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Approach To
Interstitial Lung
or
Diseases
Diffuse Parenchymal Lung
Diseases
Objectives
Review the spectrum of ILD or DPLD
Identify clues on presentation to
make the diagnosis
Review common radiographic
findings in ILD
Diagnosis and management of DPLD
/ILD
Pulmonary Interstitium
Interstitial compartment is the portion of the lung
sandwiched between the epithelial and endothelial
basement membrane
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.
Pulmonary
lesionsFoc
al
Diffu
se
Pulmonarylesion
s
 Focal
 Diffuse
Approach to
DPLD/ILD
Granulomatous
Lung Diseases
(Sarcoidosis)
DPLD of known
Cause
Idiopathic Interstitial
Pneumonias
Others
LAM
Histiocytosis X
Malignancy
Drugs Exposure CTD IPF IIP other than IPF
Hypersensitivity
Pneumonitis
Desquamative Interstitial
Pneumonia
Respiratory Bronchiolitis-
Interstitial Lung disease
Pneumoconiosis
IPF: 47-64%
NSIP: 14 to 36%
RBILD/DIP: 10-
17%Cryptogenic Organizing
Pneumonia
Acute Interstitial
Pneumonia
Toxic Inhalation Radiation
COP: 4-12%
AIP: 2%
LIP: 2%
Lymphocytic Interstitial
Pneumonia
Non Specific Interstitial
Pneumonia
Clinical Presentation
• Dyspnea on exertion or a persistent
productive cough
Abnormal CXR,CT chest
non
•
• Pulmonary symptoms associated with
another disease, such as CVD
PFT abnormalities•
DIAGNOSIS OF DPLD/ILD
•
•
•
•
History
Physical Exam
Chest Radiograph,HRCT
Pulmonary Function
– At Rest
– Exercise
Serologic Studies
Tissue examination,
BAL, TBr, VATS, Open lung biopsy ,
Testing
•
•
History: Smoking
• All of the following
DPLD are associated
• In Goodpasture’s
syndrome
with smoking : 100% of smokers vs. 20%
of nonsmokers
experience pulmonary
hemorrhage
Individuals exposed to
asbestos who smoke are
more likely to develop
asbestosis
–
a)
b)
c)
d)
IPF
RBILD, DIP
Histiocytosis
•
X
Syndrome of IPF
emphysema
&
Occupational ????
History: Duration of Illness
1. Acute Diseases (Days to weeks)
• DAD (AIP), EP, Vasculitis/DPH, Drug, CTD
2. Subacute Diseases (weeks to months)
• HSP, Sarcoid, Cellular NSIP, Drug,
3. Chronic Diseases (months to years)
• UIP, Fibrotic NSIP, Pneumoconioses,
HSP
PLCH)
CTD-related, Chronic
Smoking (RBILD and
Patterns of Interstitial
Lung Disease
Interstitial peumonia
50 Y F, with cough and fever
Lymphangitis
carinomatosa
F 59Y, with radical mastectomy
Drug induced lung
diseases
Immunologic reaction to drugs
• Interstitial pattern similar to interstitial edema
which progresses to alveolar pattern
[busulfan, bleomycin, cytoxan,..]
Alveolar in filtrates similar to pulmonary
edema [penicillin, sulfonamides,..]
•
• Pleural and pericardial effusion + basal
infilterates [isonaizid,…]
Hilar adenopathy [antionvulsant,..]•
Busulfan
interstitial lung disease
Sarcoidos
is
Nodal and Interstitial
Pulmonary edema
Diffuse pulmonary
hemorrhage
Hemoptysis, anemia and air space opacities
Appear rapidly and clear within few days
Spare the lung apex and peripheral zones
Bilateral, may be asymmetric, air bronchogram
Repeated attacks → pulmonary fibrosis
Pulmonary
hemorrhage(normal heart) [3 days,
6days, one
month]
Pulmonary hemorrhage in SLE
Silicosis
Inhalation of high concentrations of silicon
dioxide
•
•
Fine interstitial opacities with B Kerley’s lines (early)
Multiple nodular shadows scattered in the lungs (classic)
•
•
Sparing apex and base
Calcification may occur
Complicated silicosis. PA chest radiograph shows multiple
nodules involving the upper and middle lungs, with coalescence
of nodules in the left upper lobe resulting in early progressive
massive fibrosis
CXR: LlMITATIONS
• CXR is normal:
– in 10 to 15 % of symptomatic patients with
proven
30% of
~ 60 %
infiltrative lung disease
those with bronchiectasis
of patients with emphysema
–
–
• CXR has a sensitivity of 80% and a
specificity of 82% percent for detection
of DPLD
CXR can provide a confident diagnosis
in ~ 23 % of cases
•




Radiographic
patterns
Reticular pattern
Ground glass pattern
Nodular pattern
Cystic pattern
Reticular pattern
[ Interlacing linear shadows appearing as a mesh or
net]
Usual interstitial pneumonia
Desquamative interstitial pneumonia
Acute interstitial pneumonia
Non specific interstitial pneumonia
Interstitial pulmonary edema
Idiopathic pulmonary fibrosis
Collagen vascular diseases
Drug induced lung diseases
Radiation induced lung diseases
Interstitial lung
disease
Honeycombing
HRCT showing
subpleural
broncheolectasis
IPF
Groundglass appearence
-Opacity without
obscuration of vessels.
