pulmonary fellows

1. DIFFUSE PARENCHYMAL LUNG
DISEASE
Dr Nahid Sherbini
Internal Medicine & Pulmonary Consultant
Certified from Harvard Medical School in Practice of clinical Research

2. LearningTargets -I-
•To elaborate the general diagnostic approach to
ILDs and Classifications
•To diagnose IPF and understand treatment
options
•To recognize forms of IIP and clinical relevance

3. DPLD I
Idiopathic Interstitial Pneumonias (IIPs)
• IPF
• Other IIPs
• Familial IP
• IP with autoimmune features (IPAF)
• Smoking-related ILDs

4. LearningTargets -II-
To elaborate the diagnosis to other
ILDs including clinical , radiological
findings and management lines.

5. DPLD II
• CTD-associated ILDs
•Diffuse Cystic Lung Diseases
Lymphangioleiomyomatosis (LAM)
Pulmonary Langerhans cell histiocytosis (PLCH)
•Others , Pulmonary alveolar proteinosis & DAD
•Radiation Iduced Lung Injury

6. Introduction
(ILDs) are a heterogeneous group of
disorders that are classified together
because of similar clinical, radiographic,
physiologic, or pathologic manifestations .

7. Pulmonary interstitium
• Alveolar lining cells
(types 1 and 2)
• Thin elastin-rich
connective
component
containing capillary
blood vessels

8. What is the Pulmonary Interstitium?
•between the epithelial and
endothelial basement
membrane
•Expansion of the interstitial
compartment by
inflammation with or
without fibrosis
• Necrosis
• Hyperplasia
• Collapse of basement
membrane
• Inflammatory cells

9. Pathogenesis
The pathogenesis of ILDs is unknown.
But more and more facts have shown that
immune cells and their cytokines play an important
role in the course of ILDs.

10. Classification
•Divided into
-Associated with known causes and
-Idiopathic.
The treatment choices and prognosis vary among the
different causes and types of ILD

11. Classification

12. ILD/DPLD
DPLD of known
Cause
Drugs Exposure
Hypersensitivity
Pneumonitis
Pneumoconiosis
Toxic Inhalation Radiation
CVD
Idiopathic Interstitial
Pneumonias
IPF IIP other than IPF
Desquamative
Interstitial
Pneumonia
Respiratory
Bronchiolitis-
Interstitial Lung
disease
Acute Interstitial
Pneumonia
Cryptogenic
Organizing
Pneumonia
Lymphocytic Interstitial
Pneumonia
Non Specific Interstitial
Pneumonia
Granulomatous
Lung Diseases
(Sarcoidosis)
Others
LAM
Histiocytosis X
Malignancy
IPF: 47-64%
NSIP: 14 to
36%
RBILD/DIP:
10-17%
COP: 4-12%
AIP: 2%
LIP: 2%

13. Q
• What is the commonest ILD ?
a. Sarcoidosis
b. IPF
c. HP
d. NSIP
e. LIP

14. Incidence of ILD
Sarcoidosis
8%
Occupation
11% DILD
5% DAH
4%
CTD
9%
Other
11%
Pulmonary Fibrosis
52%
Coultas AJRCCM ; 150:967
(Incidence of IPF=26-31 per 100,000)

15. To Diagnose ILD
1- Clinical context (clinical features, labs,
PFTs)
2- Disease nature: acute/subacute, chronic
3-Radiologic (HRCT) findings : Distribution
• Pattern of opacities • Associated findings

16. Q
What is the most important predictor for mortality in IPF patients?
a. Male gender
b. Age
c. DLCO
d. 6MWTS distance
e. Low FVC

17. Age and Gender
• LAM
Age
Gender

18. 3. Subacute Diseases (weeks to months)
• HSP, Sarcoid, Cellular NSIP, Drug,
“Chronic” EP
__________________________________________________________________________________________________________________
4. Chronic Diseases (months to years)
• UIP, Fibrotic NSIP, Pneumoconioses,
CVD-related, Chronic HSP
Smoking (RBILD and PLCH)
2. Acute Diseases (Days to weeks)
• DAD (AIP), EP, Vasculitis/DPH, Drug, CVD
________________________________________________________________________________________________________________
History: Duration of Illness

