Transcript Ragheb A. Assaly Professor of Medicine Pulmonary/Critical Care/Sleep University Of Toledo Medical Center.

Ragheb A. Assaly
Professor of Medicine
Pulmonary/Critical Care/Sleep
University Of Toledo Medical Center
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Anatomy of the pulmonary interstitium.
ILD patterns on CT scan.
Idiopathic Interstitial Fibrosis
Nonspecific Interstitial pneumonia
Collagen vascular Diseases associated ILD.
Sarcoidosis
Common presentation of Uncommon
Interstitial Lung Diseases.
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How may % of patients with proven Interstitial
Lung Disease have normal Chest X-ray?
5%
16-20%
30%
1%
50%
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60 year old female. 1 year history of increasing
shortness of breath following recurrent UTI.
Primary Physician concerned that lots of basal
crackles on examination.
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Affects women more women than men
Upper and middle lobes are frequently
affected.
Smokers are not more likely to develop IPF.
Ground glass opacity is a common feature.
Honeycombing is rare.
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Affects upper lobe more than lower lobes
Honeycombing is common
Five year survival is less than 5%years
Predominant pathology in collagen vascular
disease
a) Interstitial lung disease (ILD) affects 70-85%%
b) Aspiration pneumonia is rare.
c) Pneumomediastinum is a known pulmonary
complication of dermatomyositis
d) Anti-Jo-1 antibodies is positive in 90% of the
patients
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Nodular thickening along the bronchovascular
bundles and interlobar fissures.
Erythema Nodosum is a poor prognostic
indicator
affects African population more than
Scandinavian.
Most marked in lower zones
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60% of patients with Lymphangiolyomatosis
have angiolipoma of the kidney
GM-CSF has a no role in maturation of
macrophages.
Anti GM-CSF has been incriminated in alveolar
proteinosis.
Eosinophilic granuloma can be associated with
Diabetes Mellitus
The secondary pulmonary lobule is a unit of lung
supplied by three to five
terminal bronchioles and contained by fibrous
septa .
Smallest unit of lung structure marginated by
connective tissue septae.
Airways, pulmonary arteries, veins, lymphatics,
and the lung interstitium are all represented at
the level of the secondary lobule.
Airspaces and the Interstitium
THE SECONDARY PULMONARY LOBULE
Secondary pulmonary lobule and acinus
The lobular bronchiole is shown dividing into smaller
branches, which supply the acini.
lobular bronchiole
acinus.
Diseases that affect the terminal airspaces.
 Airspace disease/Alveolar
disease/consolidation
 Replacement of gas in the airspaces
pus
blood
edema
cells
Definition:- disease of the connective tissue framework of the lung
Interlobular septal thickening in pulmonary edema. Transverse thinsection CT scan shows thickened septa (small arrows) in upper lobes.
Smooth thickening of interlobular septa outline a number of secondary
pulmonary lobules. Visible lobules vary in size, at least partly because of
the position of lobules relative to the scan plane. Pulmonary veins (large
arrows) in septa are visible as small rounded dots or linear or branching
opacities. Septa arewell developed in the apices, and septal thickening
is often well depicted in this region.
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CXR limited in sensitivity and
specificity
10-16% of patients with biopsy
proven interstitial lung disease have
normal chest radiographs.
High percentage (20%) false
positives.
Increased lung opacity that does not obscure underlying vessels
Tree-in-bud sign associated with
bronchiolar infection. (a) Transverse
thin-section CT scan through right lower
lobe in a patient with airways disease
and bacterial infection related to
acquired immunodeficiency syndrome.
Multiple impacted centrilobular
bronchioles result in tree in- bud
appearance (arrowheads).
Bronchiectasis is also present. (b) Lung
slice from patient with
bronchopneumonia. Impacted mucusand pusfilled bronchioles (arrows) are
visible throughout the lung; this is the
pathologic examination equivalent of the
tree-in-bud sign.
Centrilobular nodules in
hypersensitivity pneumonitis.
Transverse thin-section
CT scan shows small ill-defined
centrilobular nodules separated
from pleural surface and fissure
by several millimeters. The
nodules arise in relation
to centrilobular bronchioles and
appear as lobular rosettes
(arrows).
Is the ILD acute or chronic?
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Usual interstitial Pneumonia
Confident HRCT diagnosis usually correct.
Positive predictive value of 95-100%.
Confident diagnosis cannot be made in up to
50%.
Confident diagnosis difficult to make if no
honeycombing.
CXR peripheral basal interstitial
lung disease with
Irregular reticular opacities and
traction bronchiectasis.
– HONEYCOMBING.
– Distribution basal and peripheral
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Widespread ground-glass opacity.
Very subtle background reticulation.
Basal predominance.
No honeycombing.
