1

Infiltrative and Fibrotic Lung

Diseases

2
Diffuse Parenchymal
Lung Disease
Nonmalignant
infiltration of the
gas-exchanging
components of the
lungs. 3
DPLD is characterized
clinically by dyspnea
on exertion, cough,
and radiographic
findings of diffuse
pulmonary infiltrates.
4
 DPLD is categorized as
1-idiopathic interstitial pneumonia (IIP),

2-Interstitial pneumonitis associated with a

systemic rheumatologic disorder,

3-Interstitial pneumonitis associated with

environmental exposure, or

4-sarcoidosis

(British Thoracic Society Guidelines for DPLD).

5
When a DPLD is identified, a thorough, directed
history is needed to make a specific diagnosis.

Details about the patient’s pre-existing chronic

medical conditions, immune states, and
medication history should be obtained.

An in-depth occupational and environmental

exposure history should focus on the patient’s
exposure to asbestos, silica, beryllium, and
organic dust.

Review of systems should include questions

about collagen vascular diseases.
6
HRCT provides detailed
imaging of the lung
parenchyma, aids in
the differential
diagnosis, and locates
regions of interest for
biopsy 7
 Idiopathic Interstitial Pneumonia
Patients with IIP present with chronic,
progressive exertional dyspnea and
nonproductive cough.
Signs of right heart failure and respiratory
insufficiency appear late.
Some history and physical examination
findings differentiate IIP.
Clubbing is common in IPF

8
Pulmonary function studies of
IIP generally reveal
restrictive physiology,
although mixed airways
obstruction and parenchyma
restriction may occur in
current or former smokers

9
Laboratory studies generally are
normal, although indicators
of systemic inflammation may be
mildly elevated, and low levels
of rheumatoid factor or
antinuclear antibodies
are found in 15% of cases.

10
11
 Pulmonary
Manifestations of
Collagen
Vascular Diseases
12
 Rheumatoid Arthritis
Pulmonary manifestations in rheumatoid
arthritis range from
common (pleural effusions)

To Rare (bronchiolitis obliterans).

13
Rheumatoid pleuritis may present
with fever and chest pain or may be
asymptomatic.

Effusions are unilateral in more than

75% of cases.

The pleural fluid is characteristically

exudative with a low glucose level
(<40 mg/dL in 70%). 14
 The pleural disease
rarely requires specific
therapy, and effusions
generally resolve with
treatment of
inflammatory arthritis
15
Restrictive lung disease occasionally
may develop as a sequel of pleural
fibrosis.

Necrobiotic pulmonary nodules

frequently develop in patients with
rheumatoid arthritis and are
histological identical to rheumatoid
subcutaneous nodules.

16
In rheumatoid arthritis, the
airway may be involved
anywhere from the larynx to the
terminal bronchioles.

Cricoarytenoid joint involvement

is associated with symptoms of
sore throat or inspiratory stridor
with exertion or during sleep 17
Rheumatoid arthritis is
associated with
interstitial lung disease
that may be difficult to
differentiate from
disease modifying drug
toxicity. 18
 Systemic Lupus Erythematosus

Numerous pulmonary syndromes have

been described in patients with (SLE).

Pleurisy, with or without pleural

effusions, is the most common form of
lupus lung disease; pleuropulmonary
disease has a 70% prevalence in SLE.

19
The effusions are
inflammatory exudates
with normal pH and
glucose levels.
Several forms of acute
and chronic interstitial
lung disease can
develop in SLE
20
Acute lupus pneumonitis most often develops in
patients with established SLE.

Patients present with tachypnea, dyspnea, fever,

cough, and, occasionally, hemoptysis.

Chest radiography reveals prominent alveolar

consolidation.

The critical differential diagnostic considerations are

typical or atypical infectious agents.

Many patients with acute lupus pneumonitis

manifest persistent pulmonary dysfunction after
resolution of the acute illness. 21
Chronic interstitial lung disease is uncommon and
may develop independently of acute pneumonitis
or as its sequel.

Chronic interstitial lung disease is associated with

pleuritis in about 40% of cases, which clinically
distinguishes SLE-associated interstitial disease
from IIP.

Chronic interstitial lung disease is more common in

the subset of patients with SLE who display
overlapping features of systemic sclerosis
(scleroderma), such as edema of the hands and
abnormalities of the nail fold capillaries.

22
A syndrome of reversible hypoxemia with
normal chest imaging studies has
been shown in hospitalized patients with SLE.