19. Modified Liebow classification of the idiopathic interstitial
pneumonias (Katzenstein)
• Acute
• Acute interstitial pneumonia (AIP)
• Chronic
• Usual interstitial pneumonia /IPF (UIP)
• Subacute
• Nonspecific interstitial pneumonia (NSIP)
• Lymphocytic Interstitial Pneumonia (LIP)
• Cryptogenic Organizing Pneumonia (COP)
• Desquamative interstitial pneumonia (DIP)
• Respiratory bronchiolitis-associated
interstitial lung disease (RBILD)

20. Revised Classification of IIPs -2013
Major IIPs
• Idiopathic pulmonary fibrosis (idiopathic UIP)
• Idiopathic nonspecific interstitial pneumonia (iNSIP)
• Respiratory bronchiolitis-interstitial lung disease (RB-ILD)
• Desquamative interstitial pneumonia (DIP)
• Cryptogenic organizing pneumonia (COP)
• Acute interstitial pneumonia (AIP)
Rare IIPs
• Idiopathic lymphoid interstitial pneumonia (iLIP)
• Idiopathic pleuroparenchymal fibroelastosis (iPPFE)
Unclassifiable IIP

21. Physical examinations
•Bilateral basilar, crepitant velcro-like rale
•wheezing, rhonchi and coarse rales are occasionally
heard
•with advanced disease, patients may have tachypnea
and tachycardia
•At last, pulmonary hypertention and cor pulmonale
may be exist

22. Physical Findings
•RestingTachypnea
•Shallow breathing
•Dry crackles
•Digital clubbing
•Pulmonary HTN
•Non-pulmonary
findings

23. Q
• What is the Diagnosis?
a. RA
b. SLE
c. CREST
d. DM

24. What is the Diagnosis?
B.
A.

25. RADIOLOGICAL
FINDINGS -ILD

26. Q. 1
Classical HRCT findings for IPF , All true except :
a. Traction Bronchiectasis
b. Basal , Sub pleural , Central
c. Honeycombing
d. Reticular Pattern

27. Images courtesy of W. Richard Webb, MD.
Basal and peripheral reticulationReduced lung volume

28. nodular
linear

29. nodular
linear

30. Honeycomb

31. Classic IPF HRCT
Image courtesy ofW. RichardWebb, MD.
Reticular opacities Traction
bronchiectasis
Honeycombing
Basal and subpleural predominance

32. RADIOLOGICAL
FINDINGS

33. NSIP >30%
Sjogren’s Syndrome
WITH LIP
Am J Respir Crit Care Med. 2000;161:646-664. Slide courtesy of Ganesh Raghu, MD.

34. DIP

35. RB-ILD

36. Peripheral Location- Give Diagnosis?
A. B.

37. Alveolar Infiltrates / Give DD

38. DD
W (Wegner’s)
E (EP)
B (BOOP) COP
A (PAP, Aspiration)
L (Lymphoma)
L (Lipoid Pneumonia)
S (Sacroidosis)

39. Q. 2
55 year male with history of SOB and dry cough over 6 months. He
smokes occasionally . PFT Restrictive pattern with reduced capacity .
HIS HRCT show GGO with traction bronchiectasis .
Which of the following is the most likely diagnosis?
A. NSIP
B. UIP
C. RB-ILD
D. LIP

40. A2
• A

41. Q.3
In interstitial lung diseases, lung function tests most often show:
A. Reduced carbon monoxide diffusing capacity (DLCO)
B. Increased total lung capacity (TLC)
C. Airflow obstruction
D. Elevated arterial PCO2

42. A3
• A

43. PFT
•A restrictive defect :
(TLC), (FRC), (RV) ,(FVC) and (FEV1)
but usually the changes are in proportion to the
decreased lung volumes
Low DLCO

