.interestitial lung disease

1. INTERSTITIAL & INFILTRATIVE
PULMONARY DISEASES
Dr.Bilal Natiq Nuaman,MD
C.A.B.M.,F.I.B.M.S.,D.I.M.
2016-20171

2. Diffuse parenchymal lung disease
(Interstitial lung disease)
• The diffuse parenchymal lung diseases (DPLDs)
are a heterogeneous group of conditions
affecting the pulmonary parenchyma
(interstitium)and/or alveolar lumen, which are
frequently considered collectively as they
share a sufficient number of clinical
physiological and radiographic similarities.
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Drugs causing interstitial lung disease

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8. Idiopathic pulmonary fibrosis
• Idiopathic pulmonary fibrosis (IPF) is defined as
a progressive fibrosing interstitial pneumonia
of unknown cause, occurring in adults.
Clinical features
• IPF usually presents in the older adult and is
uncommon before the age of 50 years.
• more typically presents with progressive
breathlessness and a non-productive cough.
Clinical findings include
finger clubbing and the presence of bi-basal
fine late inspiratory crackles.
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9. Investigations
Established IPF will be apparent on chest X-ray
as bilateral lower lobe and subpleural
reticular shadowing.
HRCT typically demonstrates a patchy,
predominantly peripheral, subpleural and
basal reticular pattern and, in more advanced
disease, the presence of honeycombing cysts
and traction bronchiectasis.
Pulmonary function tests classically show a
restrictive defect with Reduced lung volumes
and gas transfer.
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12. prognosis
In advanced disease, central cyanosis is
detectable and patients may develop
features of right heart failure.
A median survival is 3 years .
• Various autoimmune diseases are associated
with IPF (SLE,RA).
IPF is associated with an increased risk of
carcinoma of the lung.
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13. Management
Treatment is difficult.
Oxygen may help breathlessness but opiates
may be required to relieve severe dyspnea.
Where appropriate, lung transplantation
should be considered.
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Sarcoidosis
is a multi system disorder,
characterized by noncaseating
epithelioid granulomas.
The etiology is unknown, although
atypical mycobacteria, viruses and
genetic factors have been proposed.
Sarcoidosis is less common in smokers.
sparing Arab and Chinese populations.

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Clinical features
Any organ may be affected but 90% of cases affect the
lungs. Otherwise, lymph glands, liver, spleen, skin, eyes,
parotid glands and phalangeal bones are the most
frequently involved sites.
bilateral hilar lymphadenopathy( BHL) may be detected
in an otherwise asymptomatic individual undergoing
CXR.
Löfgren’s syndrome – erythema nodosum, peripheral
arthropathy, uveitis, bilateral hilar lymphadenopathy
(BHL), lethargy and occasionally fever – commonly
presents in the third or fourth decade.
Pulmonary disease may present insidiously with cough,
exertional breathlessness and radiographic infiltrates.

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The most common cause for
bilateral hilar enlargement in
an asymptomatic person
(incidental radiologic finding)
is sarcoidosis.

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Investigations
● FBC: demonstrates lymphopenia.
● LFTs: may be mildly deranged.
● Ca2+: may be elevated.
● Serum ACE: may provide a nonspecific marker of disease
activity.
● Chest x-ray : bilateral hilar lymphadenopathy and/or
parenchymal infiltrates.
● HRCT: characteristic appearances include reticulonodular opaci-
ties.
● Pulmonary function test : restrictive pattern (normal FEV1/FVC)
● Bronchoscopy: may demonstrate a ‘cobblestone’ appearance of
the mucosa, and bronchial and transbronchial biopsies usually
show noncaseating granulomas.
The occurrence of erythema nodosum in patients in the
second to third decades with BHL on CXR is often sufficient
for a confident diagnosis.

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Management
The majority of patients enjoy spontaneous remission.
Patients who present with acute illness and erythema nodosum
may require NSAIDs and, if systemically unwell, a short course of
corticosteroids.
Systemic corticosteroids are also indicated in the presence of
hypercalcaemia,
pulmonary function impairment and
renal impairment.
Uveitis responds to topical steroids.
In patients with severe disease, both methotrexate and
azathioprine have been used successfully and selected patients
with severe disease may be referred for single lung
transplantation.