This disorder is attributed to leukocyte

aggregation in the pulmonary capillaries in
acutely ill patients with SLE.

These cell aggregates lead to substantial

ventilation–perfusion mismatching.

23
The sudden onset of respiratory insufficiency and
hemoptysis with progressive pulmonary infiltrates
in a patient with active SLE should suggest
pulmonary hemorrhage.

Pulmonary hemorrhage may complicate acute lupus

pneumonitis or can occur as an independent
small-vessel vacuities

An unexpected elevation in DLCO, blood visualized

at bronchoscope, or
hemosiderin-laden macrophages retrieved by
bronchoalveolar lavage support the diagnosis of
pulmonary hemorrhage.

24
The shrinking lung syndrome of SLE
may mimic interstitial lung disease.
Patients present with exertional
dyspnea, and lower lobe atelectasis is
shown on chest X ray.
The shrinking lung syndrome is caused
by diaphragmatic dysfunction and
respiratory muscle weakness that
produce a restrictive ventilatory
defect manifested by low lung
volumes and elevated diaphragms.
25
 Systemic Sclerosis
Pulmonary disease occurs in 70% to 85%
of patients with systemic sclerosis.
Primary interstitial lung disease frequently
occurs and is clinically or
radiographically indistinguishable from
IIP

26
Patients with esophageal dysmotility may
present with recurrent aspiration and
resulting lower lobe lung disease, which is
easily confused with primary interstitial lung
disease.
Pulmonary hypertension is the cause of death
in up to 60% of patients with diffuse or
limited systemic sclerosis.
This may occur in the absence of interstitial
lung disease and has been associated with
peripheral Raynaud’s phenomenon,
suggesting an abnormality in pulmonary
vascular reactivity as a primary event.
27
Systemic sclerosis has an
increased (fourfold to
seventeenfold) risk for lung
cancer that is independent of
cigarette smoking, but can be
related to the presence of
pulmonary fibrosis.

28
 Inflammatory Myopathies
Pulmonary disease in IM commonly
occurs through four processes:
1) Aspiration due to bulbar muscle
dysfunction;
2) ventilatory insufficiency from
respiratory muscle weakness;
3) infection from immunomodulatory
drug therapy;and
4) interstitial lung disease.
29
In one third of cases, the interstitial lung
disease of PM/DM antedates muscle
involvement or occurs in patients with
only minimal myopathy.
Antibodies to the enzyme histadyl-tRNA-
synthetase (anti-Jo-1) are detectable in
patients with IM and concomitant
interstitial lung disease.

30
Hypersensitivity Pneumonitis

Hypersensitivity pneumonitis
(HP), also known as
extrinsic allergic alveolitis,
is a specific allergic reaction
in the lower respiratory tract
to inhaled low-molecular
weight antigens 31
The clinical presentation is recurrent acute
episodes of
fever, cough, and dyspnea that start 4 to 6
hours after antigen exposure, with
spontaneous resolution 24 to 48 hours after
antigen avoidance.
Physical examination may reveal crackles in
the lower lobes and, rarely, wheezing.
Chest CT scans shows bilateral patchy
consolidation, a ground-glass appearance, or
diffuse nodular infiltrates.

32
Pulmonary function testing
shows a restrictive defect with
impaired gas transfer.
Laboratory studies show a
leukocytosis with neutrophilia.

33
A diagnosis of HP is suggested by:
1) a compatible clinical presentation
with a history of exposure to a candidate
antigen;
2) demonstration of a specific
immune response to that antigen; and

A specific immune response may be confirmed

by the detection of circulating precipitating
anti-bodies to the antigen of interest.

34
However, the majority of exposed persons
develop precipitating antibodies or indices of
cell-mediated immune responses to
antigens with only a minority manifesting
clinical signs of HP.

Therefore, the presence of the circulating

antibody is necessary but not sufficient
evidence to make the diagnosis of HP in the
absence of an appropriate clinical scenario.

35
Therapy primarily is aimed at
antigen avoidance when
possible.

In acute HP, corticosteroids

reduce the duration of
presenting symptoms.

36
 Sarcoidosis
Sarcoidosis is an idiopathic systemic illness
characterized by tissue infiltration
with well-formed noncaseating granulomata.
More than 90% of patients have pulmonary
involvement.
Other organs frequently involved include the
skin, bone, eye, liver, and spleen.
Cardiac and central nervous system
involvement are uncommon, but potentially
lethal manifestations of sarcoidosis.