- Resolving pulmonary
haemorrhage
Ground glass pattern
[ Increased attenuation of the lung with
preserved
broncho vascular marking
]
 Patients with AIDS, ground glass
opacities= P.carinii pneumonia
 Patients
ground
with lung transplant
opacities=glass
cytomegalovirus pneumonia or
rejection
P.carinii pneumonia
in
an AIDS patient
ERS 2008
GROUND GLASS:
PREVAILING FEATURE
GGO in: Outpatients with
Slowly Progressive Dyspnea
DI
P
Typically: subpleural /lower lung
zones
Reticulation seen in ~40-50%
Honeycombing NOT significant
ERS 2008
eds
Nodular pattern
10mm]
[ multiple rounded opacities
1-
Miliary [1-2mm], the size of millet s
TB
Metastases
Pneumoconiosis
Sarcoidosis
Alveolar cell carcinoma
 Innumerable fine nodules
Miliary
TB
 Hematogenous dissemination
 Uniform distribution
 Mild thickening of
the interstitial lung markings
Hypersensitivity pneumonitis
Extr. Allerg. Alveolitis (EAA) HRCT
Morphology
acute - subacute
acinar (centrilobular) unsharp densities
ground glass (patchy - diffuse)
chronic: fibrosis
Intra- / interlobular
septal thickening
Irregular interfaces
Traction
bronchiectasis
Young woman Dry mouth Smoker
LAM LIP Histiocytosis
CHURG STRAUSS SYNDROME
Eosinophilic Pneumonia
Chronic Eosinophilic Pneumonia
Acute Eosinophilic Pneumonia
Air space
filling ALVEOLAR EDEMA *
PNEUMONIA*
HEMORRHAGIC DISORDERS*
ALVEOLAR CELL CACINOMA
ALVEOLAR PROTIENOSIS*





TRASEUDATE
EXEUDATE
BLOOD
TUMOR CELLS
PROTEINS
MANAGEMENT OF DPLD/ILD
Interstitial fibrosis
Probability of Histologic Diagnosis of Diffuse
Transbronchial
Biopsy
Diseases
Surgical
Biopsy
Sometimes
Courtesy ofKevin O. Leslie, MD.
1. Granulomatous diseases
2. Malignant tumors/lymphangitic
3. DAD (any cause)
4. Certain infections Often
5. Alveolar proteinosis
6. Eosinophilic pneumonia
7. Vasculitis
8. Amyloidosis
9. EG/HX/PLCH Some
10. LAM
11. RB/RBILD/DIP
12. UIP/NSIP/LIP COP
13. Small airways disease Never
14. PHT and PVOD
s.no ILD type Current therapy* Additional and/or
alternative
therapies
1. IPF Supportive care
Consider anti-reflux
measures
Anti-reflux surgery
Acid suppressants
(e.g. PPI)
Pirfenidone
Lung transplantation
Anti-reflux therapy
N-acetylcysteine
2. SARCOIDOSIS Observation
(mild/stable disease
Corticosteroids
Methotrexate
Infliximab
Other IS agent
Lung transplantation
3. NSIP Lung
transplantation
Mycophenolate
Lung
transplantation
4. COP Corticosteroids Other IS drugs
Macrolides
5. HP Exposure cessation
Corticosteroids
Other IS drugs
Lung transplantation
6. Eosinophilic
pneumonia
Corticosteroids Other IS drugs
Lung
transplantation
7. CTD-ILD Corticosteroids
Mycophenolate
Other DMARD
agent(s)
Anti-reflux therapy
Treatment of PH
8 AIP/DAD Corticosteroids Cytotoxic drugs
Resting Tachypnea
Shallow breathing
Dry crackles
Digital clubbing
Pulmonary HTN
Non-pulmonary
findings
ILD/DPLD
• 81 in 100,000 prevalence in men
• 67 in 100,000 in women
• 32.5 in 100,000 incidence in men
• 26 in 100,000 in women
• 200 in 100,000 incidence in age >75
• 30-40% of all ILD “IPF”
•
•

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approach to interstitial lung disease

  • 2. Objectives Review the spectrum of ILD or DPLD Identify clues on presentation to make the diagnosis Review common radiographic findings in ILD Diagnosis and management of DPLD /ILD
  • 3. Pulmonary Interstitium Interstitial compartment is the portion of the lung sandwiched between the epithelial and endothelial basement membrane 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.