44. PFT
• A reduction (DLCO) is a common, but nonspecific finding in ILD- , the
severity of the DLCO reduction does not correlate well with disease
prognosis, unless the DLCO is less than 35 %.
• Due to effacement of the alveolar capillary units but more importantly, to
the extent of mismatching of ventilation and perfusion of the alveoli.
• In some ILDs, i.e sarcoidosis , there can be considerable reduction in lung
volumes and/or severe hypoxemia but normal or only slightly reduced
DLCO

45. Moderate to severe reduction of DLCO in the presence of
normal lung volumes in a patient with ILD suggests one
of the following:
a. Combined emphysema and ILD
b. Combined ILD and PVD
c. Pulmonary Langerhans cell histiocytosis
d. Pulmonary lymphangioleiomyomatosis
e. All of the above
Q.4

46. Q. 5
An interstitial pattern onCXR accompanied by obstructive airflow
suggestive of :
a. Sarcoidosis
b. Diffuse alveolar hemorrhage
c. Pulmonary lymphoangioliomatosis
d. Combined COPD and ILD
e. All of the above

47. An interstitial pattern on CXR accompanied by obstructive
airflow suggestive of :
• Sarcoidosis
• Lymphangioleiomyomatosis
• HP
• PLCH
• Combined COPD and ILD

48. 6MWT
•6MWT have correlated with prognosis in several studies of IPF
• Pulse oximetry desaturation to 88 during the 6MWT is
associated with a median survival of 3.21 y compared with a
median survival of 6.63 y in those who did not desaturate
below 89%.

49. Q. 6
Which of the following is true regarding Pulmonary hypertension in
ILDs ?
A.The cause of PH in ILD is likely multifactorial.
b.There is a linear correlation between PFT and PH in ILD.
C. Genetic predisposition not play a role
D. Proposed pathogenesis include presence of vaso-dilation
,angiopathy and PE
E.All are False

50. The cause of PH in ILD is likely multifactorial
The absence of a linear correlation between PFT
PH in ILD suggests that other factors may play a role ,These include the
following:
1.Vasoconstriction and vascular remodeling;
2.Perivascular fibrosis and vascular destruction;
3.Hypoxemia, both nocturnal and exertional;
4.Thrombotic angiopathy and pulmonary emboli;
5.Elevated pulmonary capillary wedge pressure resulting from peripheral
vascular occlusive disease, which has been described in both IPF and
sarcoidosis and/or diastolic dysfunction;
6.Microvascular inflammation and injury;
7.Pathobiological process (ie, vascular granulomas in sarcoidosis, PH of
Langerhan's cell histiocytosis); and
8.Genetic predisposition and varying gene expression

51. Q. 7
Which is true about BAL in ILDs ?
a. BAL is likely to be helpful in patients with a radiographic pattern
suggestive of IPF.
b. BAL does not have an established role in the assessment of ILD
progression or response to therapy
c. Consist normally of macrophages >85%, lymphocytes 10-15%,
neutrophils ≤3%, eosinophils ≤1%, epithelial ≤5%
d. High CD4 /CD8 ratio in sarcoidosis and rheumatoid lung while
reveals low ratio in HP .
e. All are true

52. Q.
Which of the following associated with neutrophilicCellular pattern
in BAL ?
A. IPF
B. HP
C. COP
D. Drug induced Pneumonitis
E. Radiation Pneumonitis

53. ROLE OF BRONCHOALVEOLAR
LAVAGE (BAL)
•The lavage fluid is sent for cell counts, cultures for
mycobacterial, viral and fungal pathogens, and cytological
analysis.
•Virtually all patients presenting with hemoptysis and
radiographic ILD should undergo BAL to confirm an alveolar
source of bleeding and identify any infectious etiologies.