37
 Cough and dyspnea
are common
presenting
symptoms due to the
frequency
of pulmonary disease.
38
Pulmonary function
tests may show
restriction,
obstruction, a mixed
pattern, or may be
normal.
39
Many patients without
symptoms are diagnosed
with sarcoidosis from routine
chest radiography.
Hypercalcemia, hypercalciuria,
or both, show aberrant
calcium metabolism due
to 1,25-dihydroxy vitamin D3
production by granulomas.
40
Patients with Lofgren’s syndrome have
symptoms of fever, erythema nodosa, and
bilateral hilar lymphadenopathy.

Uveoparotid fever is another presentation

of sarcoidosis with fever and parotid
swelling often with facial nerve palsy.

41
Tissue biopsy shows
granulomas without
organisms.

Transbronchial lung biopsy

will show granulomas in
more than 50% of cases with
stage I sarcoidosis.
42
Higher yields are obtained with more
advanced
radiographic disease.

Patients with radiographic stage I disease and

no systemic symptoms have a spontaneous
remission rate greater than 70%.

Similarly, patients with Lِfgren’s syndrome

have a benign course and may be managed
with observation alone

43
Most patients experience a
durable spontaneous remission.

Therefore, treatment is generally

reserved for those with persistent
respiratory symptoms, evidence of
radiographic or physiologic
progressive lung disease, or
symptomatic extra pulmonary
sarcoidosis.
44
The cornerstone of therapy is
daily corticosteroids for 6 to
12 months.
Although such treatment
reduces symptoms and
improves chest radiograph
findings and pulmonary
function test results, it is of
unproven benefit in altering
long-term outcome
45
Drugs which can cause lung
fibrosis.
Busulphan, bleomycin and
methotrexate (cytotoxic agents),
Amiodarone, azathioprine,
antibiotics (nitrofurantoin,
sulfasalazine), penicillamine,
gold, phenytoin.
46
 Lofgren's syndrome is a self
limiting subset of sarcoidosis.
It consists of the triad of fever,
bilateral hilar lymphadenopathy
and erythema nodosum.
Therefore, the prognosis is good
and steroids are not required.
47
A 60 year old man presents with haemoptysis of
small amounts. he does not have pleuritic
chest pains and has normal oxygen
saturations. In considering the potential
diagnosis, haemoptysis can occur with which
one of the following diagnosis?
A. Pulmonary fibrosis
B. Melanoma
C. Goitre
D. Thymoma
E. Aspergilloma

48
Answer: e) Aspergilloma.
Hemoptysis can be caused by
pulmonary embolus,
tuberculous infection,
aspergilloma,
bronchial carcinoma,
Goodpasture's syndrome and
Wegener's granulomatosis.
49
A 35 year old lady with systemic sclerosis has
breathlesness on exertion. She has bilateral
basal crepitations in the chests and
corresponding interstitial shadowing on the
CXR. Which is likely to be found on her lung
function tests?
A. P02 of 11 desaturating to 10 on exertion
B. FEV1 to FVC ratio of 65%
C. Diffusion capacity (DLCO) of 17 (predicted 23)
D. Increased residual volume
E. FEV1 of 5 L

50
Answer: c) diffusion capacity
(DLCO) of 17 (predicted 23).
A decrease in diffusion
capacity (DLCO) indicates
interstitial lung disease, which
is likely in a patient with basal
crepitations and a
predisposing connective
tissue disease
51
A 60 year old miner has been in the occupation
for 20 years. He presents with a cough and
breathlessness. Chest XR shows diffuse
interstitial shadowing. A sputum sample is
positive for acid fast bacilli.
Which of the following dusts is most likely to
have predisposed the patient to
tuberculosis?
A. Beryllium
B. Cadmium
C. Coal
D. Silica
E. House dust
52
Answer: d) silica. Slate workers,
stonemasons and miners are exposed to
silica dust. Silicosis impairs
macrophage function, and in particular,
predisposes to TB infection.

