Download Interstitial Lung Disease

Interstitial Lung Disease
TUCOM
Internal Medicine
4th class
Dr. Hasan.I.Sultan
Interstitial Lung Disease (ILD)
ILD is a heterogeneous group of pulmonary diseases
characterized by diffuse lung injury and
inflammation that frequently progresses to
irreversible fibrosis and severely compromised gas
exchange.
classified together because of similar clinical,
radiographic, physiologic, or pathologic
manifestations.
Features common to ILD
Clinical presentation
• Cough: usually dry, persistent and distressing
• Breathlessness: usually slowly progressive, insidious onset.
Examination findings
• Crackles: typically bilateral and basal
• Clubbing: common in idiopathic pulmonary fibrosis
• Central cyanosis and signs of right heart failure in advanced disease
Radiology
• Chest X-ray: typically small lung volumes with reticulonodular
shadowing
• HRCT: reticulonodular shadowing in early stage and honeycomb
cysts and traction bronchiectasis in advance stage.
Pulmonary function
• Typically restrictive ventilatory defect with
• reduced lung volumes and impaired gas transfer.
results of PFT in restrictive lung disease:
• Both forced expiratory volume in one second
(FEV1) and forced vital capacity (FVC) are reduced,
however, the decline in FVC is more than that of
FEV1, resulting in FEV1/FVC ratio higher than 80%.
• Both total lung capacity and lung volumes are
reduced.
• Reduce diffusing capacity of the lung: decrease the
transfer of gas from air to the lung by using carbon
monoxide (CO), (DLCO).
The Lung Interstitium
The interstitial space is defined as continuum of
loose connective tissue throughout the lung. It
concerns with alveolar epithelium, pulmonary
capillary endothelium, basement membrane,
perivascular and perilymphatic tissues, subpleural
and interlobular septae.
The interstitium of the lung is not normally visible
radiographically; it becomes visible only when
disease (e.g. edema, fibrosis, tumor) increases its
volume and attenuation.
Classification
1. ILD of unknown causes; Sarcoidosis and
idiopathic pulmonary Fibrosis (fibrosing alveolitis)
2. ILD due to organic dusts exposure;
a-Hypersensitivity pneumonitis (extrinsic allergic
alveolitis); Farmer's lung, Bird fancier's lung, Malt
worker's lung, Cheese worker's lung, Maple bark
stripper's lung.
b-Byssinosis; is acute bronchiolitis due to cotton
dust inhalation.
c-Inhalation ('humidifier') fever.
3- ILD due to inhalation of inorganic dusts;
a-Coal worker's pneumoconiosis; inhalation of coal
dust .
b-Silicosis; inhalation of silica dust.
c-Asbestosis; due to inhalation of asbestos fibers.
d-Berylliosis; due to inhalation Beryllium.
4- ILD due to systemic inflammatory diseases; due to
connective tissue disorders; Rheumatoid disease,
systemic lupus erythematosus, systemic sclerosis.
5- ILD due to irradiation.
Idiopathic pulmonary fibrosis
IPF is defined as a progressive fibrosing interstitial
pneumonia of unknown cause, occurring in adults and
associated with the histological or radiological pattern
of usual interstitial pneumonia (UIP). Previously
known as cryptogenic fibrosing alveolitis
There is some role of viral exposure e.g. EBV,
occupational dust e.g. metal or wood, drugs e.g.
antidepressant or GERD. There is a strong association
with cigarette smoking.
Clinical features; usually patients more than 50 yrs,
progressive breathlessness and a non-productive
cough.
Arthralgia may be reported. Finger clubbing and late
inspiratory crackles at lung bases. advanced cases
central cyanosis and cor pulmnale.
Investigations
• Rheumatoid factor and antinuclear factor can be detected
in 30-50% of patients. (ESR) is elevated in most cases.
• Pulmonary function tests show a restrictive defect with
reduced lung volumes and gas transfer.