21. OCCUPATIONAL LUNG DISEASE
Exposure to dusts, gases, vapors and fumes at work
can Cause several different types of lung disease:
• Acute bronchitis and even pulmonary edema
from irritants such as sulphur dioxide, chlorine or
ammonia.
• Occupational asthma-this is now the Commonest
industrial lung disease in the developed world
• Occupational pneumonia.
• Hypersensitivity pneumonitis.
• Pulmonary fibrosis due to mineral dust.
• Bronchial carcinoma and mesothelioma due to
industrial agents( e.g. Asbestos, silicosis). 21

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23. Occupational pneumonia
• Welders appear to be at increased risk of
pneumonia.
• Farm, and abattoir workers may be exposed to
Coxiella burnetii, the causative agent of Q fever
(Atypical pneumonia+hepatitis+endocarditis).
• Birds (parrots) infected with Chlamydia psittaci
can cause psittacosis in humans.
• Sewage workers, farmers, animal handlers and
veterinarians run an Increased risk of
contracting leptospirosis (pneumonia+hepatitis
+renal failure).
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24. Hypersensitivity pneumonitis
Hypersensitivity pneumonitis (HP; also called extrinsic
allergic alveolitis) results from the inhalation of a wide
variety of organic antigens, which give rise to a diffuse
immune complex reaction in the alveoli and
bronchioles.
Common causes include farmer’s lung and bird
fancier’s
lung.
Cigarette smokers have a lower risk of
developing the disease due to decreased antibody
reaction to the antigen.
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25. Clinical features
Typically fever, malaise, cough and shortness of
breath come on several hours after exposure to
the causative antigen. Thus, a farmer forking hay
in the morning may notice symptoms during the
late afternoon and evening with resolution by the
following morning.
On examination, the patient may have a fever,
tachypnoea, and coarse end inspiratory
crackles and wheezes throughout the chest.
Continued exposure leads to a chronic illness
characterized by severe weight loss, effort
dyspnea and cough as well as the features of
idiopathic pulmonary fibrosis .
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26. Management
If practical, the patient should cease exposure to
the inciting agent.
Dust masks with appropriate filters may minimize
exposure .
In acute cases, prednisolone should be given for 3–4
weeks, starting with an oral dose of 40 mg per
day. Severely hypoxemic patients may require
high concentration oxygen therapy initially.
Most patients recover completely but, if
unchecked, fibrosis may progress to cause severe
respiratory disability, hypoxemia, pulmonary
hypertension, corpulmonale and eventually
death.
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Pneumoconiosis
This means a permanent alteration of lung structure due to
the inhalation of (inorganic) mineral dust, excluding
bronchitis and emphysema.
Pneumoconiosis is a general term used to describe lung
fibrosis resulting from inhalation of dusts such as coal, silica
and asbestos.

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Coal worker’s pneumoconiosis (CWP): Prolonged inhalation
of coal dust overwhelms alveolar macrophages, leading to a
fibrotic reaction. Simple coal worker’s pneumoconiosis
(SCWP) refers to the appearance of small radiographic
nodules in an other wise well individual. Progressive
massive fibrosis (PMF) refers to the formation of
conglomerate masses (mainly in the upper lobes), which
may cavitate, associated with cough, sputum and
breathlessness. PMF may progress after coal dust exposure
ceases and, in extreme cases, causes respiratory failure.

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Silicosis:
This occurs in stonemasons who inhale crystalline
silica, usually as quartz dust.
Classic silicosis develops slowly after years of
asymptomatic exposure.
Radiological features are similar to those of Coal
worker’s pneumoconiosis (CWP), with multiple 3–5
mm nodular opacities, in the mid and upper zones.
As the disease progresses, Progressive massive
fibrosis (PMF) may develop.
Enlargement of the hilar glands with an ‘eggshell’
calcification is uncommon and non specific.
Fibrosis progresses even when exposure ceases.
Individuals with silicosis are at increased risk of TB,
lung cancer and COPD.