54. BAL
• Normal: macrophages >85%, lymphocytes 10-15%,
neutrophils ≤3%, eosinophils ≤1%, epithelial ≤5%
•Lymphocytic (>15%): sarcoidosis, NSIP, HP,
druginduced, CTDs, radiation, COP, lymphoproliferative
disorders
• Eosinophilic (>1%): eosinophilic pneumonias,
druginduced, BM transplant, asthma, ABPA, infections,
Hodgkin
• Neutrophilic (>3%): CTDs, IPF, aspiration, infections,
bronchitis, asbestosis, ARDS/DAD Bronchoalveolar
Lavage (BAL): Cell Patterns
ATS Guideline.AJRCCM 2012

55. ILD BAL CD4 CD8T Lymphocyte

56. Q. 8
Which is true regardingVATS use for diagnosing ILDs ?
A. Low diagnostic accuracy
B. More morbidity and mortality than open lung biopsy
C. Role of BAL andTBBX is highly diagnostic in all IIP
D. Ideal biopsy include two or more surgical wedge biopsies with
areas of normal lung and samples should measure 3-5 cm in length
and 2-3 cm in depth
E. None of the above

57. Video AssistedThoracic Surgery (VATS)
ChangAC, et al. AnnThorac Surg. 2002.74;1942-1946.Rena O, et al. Eur JCardiothorac Surg. 1999;16:624-627.
• VATS is the preferred procedure for obtaining a lung biopsy
 High diagnostic accuracy
 Less morbidity and mortality than open lung biopsy
 BAL andTBBx limited to excluding other IPF mimickers
• Ideal biopsy
 Two or more surgical wedge biopsies with areas of normal lung
 Samples should measure 35 cm in length and 23 cm in depth
• Outpatient thoracoscopic lung biopsy can be a safe and effective
procedure for patients with interstitial or focal lung disease
 Diagnosis obtained in 61/62 patients
 72.5 % discharged home within 8 hours
 22.5% discharged home within 23 hours
ATS/ERSConsensus Statement. AmJ Respir Crit Care Med. 2000;161:646-664.

58. Probability of Histologic Diagnosis of Diffuse Diseases
Surgical
Biopsy
1. Granulomatous diseases
2. Malignant tumors/lymphangitic
3. DAD (any cause)
4. Certain infections
5. Alveolar proteinosis
6. Eosinophilic pneumonia
7.Vasculitis
8. Amyloidosis
9. EG/HX/PLCH
10. LAM
11. RB/RBILD/DIP
12. UIP/NSIP/LIP COP
13. Small airways disease
14. PHT and PVOD
Often
Sometimes
Rare
Transbronchial
Biopsy
Courtesy of Kevin O. Leslie, MD.

59. Some Pathology Slides

60. More Slides

62. Q. 9
• 55 y old , 30 y pack history
• Progressive dyspnea and cough
• Was working in plastic factory for
the last 30 y
• Bilateral infiltrate in chest
radiograph and cyst
• Surgical biopsy shown
What is true about the nature of the
disease ?
a.This stage carry good prognosis
b. Respond to Steroids
c. Changing his work will be
beneficial
d. Poor Prognosis

63. Q.
• 55 y old , 30 y pack history
• Progressive dyspnea and cough
• Was working in plastic factory for
the last 30 y
• Bilateral infiltrate in chest
radiograph and cyst
• Surgical biopsy shown
What is true about the nature of the
disease ?
a.This stage carry good prognosis
b. Respond to Steroids
c. Changing his work will be
beneficial
d. Poor Prognosis
• Fibrosis with honeycombing
• Architectural destruction
• Peripheral and basal distribution
• Patchy (i.e. normal and abnormal lung)
• Fibroblastic foci UIP

64. IPF

65. Average survival diagnosis of IPF is
approximately 2.5–3.5 years1 from diagnosis
Onset of symptoms
Initial visit
Kaplan-Meier plot of the survival
probability in IPF patients (n=238)2
1. Ley B et al.Am J Respir Crit Care Med 2010 October 8 2. KingTE et al. Am J Respir Crit Care Med 2001; 164: 1171-1181

66. Prevalence IN ME

67. Q .10
Which of the following conditions cause UIP pattern in
HRCT ?
a. IPF
b. Chronic HP
c. Drug Induced
d. Infections e.gTB
e. All of the above

68. Establish Diagnosis
Multi-Disciplinary Team (MDT)
Discussion
Clinical
• Symptoms
• Smoking history
• Exposures
• Features of CTD
• Examination
Investigations
• CXR
• CTThorax
• Blood tests
• Lung Function
Pathology
• Bronchoalveolar
lavage
• Surgical lung biopsy