• Abnormal chest X-ray at presentation with lower zone bibasal reticular and reticulonodular opacities. 'honeycomb'
appearance in advanced disease.
• HRCT may be diagnostic, demonstrating a patchy,
predominantly peripheral, subpleural and basal reticular
pattern with subpleural cysts (honeycombing).
• BAL (bronchoalveolar lavage) and transbronchial biopsy
may be used to exclude alternative diagnoses. Not require
in typical clinical features and HRCT.
Management
• Treatment is difficult. Although the combination of
prednisolone, azathioprine and N-acetylcysteine in
large studies suggest that it is mostly ineffective.
• Treat gastro-oesophageal reflux, pulmonary
hypertension and supplementation of oxygen.
• Lung transplantation should be considered in
young patients with advanced disease.
• A median survival of 3 years.
sarcoidosis
Sarcoidosis is a multisystemic
granulomatous disorder of
unknown aetiology. seen in
colder parts of Northern
Europe. Characterized by
non-caseating granuloma.
Over 90% of cases affect the
lungs.
Berylliosis
Similar clinical and
radiological features to
sarcoidosis, charactrized by
lung granulomas and
interstitial fibrosis.
occur in persons working in
aircraft, atomic energy and
electronics industries.
Coal worker's pneumoconiosis
Pulmonary fibrosis caused by prolonged inhalation of coal
dust. Which divided into;
1-Simple coal worker's pneumoconiosis (SCWP); scattered
discrete fibrotic lesions, does not cause pulmonary
function abnormalities or progress following cessation of
exposure.
2-Complicated pneumoconiosis; large dense masses appear
mainly in the upper lobes (also known as progressive
massive fibrosis, PMF). Presented as cough, production of
sputum, that may be black (melanoptysis) and
breathlessness. Respiratory failure after cessation of
exposure and right ventricular failure.
3-Caplan's syndrome describes the coexistence of
rheumatoid arthritis and rounded fibrotic nodules 0.5-5
cm in diameter.
Coal mining
Coal dust
Simple coal worker's pneumoconiosis
Complicated pneumoconiosis
Caplan's syndrome
Silicosis
• Silicosis results from the inhalation of crystalline or free
silica.
• Silica is highly fibrogenic and the disease is usually
progressive (even when exposure ceases).
• The clinical and radiological features are similar to those
of coal worker's pneumoconiosis, with multiple well
circumscribed 3–5-mm nodular opacities, predominantly
in the mid- and upper zones
• Enlargment of the hilar glands with an 'egg-shell' pattern
of calcification is characterestic.
• Silicosis are at increased risk of tuberculosis
(silicotuberculosis), lung cancer and COPD.
metal grinding
stone dressing
pottery
Asbestosis
Is a diffuse interstitial fibrosis of the lungs that may
or may not be associated with pleural fibrosis due
to exposure to fibrous mineral asbestos.
Requires substantial exposure over several years
Risk factor for carcinoma of the lung and larynx
Exertional breathlessness and fine, late inspiratory
crackles over the lower zones and digital clubbing.
Chest X-ray; Shows bi-basal reticular nodular
shadowing and asbestos-related pleural disease is
usually present. HRCT scanning is more sensitive.
Pulmonary function tests; Typically show a
restrictive defect with decreased lung volumes and
reduced gas transfer factor.
Asbestos bodies may be identified in sputum or BAL
and confirm asbestos exposure.
Asbestos dust
Demolition
Ship breaking
manufacture of fireproof insulating materials and brake-pads
Pipe Insulation
Management
• No specific treatment is available
• Prevention of occupational lung disease:
1. Limit exposure to safe level with specific
respiratory protection
2. Medical screening for early evidence of disease
3. Stop smoking
4. Change the job of patient because respiratory
symptoms related to occupation
5. Patient develop work related disability must be
established in worker’s compensation systems.
Thanks