53
An 13 year old male has recent onset
breathlessness. He wheezes
particularly during the summer when
the pollen count is high.
Which of these tests would help to
confirm the diagnosis?
A. Trial of inhaled corticosteroids
B. Trial of anticholinergics
C. Serial peak flow measurements
D. Lung function tests
E. Chest X ray
54
Answer: c) serial peak flow
measurements.
The best diagnostic test for
asthma would be
demonstration of variable
airways obstruction with
serial peak flow
measurements.
55
A 32 year old man has a history of dry eyes and
mouth. His blood tests reveal a positive ANA,
Ro and La extra nuclear antigens are also
positive. Which of the following is he
predisposed to developing?
A. SLE
B. Small cell carcinoma of the lung
C. Lymphoma of the lung
D. Restrictive lung disease
E. Pulmonary eosinophilia
56
Answer: c) lymphoma of •
the lung.
The patient has Sjogren's •
syndrome.
This predisposes to •
lymphoma of the lung.
57
The relatives of a gentleman who is deceased
has pursued compensation for him dying
due to occupational lung disease. Results
of lung histology showed non-caseating
granulomata and interstitial fibrosis.
Which one of the following is likely?
A. Tuberculosis
B. Berylliosis
C. Silicosis
D. Asbestosis
E. Isocyanate exposure
58
Answer: b) berylliosis.
There is are sarcoidosis
like changes on the
histology - non
caseating granuloma
and interstitial fibrosis in
berylliosis
59
A 50 year old Afro-Caribbean man has a
cough. He also has generalised arthralgia.
There is an eruption of tender red areas on his
shin. Which of the following is
important to determine whether this
condition is self limiting?
A. Hilar lymphadenopathy and eosinophilia
B. Response to Kveim test
C. Hilar lymphadenopathy and erythema
nodosum
D. Serum calcium levels
E. Serum ACE levels
60
Answer: c) hilar lymphadenopathy
and erythema nodosum.
• The sarcoid subset of Lofgren's
syndrome as described here, is
self limiting. It consists of the
triad of fever, bilateral hilar
lymphadenopathy and erythema
nodosum. Therefore, the
prognosis is good and steroids
are not required
61
Bilateral hilar lymphadenopathy

62
An 18 year old girl with severe difficulty in
breathing to A+E. She has had a history of
asthma with two previous ITU admissions.
Her peak flow is currently 100.
She is unable to say more than a few words.
She was given nebulised salbutamol and iv
hydrocortisone 200mg by the paramedics.
Which treatment should she now have?
A. Further iv hydrocortisone 200 mg
B. Iv magnesium 2g
C. Intubation and ventilation
D. Iv salbutamol
E. Iv ipatropium

63
Answer: b) iv magnesium 2g.
According to BTS guidelines, single dose
Magnesium of 2g IV should be instituted
in cases of acute severe asthma
following administration of conventional
therapy.
Aminophylline is no longer recommended
as the second line treatment in most
trusts.

64
A 60 year man has been a long standing
smoker of 20 a day. He has a cough and
difficulty in breathing.
Arterial blood gases show:
pH 7.25
P02 16 kPa
PCO2 8.2 kPa
What should be the next management step?
A. Non invasive ventilation
B. Intravenous antibiotics
C. Intubation and ventilation
D. Reduce inspired oxygen concentration
E. Increase oxygen concentration
65
Answer: d) reduce inspired oxygen
concentration.
This lady with COPD has blood gases
showing type II respiratory failure with
acidosis. Her respiratory drive is
suppressed by too much oxygen
inspired, and hence reduction to a
lower concentration (e.g. 2 litres) to
maintain a PO2 above 8.5kPa is
recommended.
66
A 30 year old man has breathlessness and
wheeze.
Investigations show:
Neutrophils 6 (2-7.5)
Lymphocytes 3 (1.3-3.5)
Eosinophils 19 (0.04-0.44)
CXR shows reticulonodular shadowing
What is the likely diagnosis?
A. Tuberculosis
B. Loeffler's syndrome
C. Sarcoidosis
D. Pulmonary embolism
E. Wegener's granulomatosis
67
Answer: b) Loeffler's syndrome.
Hypereosinophilic syndrome is a rare
condition where there is an idiopathic
eosinophil count of > 15 x 10^9/dl.
It is associated with Loeffler's syndrome which
is a transient pulmonary reaction with
nodular or reticular shadowing (diffuse, fan
shaped shadowing) on chest radiology and
eosinophilia.
Hypereosinophilic syndrome generally affects
young men ages 20-50.
Thrombotic tendency, neurological
involvement and restrictive cardiomyopathy
occur. There is response to steroids.
68
A 50 year old man presents with a 3 month history of cough
and breathlessness. He was apyrexial on admission.
His blood tests show: ESR 60 mm/hr
urea 7 μmol/l
creatinine 100 μmol/l
sodium 137 mmol/l
potassium 4.1 mmol/l
corrected calcium 2.75 (2.2-2.7) mmol/l
phosphate 0.82 (0.8-1.4)mmol/l
Chest X ray shows bilateral hilar lymphadenopathy and
eggshell calcification.
What is the likely diagnosis?
A. Extrinsic allergic alveolitis
B. tuberculosis
C. Sarcoidosis
D. Allergic bronchopulmonary aspergillosis
E. Leiomyoma

69
Answer: c) sarcoidosis.
Sarcoidosis can cause many
changes on the CXR.
Among these are unilateral or
bilateral hilar lymphadenopathy,
diffuse parenchymal changes,
eggshell calcification, pleural
effusions and nodules.