69. Q.11
A confident diagnosis of idiopathic pulmonary fibrosis
(IPF) requires which one of the following?
A. Surgical lung biopsy
B. Usual interstitial pneumonia (UIP) pattern on lung
biopsy or HRCT.
C. Failure to respond to corticosteroid therapy
D. Evidence of disease progression

70. To Diagnose
•1. Exclude identifiable causes of ILD (e.g.,
occupational or environmental exposures, drugs
&radiation, CTDs)
•2. UIP pattern shown by: a) HRCT or b) Surgical
lung biopsy, in the absence of HRCT features
inconsistent with UIP Diagnostic Criteria for IPF
(ATS/ERS/JRS/ALAT statement. AJRCCM 2011)

71. Q. 12
73-year-old retired insulating engineer presents with a 6-m
history of increasing dyspnoea. He worked with asbestos for 2
years, 35 years ago. He has seronegative rheumatoid arthritis,
clubbing and basal crackles on chest examination.The CT scan
is shown below.
Which one of the following is the most likely diagnosis?
a. Idiopathic pulmonary fibrosis
B. Asbestosis
c. Rheumatoid lung
d. Lung adenocarcinoma
e. Bronchiectasis

72. A. Idiopathic pulmonary fibrosis
• Asbestosis is unlikely because the patient’s asbestos exposure was only
2 years in duration and his disease began more than 20 years later.The
absence of pleural plaques is evidence against asbestosis, in which
more than 95% of patients have pleural plaques demonstrable on chest
CT.
• Rheumatoid lung with interstitial fibrosis is unlikely in seronegative
disease, and clubbing is uncommon in rheumatoid pulmonary fibrosis.
• Lung adenocarcinoma remains a possible diagnosis but in this case is
less likely than IPF and the CT does not suggest the presence of a
cancer.
• Bronchiectasis is unlikely in the absence of cough and sputum
production and clubbing seldom occurs nowadays except in patients
with cystic. Bronchiectasis is not a prominent feature in the presented
CT.

73. Q 13
• 70 year-old never-smoking man, who is former office worker,
complains of a dry cough and progressive sob (NYHA class III) for
6 m. He takes 20 mg enalapril daily for hypertension. He has no
other diseases. He has not kept animals, or been exposed to dust
or fumes. Auscultation revealsVelcro rales over both lung bases.
There is no clubbing. Pulmonary function tests cannot be
performed because of impressive, possibly psychogenic,
hyperventilation.While breathing room air,ABG shows PaO2 72
mmHg, PaCO2 41 mmHg, pH 7.36 and SaO2 94%. His chest CT
image is shown below.

74. Q13
What is the initial
diagnostic test ?
a. Serology for CTD
b. VATS
c. BAL
d. TBB
e. Serum precipitating
Ab

75. Q 14
In patients with suspected idiopathic pulmonary fibrosis, the most
valuable measure is:
A. Bronchoscopy
B. ESR
C.Trial of steroids
D.Video-assisted thorascopic surgery (VATS)
E. None of the above

76. Q 15
75-year-old female is referred for dyspnoea on exertion and chronic cough
that have worsened progressively over the past 12 months. Pulmonary
function testing reveals an FVC of 72% predicted, FEV1 of 80% predicted
andTLCO of 38% predicted.The chest radiograph shows bilateral interstitial
basal infiltrates. On HRCT, bilateral reticular opacities and clustered basal
honeycombing are found. Open-lung biopsy reveals randomly distributed
foci of usual interstitial pneumonia surrounded by normal lung parenchyma.
What is the most appropriate therapy for this patient?
a. Pirfenidone
b. Bosentan
C. Acetylcysteine
d. Prednisolone/azathioprine
e. Supportive care

78. The recommendation against the use of the
following agents for the treatment of IPF
• Anticoagulation
• Imatinib
• Combination prednisone, azathioprine, and N-
acetylcysteine
• Selective endothelin receptor antagonist (ambrisentan
• Phosphodiesterase-5 inhibitor (sildenafil)
• Dual endothelin receptor antagonists (macitentan,
bosentan)
(ATS/ERS/JRS/ALAT Guideline.AJRCCM 2015)