  • 6. Approach to DPLD/ILD Granulomatous Lung Diseases (Sarcoidosis) DPLD of known Cause Idiopathic Interstitial Pneumonias Others LAM Histiocytosis X Malignancy Drugs Exposure CTD IPF IIP other than IPF Hypersensitivity Pneumonitis Desquamative Interstitial Pneumonia Respiratory Bronchiolitis- Interstitial Lung disease Pneumoconiosis IPF: 47-64% NSIP: 14 to 36% RBILD/DIP: 10- 17%Cryptogenic Organizing Pneumonia Acute Interstitial Pneumonia Toxic Inhalation Radiation COP: 4-12% AIP: 2% LIP: 2% Lymphocytic Interstitial Pneumonia Non Specific Interstitial Pneumonia
  • 7. Clinical Presentation • Dyspnea on exertion or a persistent productive cough Abnormal CXR,CT chest non • • Pulmonary symptoms associated with another disease, such as CVD PFT abnormalities•
  • 8. DIAGNOSIS OF DPLD/ILD • • • • History Physical Exam Chest Radiograph,HRCT Pulmonary Function – At Rest – Exercise Serologic Studies Tissue examination, BAL, TBr, VATS, Open lung biopsy , Testing • •
  • 9. History: Smoking • All of the following DPLD are associated • In Goodpasture’s syndrome with smoking : 100% of smokers vs. 20% of nonsmokers experience pulmonary hemorrhage Individuals exposed to asbestos who smoke are more likely to develop asbestosis – a) b) c) d) IPF RBILD, DIP Histiocytosis • X Syndrome of IPF emphysema &
  • 11. History: Duration of Illness 1. Acute Diseases (Days to weeks) • DAD (AIP), EP, Vasculitis/DPH, Drug, CTD 2. Subacute Diseases (weeks to months) • HSP, Sarcoid, Cellular NSIP, Drug, 3. Chronic Diseases (months to years) • UIP, Fibrotic NSIP, Pneumoconioses, HSP PLCH) CTD-related, Chronic Smoking (RBILD and
  • 12.
  • 14. Interstitial peumonia 50 Y F, with cough and fever
  • 16. Drug induced lung diseases Immunologic reaction to drugs • Interstitial pattern similar to interstitial edema which progresses to alveolar pattern [busulfan, bleomycin, cytoxan,..] Alveolar in filtrates similar to pulmonary edema [penicillin, sulfonamides,..] • • Pleural and pericardial effusion + basal infilterates [isonaizid,…] Hilar adenopathy [antionvulsant,..]• Busulfan interstitial lung disease
  • 19. Diffuse pulmonary hemorrhage Hemoptysis, anemia and air space opacities Appear rapidly and clear within few days Spare the lung apex and peripheral zones Bilateral, may be asymmetric, air bronchogram Repeated attacks → pulmonary fibrosis Pulmonary hemorrhage(normal heart) [3 days, 6days, one month]
  • 21. Silicosis Inhalation of high concentrations of silicon dioxide • • Fine interstitial opacities with B Kerley’s lines (early) Multiple nodular shadows scattered in the lungs (classic) • • Sparing apex and base Calcification may occur
  • 22. Complicated silicosis. PA chest radiograph shows multiple nodules involving the upper and middle lungs, with coalescence of nodules in the left upper lobe resulting in early progressive massive fibrosis
  • 23. CXR: LlMITATIONS • CXR is normal: – in 10 to 15 % of symptomatic patients with proven 30% of ~ 60 % infiltrative lung disease those with bronchiectasis of patients with emphysema – – • CXR has a sensitivity of 80% and a specificity of 82% percent for detection of DPLD CXR can provide a confident diagnosis in ~ 23 % of cases •