70
A 65 year old farmer has breathlessness.
During investigation he was found to
have raised serum precipitins to
micropolyspora faeni.
What is the diagnosis?
A. Tuberculosis
B. Wegener's granulomatosis
C. Churg Strauss syndrome
D. Extrinsic allergic alveolitis
E. Pulmonary eosinophilia
71
Answer: d) extrinsic allergic alveolitis.
Extrinsic allergic alveolitis causes a
neutrophilia due to cell mediation but
eosinophil count is normal.
Bronchoalveolar lavage shows lymphocytes
and mast cells.
EAA is a delayed hypersensitivity reaction
which may be immune complex (III) mediated
or cell mediated (type IV) in chronic disease.
Precipitins to micropolyspora faeni in farmer’s
lung or aspergillus are seen.
Upper zone fibrosis causes crackles which can
be heard. Symptoms are typically of
breathlessness but not wheeze.
72
EAA causing upper zone fibrosis

73
A 40 year old man has an X-ray
suggestive of fibrotic changes in the
upper zone.
Which one of the following is likely
to be a cause?
A. Systemic lupus erythematosus
B. Streptococcal pneumonia
C. COPD
D. Asthma
E. Extrinsic allergic alveolitis
74
Answer: e) extrinsic allergic alveolitis .
The causes of upper zone fibrosis in the lung
are:
TB
Extrinsic allergic alveolitis
ankylosing spondylitis
radiation
sarcoidosis
silicosis
The causes of lower zone fibrosis are:
Asbestosis
bronchiectasis
cryptogenic fibrosing alveolitis
drugs 75
A 55 year old COPD patient is admitted with vomiting and
is very unwell. She is on salbutamol home nebulisers
and theophylline regularly.
Following assessment, she is suspected of having
theophylline poisoning.
Which of the following statements is true
regarding theophylline poisoning?
A. Hyperkalaemia is a feature
B. Activated charcoal is of little value
C. Ipecacuanha is the method of choice to reduce gut
absorption
D. Convulsions are a recognised feature
E. Assisted elimination should be avoided

76
Answer: d) convulsions are a recognised
feature.
Theophylline poisoning results in profound
Hypokalaemia, SVT and VT, vomiting, agitation
and convulsions.
Activated charcoal is the method of choice for
elimination.
Diazepam may be used to control convulsions
and Propanolol may help tachycardia and
reversal of hyperglycaemia (however, beware
of using it in asthma).
77
A 45 year old woman has progressive
shortness of breath.
On examination she has O2 sats of 93% on air
and finger clubbing was noticed.
which one of the following is a typical
feature of cryptogenic fibrosing
alveolitis?
A. Dry cough
B. Haemoptysis
C. Haematuria
D. Wheeze
78
E. Abdominal pain
Answer: a) dry cough.
In extrinsic allergic alveolitis,
haemoptysis and wheeze
are uncommon.
Dry cough and
breathlessness are common
features.
79
A 45 year old man who works in a cotton mill presents
with fluctuating breathlessness. He has been
getting progressively more breathless for 10
months.
There is also associated chest tightness and cough. He
has been smoking 10 cigarettes a day for the past
10 years.
Upon assessment the patient has oxygen saturations
of 95% and a normal chest x ray.
What is the likely diagnosis?
A. Occupational asthma
B. Berylliosis
C. Extrinsic allergic alveolitis
D. Byssinosis
E. Sarcoidosis
80
Answer: d) byssinosis.
Byssinosis is caused by cotton dust, and is
commoner among smokers.
Immunologically, it is different from farmer’s
lung but is more akin to occupational
asthma – wheeze occurs after exposure to
cotton and hemp.
The condition is typically worse on Mondays
when work begins and lung function
stabilises throughout the week.
The CXR is normal, unlike extrinsic allergic
alveolitis, where mottling is seen on CXR
(interstitial pneumonitis).

81
82
83
84
85
86
87
88
89
90
91
92
93
94
95
96
97
98
99
100