79. Q.
InAscend and CAPACITYTrial ,What is most common reported side
effect of Perfinidone compared to placebo?
A. Vomiting
B. Insomnia
C. Rash
D. Headache
E. Nausea

80. Q
Pooling the results ofAscend , Capacity 1 and Capacity 2 ,Which is
false ?
a. Improve dyspnoea
b. Reduce mortality
c. Reduce decline in 6 MWT
d. Decrease all cause mortality
e. Reduce decline in FVC

82. Pirfenidone

83. ASCEND: Key inclusion criteria
40–80 years of age
Definite UIP on HRCT, or possible UIP on HRCT plus definite or
probable UIP on surgical lung biopsy
Extent of fibrotic changes greater than extent of emphysema on
HRCT scan
FVC ≥50% and ≤90% predicted
DLCO ≥30% and ≤90% predicted
FEV1/FVC ratio ≥0.8
6MWT distance ≥150 m
King TE Jr, et al. N Engl J Med 2014;370:2083-2092.

84. ASCENDTrial

85. TOMORROW: Annual rate of decline in
FVC
Difference between nintedanib 150 mg bid and placebo: p=0.064 vs placebo (pre-specified primary multiplicity-corrected
analysis [closed testing procedure]); p=0.014 vs placebo (pre-specified hierarchical testing procedure).
Richeldi L, et al. N Engl J Med 2011;365:1079–1087.
-0.19
-0.17
-0.21
-0.16
-0.06
-0.30
-0.25
-0.20
-0.15
-0.10
-0.05
0.00
Placebo
(n=83)
Nintedanib
50 mg qd
(n=85)
Nintedanib
50 mg bid
(n=86)
Nintedanib
100 mg bid
(n=85)
Nintedanib
150 mg bid
(n=84)
AnnualrateofFVCdecline,L/year
[Mean(SE)]

86. TOMORROW: Preservation of health-related
quality of life
5.46 4.67
2.18
1.48
-0.66
-4
-2
0
2
4
6
8
Placebo
(n=79)
Nintedanib
50 mg qd
(n=76)
Nintedanib
50 mg bid
(n=82)
Nintedanib
100 mg bid
(n=82)
Nintedanib
150 mg bid
(n=75)
ChangeinSGRQtotalscore
[Mean(SE)]
*
*p=0.007 vs placebo.
SGRQ, St George’s Respiratory Questionnaire.
Richeldi L, et al. N Engl J Med 2011;365:1079–1087.

88. All-cause mortality over 52 weeks: Pooled data
from INPULSIS®
Placebo
Nintedanib 150 mg bid
HR 0.70
(95% CI; 0.43, 1.12)
p=0.1399
Richeldi L, et al. N Engl J Med 2014;370:2071–2082.

89. Pirfenidone Experience in Saudi

90. Q 16
About GERD in patients with IPF ,Which of the
following statement correct ?
a. GERD is common (60-90%) in IPF
b. Majority (50-75%) asymptomatic.
c. May contribute to fibrosis progression, AE.
d. Some studies suggest use of GERD medications to be
an independent predictor of longer survival time in IPF,
associated with slower decline in FVC, decreasedAE.
e. All are true

91. Treatment of IPF
• “We suggest that clinicians use regular antacid
treatment for patients with IPF.”
• “ lung transplantation in patients with IPF.”
• “The committee did not make a recommendation
regarding treatment of PH in patients with IPF.”
(ATS/ERS/JRS/ALAT Guideline. AJRCCM 2015)

92. Q 17
Which of the following is true regarding NSIP?
A. NSIP is a specific disease entity.
B. Prognosis associated with NSIP and UIP are
similar.
C. NSIP is commonly associated with
connective tissue diseases.
D. NSIP commonly manifests cystic lung lesions
on HRCT.

