicity
2. UIP pattern on HRCT (Pt not subjected to Lung
Biopsy)
3. Specific combinations of HRCT and Surgical Lung Bi
opsy in Patients subjected to Lung Biopsy
Note: Multi Disciplinary Discussion (Pulmonary, Radiology
& Pathology) has been shown to improve accuracy of
114. Approach to the Diagnosis of ILD
Clinical
• History
• Physical
• Laboratory
• PFTs
Primary care
physicians
Pulmonologists Radiologists Pathologists
Multidimensional and multidisciplinary
Radiology
o Chest X-ray
o HRCT
Pathology
o Surgical lung
biopsy
115. • In the appropriate clinical setting (The typical
clinical setting for IPF is the adult(commonly a
male of 60 yrs of age) without collagen vascular
disease and exclusion of causes known to cause
and/or associated with ILD.), the presence of the
criteria for the pattern of UIP on HRCT images is
sufficient to make an accurate diagnosis of IPF.
116. 1. Subpleural, basal predominance
2. Reticular abnormality
3. Honeycombing with or without traction
bronchiectasis
• Absence of features inconsistent with UIP pattern
A. Upper/mid lung predominance
B. Extensive ground glass opacities
C. Micronodules/cysts/consolidations.
UIP PATTERN/HRCT FEATURES 2011
117. • In patients demonstrating features that meet the
criteria for ‘‘possible UIP’’ and/or ‘‘inconsistent with
UIP’’ patterns on HRCT images, the
histopathological features of the ‘‘ surgical lung
biopsy ’’ SLB are required to make an accurate
diagnosis of IPF.
121. • “The committee did not find sufficient evidence to
support the use of any specific pharmacologic
therapy for patients with IPF.”
An Official ATS/ERS/JRS/ALAT Statement 2011:
Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines
for Diagnosis and Management
PHARMACOLOGICAL THERAPIES
135. • Cryptogenic organising pneumonitis (COP) or
bronchiolitis obliterans organising pneumonia is an
uncommon condition characterised by the occurrence
of intra-alveolar buds of organising fibrosis with
obliteration of bronchioles on lung biopsy.
• It particularly occurs in association with some drugs
(e.g. amiodarone, sulfasalazine, gold), connective
tissue diseases (e.g. rheumatoid disease) or ulcerative
colitis,but often no cause is identifiable.
Cryptogenic Organizing Pneumonia
136. Cryptogenic Organizing Pneumonia
(COP)
• M=F
• 4th-5th decades
• More common in non-smokers
(2:1)
• Acute to subacute onset
• Widespread crackles but no
clubbing
• Ground glass opacities on HRCT
• Restrictive impairment and mild
resting hypoxemia
• Granulation tissue in airspaces-
137. • Clinically,patients often have cough, malaise, fever,
dyspnoea with chest X-ray infiltrates and elevated
ESR. Often the patient is thought to have infective
pneumonia
• No pathogen is identified and the patient fails to
respond to antibiotics.
• A range of chest X-ray abnormalities occur including
Recurrent and fleeting (migratory)
shadows,localised alveolar infiltrates and diffuse
reticular shadowing.
139. Treatment:
• Characteristically, there is a dramatic response to
corticosteroids , results in clinical recovery in two-
thirds of the patients , although relapse may occur
as the dose is reduced.
• Corticosteroids, usually at least 6 months
• Relapse is frequent –increase steroids
144. DIP
• Clinical classification=pathology
• DISEASE OF SMOKERS!! >90% are smokers
• 4th-5th decades
• Subacute cough and dyspnea
• 50% with digital clubbing
• CT shows diffuse GG ground glass infiltrates
• More likely affects basilar and sub-pleural areas
• Fibrosis rarely seen on CXR or CT
• Smoking cessation is the primary therapy
• Corticosteroid responsive
145. RB-ILD
• SMOKING DISEASE!!
• Similar to DIP, but pathology slightly different, in
peribronchiolar distribution
• VATS required for diagnosis because disease is
centered around terminal bronchioles
• Smoking cessation +/- steroids
146. Key points
• RB-ILD & DIP , both seen in current or former
smokers.
• Typical presentation 4th to 5th decade.
• Sub acute (weeks-months)
• DIP is middle to lower lung pre dominant/RB-ILD is
upper lung predominant.
• Smoking cessation is important in the resolution of
these lesions.
• Steroid responsive.
• Prognosis is good with 10 year survival of >70%.
147. • RB-ILD (respiratory bronchiolitis interstitial lung
disease) & DIP (desquamative interstitial
pneumonia) are strongly associated with smoking
and respond well to smoking cessation
• Smoking cessation does not change course of
disease in IPF
148. Pulmonary Langerhans Cell
Histiocytosis PLCH
• Is a smoking-related diffuse lung disease that
typically affects men 20–40 years of age.
• Presenting symptoms often include cough, dyspnea,
chest pain, weight loss, and fever.
• High-resolution chest CT scan reveals upper-zone
predominant nodular opacities and thin-walled cysts.
• Pneumothorax occurs in 25% of pts
• Smoking cessation is the key therapeutic
intervention.
149. Langerhan cell histiocytosis.
This 50-year-old man had a
30 pack-year history of
cigarette smoking.
A: PA chest radiograph
shows hyperinflation of the
lungs and fine bilateral
reticular ILD.
B: CT scan shows multiple
cysts (solid arrow) and
nodules (dashed arrow).
150.
151. A quick recap……..
1. IIPs are a spectrum of interstitial lung diseases for
which no clear etiology can be determined
2. The primary responsibility, when confronted with a
patient with IIP is to differentiate whether they have
a case of UIP/IPF or a case of something else
3. Although different types of IIP can present similarly
in the clinic, radiographic and pathologic findings
can clarify the diagnosis
4. Whether it be smoking cessation, or corticosteroid
therapy, all types of IIP can be effectively treated
and even cured, except UIP/IPF
152.
153. Doing the right thing sometimes is the
hardest thing to do.