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Industries and occupations at risk for silicosis are:
➤ tunnel mining
➤ underground excavation
➤ quarrying: sandstone, granite, sand
➤ stone work: granite sheds, monumental masonry,
tombstones
➤ ceramic industry: manufacture of pottery, stone-
ware, bricks for ovens
➤ sandblasting

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Asbestosis
Asbestosis is a pneumoconiosis in which diffuse
parenchymal lung fibrosis develops as a result of
heavy prolonged exposure to asbestos. The lag
interval between exposure and the onset of
disease is typically 30–40 years, the clinical
features are similar to those of idiopathic
pulmonary fibrosis, with cough, progressive
dyspnoea, bibasal crackles, clubbing (in
advanced disease) and a restrictive ventilatory
defect (reduced lung volumes) with impaired gas
diffusion (reduced transfer factor for carbon
monoxide).
Chest X-ray shows bilateral, predominantly basal
reticulonodular shadowing.

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In contrast to silicosis,
asbestosis does not predispose to
tuberculosis.
However, it is associated with a high risk
of bronchial cancer, pleural and peritonea
mesothelioma, and gastrointestinal
cancers.

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Mesothelioma
Mesothelioma is a malignant tumor of
the pleura, in which there is an
identifiable history of asbestos
exposure(20-40 years) in at least 90%
of cases. It usually presents with pain,
dyspnoea, weight loss and lethargy, and
features of a pleural effusion .

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CHARACTERISTIC CXR PATTERNS FOR INTERSTITIAL LUNG DISEASE
· UPPER LOBE PREDOMINANCE—sarcoidosis, hypersensitivity pneumonitis,
pneumoconiosis, silicosis, ankylosing spondylitis, TB
· LOWER LOBE PREDOMINANCE—idiopathic pulmonary fibrosis, asbestosis,
rheumatoid arthritis, scleroderma.
· BILATERAL HILAR/MEDIASTINAL ADENOPATHY WITH INTERSTITIAL INFILTRATES—
sarcoidosis, TB, fungal, lymphoma.
· EGGSHELL CALCIFICATION OF HILAR/MEDIASTINAL LYMPH NODES—silicosis (other
pneumoconiosis), TB, fungal.
· CALCIFIED PLEURAL PLAQUES—asbestos
• PLEURAL EFFUSIONS WITH INTERSTITIAL INFILTRATES—HF, lymphangitic
carcinomatosis, rheumatoid arthritis, SLE

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Pulmonary hypertension
In normal lungs, the pulmonary arterial pressure is
about 20/8mmHg and the mean pulmonary artery
pressure is 12–15mmHg.
Pulmonary hypertension is defined as a mean
pulmonary artery pressure of >25 mmHg at rest.
It may occur as a result of hypoxemia and chronic
lung disease, when it is often referred to as
cor pulmonale, but in some cases there is no
demonstrable cause, which is termed idiopathic
pulmonary hypertension.
Presentation is with exertional dyspnea and
syncope.
Primary pulmonary hypertension (PPH) is a rare but
important disease that predominantly affects
women, aged 20–30 yrs.

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Causes of Secondary Pulmonary Hypertension
Cardiac Disease
Eisenmenger syndrome
Left-sided heart failure
Parenchymal Lung Disease
Chronic obstructive pulmonary disease
Interstitial lung disease
Vascular Impairment
Chronic Pulmonary thromboembolic disease
Sickle cell disease
Infections
HIV
Schistosomiasis
Miscellaneous
Obstructive sleep apnea
Collagen vascular diseases (through either direct vascular effect or interstitial
changes in the parenchyma)

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Electogardiograpgy
Eighty per cent of patients with pulmonary hypertension have an
abnormal electrocardiogram:
Right axis deviation,
‘P pulmonale’,
Dominant R in right precordial leads,
Right bundle branch block)