93. NSIP EITHER
Idiopathic iNSIP OR Identifiable cause
• Connective tissue diseases
• Drugs
• Environmental/occupational exposures
• Immunocompromised hosts
• Infections
• Resolving acute lung injury

94. Q 18.

96. LIP
•idiopathic LIP
•Identifiable cause or underlying disease
Connective tissue disorders – esp. Sjögren
Immunodeficiency
Infections
Drugs/toxins
-Radiologically with GGO ,Cysts

97. Q 19
• A 50-year-old man, current smoker and HIV with CD4 500, has been complaining
of shortness of breath and non-productive cough for 5 months. He is previously
treated with antibiotics but his symptoms have failed to improve. In the
emergency department, he is noted to be hypoxic on room air and crackles on
auscultation of his lungs. His WBC 16,000; Hgb 14; Plt 300; LDH 500.He had a chest CT
which showed below
• The cell count from the bronchial alveolar lavage reveals eosinophils 5%,
lymphocytes 15%, neutrophils 15%.The transbronchial biopsy shows
inflammatory intraluminal plugs consisting of granulation tissue with fibroblasts
and myofibroblasts in connective matrix, in small airway, ducts and alveoli with
mild interstitial inflammation.There is preservation of architecture and uniform
appearance. What is your presumptive diagnosis?
a. Chronic eosinophilic pneumonia
b. Cryptogenic organizing pneumonia
c. Desquamative interstitial pneumonia
d. Pulmonary Oedema
e. Acute eosinophilic pneumonia

98. OP
Idiopathic = cryptogenic OP= (idiopathic BOOP) OR
Identifiable cause
• Connective tissue diseases/vasculitides
• Drugs, toxins, radiation
• Infections
• Hypersensitivity pneumonitis
• Aspiration
• Chronic eosinophilic pneumonia
• Diffuse alveolar damage
• Hematologic diseases, allograft transplants

99. Q 20.
The patient has been discharged on prednisone 20 mg PO daily for 4 weeks.
He has not been compliance to his medications and he comes in
complaining of fatigue and shortness of breath. He is noted to have oxygen
saturation of 95% on room air and afebrile. Repeat radiographs show new
central sparing infiltrates on the left lung.Cultures have been obtained
which has been negative. What would be the next appropriate step?
a. Prednisone 20 mg PO daily
b. Solumedrol 1 g IV daily for 3 days
c. Piperacillin–Tazobactam 3.375 mg IV every 6 hours with vancomycin 1 g
IV every 12 hours
d. Cellcept 1,000 mg PO every 12 hours
e. Amphotericin B

100. A.
• Answer: A. Prednisone 20 mg PO daily
• The patient appears to have a relapse, which manifests as worsening
symptoms with reoccurrence of prior or new infiltrates.They are
common during steroid taper.
• Predictors of relapse include delayed treatment and mild increases
with alkaline phosphatase and gammaglutamyltransferase.
• Proposed taper of medications include prednisone 0.75 mg/kg/day for 4
weeks; followed by 0.5 mg/kg/day for 4 weeks, and then 20 mg daily
for 4 weeks, 10 mg for 6 weeks, and 5 mg daily for 6 weeks. If relapse
occur while dose <20 mg daily, increase dose to 20 mg daily and slowly
taper accordingly.
• Treatment of COP includes steroids from 0.75 to 1.5 mg/kg/day with
usual duration of up to 1 year.

101. Q.
• 25 y old lady not known to have
any medical illness , history of
recurrent abortions, presented
with dyspnea , admitted to ICU
CT shown - Bronchoscopy done
Which is true about this
condition?
a. Good prognosis
b. Bronchoscopy will not help in
diagnosis
c. Need high dose steroids
d. Complements will be normal

102. DAD
• Histologic pattern of ALI characterized by diffuse involvement with
edema, hyaline membranes, and acute interstitial inflammation
(exudative phase) evolving to loose organizing fibrosis and type II
pneumocyte hyperplasia (organizing phase).
• HRCT: Diffuse ground-glass and/or consolidative opacities
• Management: depends on the clinical context, corticosteroids
commonly used when non-infectious
• Prognosis: high short-term mortality