  • 25. Reticular pattern [ Interlacing linear shadows appearing as a mesh or net] Usual interstitial pneumonia Desquamative interstitial pneumonia Acute interstitial pneumonia Non specific interstitial pneumonia Interstitial pulmonary edema Idiopathic pulmonary fibrosis Collagen vascular diseases Drug induced lung diseases Radiation induced lung diseases Interstitial lung disease
  • 27. Groundglass appearence -Opacity without obscuration of vessels. - Resolving pulmonary haemorrhage
  • 28. Ground glass pattern [ Increased attenuation of the lung with preserved broncho vascular marking ]  Patients with AIDS, ground glass opacities= P.carinii pneumonia  Patients ground with lung transplant opacities=glass cytomegalovirus pneumonia or rejection P.carinii pneumonia in an AIDS patient
  • 29. ERS 2008 GROUND GLASS: PREVAILING FEATURE GGO in: Outpatients with Slowly Progressive Dyspnea
  • 30. DI P Typically: subpleural /lower lung zones Reticulation seen in ~40-50% Honeycombing NOT significant ERS 2008
  • 31. eds Nodular pattern 10mm] [ multiple rounded opacities 1- Miliary [1-2mm], the size of millet s TB Metastases Pneumoconiosis Sarcoidosis Alveolar cell carcinoma  Innumerable fine nodules Miliary TB  Hematogenous dissemination  Uniform distribution  Mild thickening of the interstitial lung markings
  • 32. Hypersensitivity pneumonitis Extr. Allerg. Alveolitis (EAA) HRCT Morphology acute - subacute acinar (centrilobular) unsharp densities ground glass (patchy - diffuse) chronic: fibrosis Intra- / interlobular septal thickening Irregular interfaces Traction bronchiectasis
  • 33. Young woman Dry mouth Smoker LAM LIP Histiocytosis
  • 34. CHURG STRAUSS SYNDROME Eosinophilic Pneumonia Chronic Eosinophilic Pneumonia Acute Eosinophilic Pneumonia
  • 35. Air space filling ALVEOLAR EDEMA * PNEUMONIA* HEMORRHAGIC DISORDERS* ALVEOLAR CELL CACINOMA ALVEOLAR PROTIENOSIS*      TRASEUDATE EXEUDATE BLOOD TUMOR CELLS PROTEINS
  • 38. Probability of Histologic Diagnosis of Diffuse Transbronchial Biopsy Diseases Surgical Biopsy Sometimes Courtesy ofKevin O. Leslie, MD. 1. Granulomatous diseases 2. Malignant tumors/lymphangitic 3. DAD (any cause) 4. Certain infections Often 5. Alveolar proteinosis 6. Eosinophilic pneumonia 7. Vasculitis 8. Amyloidosis 9. EG/HX/PLCH Some 10. LAM 11. RB/RBILD/DIP 12. UIP/NSIP/LIP COP 13. Small airways disease Never 14. PHT and PVOD
  • 39. s.no ILD type Current therapy* Additional and/or alternative therapies 1. IPF Supportive care Consider anti-reflux measures Anti-reflux surgery Acid suppressants (e.g. PPI) Pirfenidone Lung transplantation Anti-reflux therapy N-acetylcysteine 2. SARCOIDOSIS Observation (mild/stable disease Corticosteroids Methotrexate Infliximab Other IS agent Lung transplantation 3. NSIP Lung transplantation Mycophenolate Lung transplantation
  • 40. 4. COP Corticosteroids Other IS drugs Macrolides 5. HP Exposure cessation Corticosteroids Other IS drugs Lung transplantation 6. Eosinophilic pneumonia Corticosteroids Other IS drugs Lung transplantation 7. CTD-ILD Corticosteroids Mycophenolate Other DMARD agent(s) Anti-reflux therapy Treatment of PH 8 AIP/DAD Corticosteroids Cytotoxic drugs
  • 41. Resting Tachypnea Shallow breathing Dry crackles Digital clubbing Pulmonary HTN Non-pulmonary findings ILD/DPLD • 81 in 100,000 prevalence in men • 67 in 100,000 in women • 32.5 in 100,000 incidence in men • 26 in 100,000 in women • 200 in 100,000 incidence in age >75 • 30-40% of all ILD “IPF”