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Chest radiography
■ Shows enlarged pulmonary arteries with rapid tapering of vessels toward the
periphery of the lungs (a “pruned tree” appearance) (Fig. 22-8)
■ May also see right-heart enlargement
■ For secondary pulmonary hypertension caused by
parenchymal lung disease, look for hyperinflation and bullous disease (suggestive of
COPD) or increased interstitial markings (suggestive of interstitial lung disease)

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Management
includes diuretics, oxygen, anticoagulation and vaccination against infection.
Specific treatments include
sildenafil (viagra) and
the oral endothelin receptor antagonist bosentan;
these can dramatically improve exercise performance symptoms and prognosis in
selected cases.
Pulmonary thrombo-endarterectomy should be contemplated in patients with
chronic proximal pulmonary thromboembolism .
Heart–lung transplantation may be considered in selected patients.

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Cor Pulmonale
Cor pulmonale, often referred to as pulmonary heart
disease, can be defined as altered RV structure and/or
function in the context of chronic lung disease and is
triggered by the onset of pulmonary hypertension.
Cor pulmonale is the third most common cardiac disorder after the
age of 50.
Pulmonary embolism is the most common cause of acute cor
pulmonale.

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causes of cor pulmonale
Respiratory disorders':
· Obstructive:
- COPD.
-Chronic persistent asthma.
· Restrictive:
-Intrinsic - interstitial fibrosis, lung resection.
- Extrinsic - obesity, muscle weakness, kyphoscoliosis.
Pulmonary vascular disorders:
· Pulmonary emboli.
· Adult respiratory distress syndrome.
· Primary pulmonary hypertension.
· Vasculitis of the small pulmonary arteries.

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The most common cause of right HF is not pulmonary
parenchymal or vascular disease but left HF. Therefore, it
is important to evaluate the patient for LV systolic and
diastolic dysfunction.
Orthopnea and PND are rarely symptoms of isolated right HF
and usually point toward concurrent left heart dysfunction.
The increase in intensity of the holosystolic murmur of
tricuspid regurgitation with inspiration (“Carvallo’s sign”)
may be lost eventually as RV failure worsens.
BNP levels are elevated in patients with cor pulmonale secondary to
RV myocardial stretch

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TREATMENT
The treatment of cor pulmonale is directed at the underlying cause while at
the same time reversing hypoxemia, improving RV contractility, decreasing
pulmonary artery vascular resistance, and improving pulmonary
hypertension.
● Continuous positive airway pressure (CPAP) is used in patients with
obstructive sleep apnea.
● Phlebotomy is reserved as adjunctive therapy in polycythemia patients
(hematocrit, 55%) who have acute decompensation of cor pulmonale or
remain polycythemic despite long-term oxygen therapy.
● Acute pulmonary exacerbating conditions should be treated.
● Long-term oxygen supplementation has improved survival in hypoxemic
patients with COPD.
● RV volume overload should be treated with diuretics (e.g., furosemide);
however, overdiuresis can reduce RV filling and decrease cardiac output.
● Theophylline and sympathomimetic amines may improve diaphragmatic
excursion, myocardial contraction, and pulmonary artery vasodilation.

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the possible causes of collapse:
Malignancy (cachexia, clubbing. nicotine staining, lymphadenopathy)
Tuberculosis (apical signs, lymphadenopathy)
Hilar lymphadenopathy, i.e. sarcoidosis (erythema nodosum)
Mucus plugs(features of asthma ,COPD, or bronchiectasis)
Foreign body
Atelectasis
Atelectasis is the collapse of lung volume resulting in reduced or absent gas
exchange. It may affect part or all of a lung. It is usually not bilateral. It is a
condition where the alveoli are deflated down to little or no volume, as
distinct from pulmonary consolidation, in which they are filled with liquid.

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CLINICAL PRESENTATION AND PHYSICAL FINDINGS
• Cough, dyspnea
• Trachea deviated towards lesion
• Diminished chest expansion
• Dull chest percussion
●Decreased vocal fremitus and vocal resonance
• Decreased or absent breath sounds

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Respiratory causes of clubbing
Interstitial lung disease
Carcinoma of the lung
Mesothelioma
Bronchiectasis
Cystic fibrosis
Lung abscess
Empyema
long standing TB

51. Thank You
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