Interstitial lung disease

57 Pages·2014·3.02 MB·English

Interstitial lung disease
Deepak Aggarwal
Dept of Pulmonary Medicine
Government Medical College and Hospital,
Chandigarh
What is Interstitium
• The interstitial space is defined as
continuum of loose connective tissue
throughout the lung
• It is the tissue between the air sacs of the
lungs
Subdivisions of lung interstitium
Axial interstitium
(peribronchovascular) Centrilobular interstitium
peripheral interstitium
Surrounding the bronchi, situated between the alveolar
contains the pulmonary
arteries, and veins from the and capillary basement
venules, lymphatics and
lung root to the level of the membranes
interlobular septae
respiratory bronchiole;
• Centrilobular bronchus (single wide white arrow) and artery (double white arrow, 1-mm
size)
• Interlobular septa (single arrowhead,0.1-mm thickness)
• Pulmonary vein (double arrowheads,0.5-mm size)
• Visceral pleura (single black arrow, 0.1-mm thickness)
• pulmonary acinus (single thin white arrow,5– 10 mm size
Introduction
• There are >200 disorders
• Can classified into
– known vs unknown with subcategory
of granulomatous and
nongranulomatous. OR
– occupational vs nonoccupational
– Acute vs Chronic
ILD Classification
Approach to
ILD
Idiopathic Granulomatous
ILD of known
Interstitial Lung Diseases Others
Cause
Pneumonias (Sarcoidosis)
LAM
Drugs Exposure CVD IPF IIP other than IPF HistiocytosisX
Malignancy
Respiratory
Desquamative
Hypersensitivity Bronchiolitis-
Pneumoconiosis Interstitial
Pneumonitis Interstitial Lung
Pneumonia
disease
Cryptogenic
Acute Interstitial Organizing
Toxic Inhalation Radiation
Pneumonia Pneumonia
Lymphocytic Non Specific
Interstitial Interstitial
Pneumonia Pneumonia
Classification of idiopathic
interstitial pneumonia
Classification
Interstitial Lung Diseases
Sarcoidosis
UIP/IPF
Fibrosis
Hypersensitivity Pneumonitis
AIP
“IIP”
1970s 2007
LIP
COP
Asbestosis
Cellular
LAM
NSIP
Fibrotic
etc
IPs=Interstitial pneumonias
LAM=lymphangioleiomyomatosis RB-
DIP
Adapted from Ryu JH, et al. Mayo Clin Proc. 1998;73:1085-1101. ILD Inflammation
Adapted from ATS/ERS. Am J Respir Crit Care Med. 2002;165:277-304.
Distribution
Occupation
11% DILD
Sarcoidosis
5% DAH
8%
4%
CTD
9%
Other
11%
Pulmonary Fibrosis
52%
Common features in ILD
– Dyspnea
– Diffuse disease on X ray
• Often the first suggestion of disease but correlates poorly
with severity of disease
– Restrictive PFTs
• Restrictive intrathoracic pattern: Normal airway flow rates but
low FVC
– Elevated A-a gradients