Diffuse, uniform thickening of alveolar septa
NSIP
1. NSIP is a pattern of interstitial lung injury that is
distinct from other interstitial pneumonias, and can be
idiopathic or associated with an underlying disease.
2. It is essential to distinguish NSIP from UIP, since
patients with NSIP tend to respond to corticosteroid
therapy and have a distinctly better prognosis than
those with UIP.
3. There are currently no noninvasive tests to enable a
specific diagnosis of NSIP. Surgical lung biopsy is
required for definitive diagnosis and should be
considered in all patients suspected of having NSIP.
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HRCT more sensitive and specific
More sensitive 94% (80% CXR)
More specific 96% (82% CXR)
At least 10% more accurate in making a
diagnosis
Thin slices, 1mm
Sharp reconstruction algorithm
Images every 1-2 cm
Prone scans commonly used to avoid
misdiagnosis due to dependent lung
atelectasis
expiratory HRCT showing that some lung
regions remain transparent as a result of
air trapping, while normal lung regions
increase attenuation.
Am J Crit Care Med Tamadge King April 2005
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Physiologic Testing
Surgical Biopsy
Bronchoalveolar Lavage (BAL)
High Resolution CT (HRCT)
Classic physiologic response to ILD is a
a) restrictive pattern (FVC) .
b) and/or impaired gas exchange (DLCO).
Some ILD are associated with obstructive
pattern: Sarcoidosis , Hypersensitivity
Pneumonia, Eosinophilic Granuloma,
Lymphangioliomatosis, Tuberous sclerosis &
neurofibromatosis.
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Neither PFT or exercise testing can
discriminate between fibrosis and
inflammation.
Isolated pulmonary function studies have
limited impact on prognosis and disease
progression.
Serial measurements in an individual patient
are invaluable in determining disease
progression/response to therapy.
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Systemic sclerosis may be commonly
associated with chronic interstitial pneumonia
(especially in patients with diffuse systemic
sclerosis and anti-topoisomerase-1 antibodies),
or pulmonary arterial hypertension (most
frequently, but not exclusively, in patients with
limited systemic sclerosis with anti-centromere
antibodies).
HRCT scan of NSIP in a 41-year-old man with
Scleroderma
HRCT scan of UIP in a 63-year-old man with SS
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Acute lupus pneumonitis.
Diaphragmatic dysfunction and shrinking lung
syndrome.
Cavitating pulmonary nodules.
Pulmonary hypertension.
Pulmonary vasculitis.
Pulmonary embolism (often due to circulating
anticardiolipin antibodies).
Alveolar hemorrhage (reflecting diffuse endothelial
injury).
bronchiolitis obliterans (with or without organising
pneumonia).
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Shrinking lungs syndrome is characterized by
unexplained dyspnea,
a restrictive pattern on pulmonary function test
results, and
an elevated hemidiaphragm.
The cause of SLS remains controversial, with
several authors attributing the disorder to
diaphragmatic weakness and others suggesting
that chest wall restriction accounts for the
clinical syndrome. Good results with
corticosteroids..
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Rheumatoid arthritis may be responsible for a
broad spectrum of manifestations, including:
pleural effusion; chronic interstitial
pneumonia; lung rheumatoid nodules;
bronchiectasis; obstructive ventilatory defect
due to constrictive bronchiolitis; laryngeal
involvement; and opportunistic infections,
such as mycobacterial infections facilitated by
anti-tumor necrosis factor- treatments.
Interstitial lung disease (ILD), 5–30%
Aspiration pneumonia.
Ventilatory failure secondary to diaphragmatic
dysfunction
pneumomediastinum
Anti-Jo-1 antibodies
A fibreoptic bronchoscopy, showing studded white plaques on the bronchial
mucosa at the carina (A) and the main (B), lobar and segmental bronchi of
both lungs. Necrosis of the skin perforation.
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Nodular thickening along the bronchovascular
bundles and interlobar fissures.
Some subpleural nodules
Lymphadenopathy.
Distribution typically perilymphatic.
Most marked in upper and midzones.
Chest Radiographic Images of Stage 1 and Stage 3 Sarcoidosis
Weinberger, S. E. JAMA 2006;296:2133-2140.
Clinical Features of Sarcoidosis
Iannuzzi M et al. N Engl J Med 2007;357:2153-2165
Shortness of breath, Dry
cough for 6 months,
Normal CXR.
Patchy bilateral GGO
Small ill defined centrilobular,
nodules Lobular areas of
decreased attenuation-air
trapping.
--53-year-old man with history of pulmonary alveolar proteinosis who developed Nocardia
pulmonary abscess and Nocardia osteomyelitis of three right ribs
Holbert, J. M. et al. Am. J. Roentgenol. 2001;176:1287-1294
Copyright © 2007 by the American Roentgen Ray Society
30 year old female with chest pain and kidney mass.
42 year old heavy smoker with
polyuria,
shortness of breath