103. Acute Interstitial Pneumonia
Idiopathic (“Hamman Rich syndrome”)
Identifiable cause or underlying disease:
• Infections
•Toxic inhalants
• Drugs
• CTDs/vasculitides/alveolar hemorrhage
• Acute radiation reaction
• Acute exacerbation in ILDs
Histo – Septal thickening
and proliferation of spindle cells

104. Q 23
• 40 y old female teacher , Hypothyroidism and hypoadrenalism on
treatment, presented with shortness of breath ,cough a typical chest
pain and haemoptysis- minimal amount. History of Raynaud's and
generalized fatigability .No fever . No other systemic symptoms
• Looks Sick , Fully Oriented , BP 90/60 P130 Afebrile Spo2 90 %
• CVS S1, loud S2 with pansystolic murmur , Chest bilateral crackle and
L L oedema
• Leukopenia ,Mild elevation of transaminase
• CXR show Cardiomegaly and bilateral lung infiltrate
• Previous CT one y back :Interstitial lower lobes infiltrates with traction
bronchiectasis
• Echo Severe Pulmonary HTN 90 and dilated RA ,RV normal LV
• RHC ,ANA Positive ,all other autoimmune profile were negative

105. Q 23.
What is suggested treatment ?
A. Sildenafil ,Bosentan and Pirfenidone
B. illoprost , Bosentan and steroids
C. Lasix , Steroids ,Sildenafil and illoprost
D. Bosentan , illoprost ,Lasix and Steroids
E. Steroids only

106. Autoimmune-features ILD
(Interstitial Pneumonia with
Autoimmune Features (IPAF) )
.
Classification criteria:
• Presence of an interstitial pneumonia (by HRCT or SLBx) •
Exclusion of alternative etiologies and,
• Does not meet criteria of a defined connective tissue and, disease
and,
• At least one feature from at least 2/3 domains: clinical (e.g.,
Raynaud’s), serologic, morphologic (HRCT or SLBx features)
ERS/ATS statement. ERJ 2015

107. RARE ILDS

108. Q. 24
Which is true about Familial Interstitial Pneumonia/ Familial Pulmonary
Fibrosis?
a. >20 % relatives
b. ~40% of interstitial pneumonia / pulmonary fibrosis
c. ~25% of these familial cases have identifiable mutations
d. Specific HRCT and histopathologic pattern
e. None of the above

109. Familial Interstitial Pneumonia/
Familial Pulmonary Fibrosis
Evolving recommendations regarding
genetic testing for those with early onset
(<50 yr), positive family history, suspicious
extrapulmonary features
Borie et al. Eur Respir Rev 2017

110. Q 25
Which one of the following interstitial lung diseases is related to smoking?
A. LAM
B. Desquamative interstitial pneumonia
C. UIP
D. NSIP
E. DAD

111. Q
Which one of the following interstitial lung diseases is
NOT related to smoking?
A. Acute eosinophilic pneumonia
B. Desquamative interstitial pneumonia
C. Respiratory bronchiolitis –ILD
D. Hypersensitivity Pneumonitis
E. IPF

112. Smoking-related ILDs
1. Respiratory bronchiolitis-associated ILD (RB-ILD)
2. Desquamative interstitial pneumonia (DIP)
3. Pulmonary Langerhans cell histiocytosis (PLCH)
4. Acute eosinophilic pneumonia (AEP)
5. Combined pulmonary fibrosis and emphysema
(CPFE)
(Vassallo et al. Clin Chest Med 2012)

113. Q. 26
• 40 y old ,nurse ,15y pack history
• Progressive SOB
• No fever or haemoptysis
• RR 27 SPO2 93%RA
• Chest bilateral crepitation
• Normal Labs including ESR & ANA
• CT Shown
• Best treatment
a. Observation & Stop Smoking
b. Stop Smoking & Steriods
c. Steriods and azathioprin
d. Perfinedone

114. RB-ILD
• numerous pigmented macrophages
• Relatively uniform appearance
• Most are smoking-related
• HRCT: GGOs ± reticular opacities;
sometimes cysts, or and vague
nodules
• Management: smoking cessation,
corticosteroids
• Prognosis: generally good, up to 30%
mortality

115. Q 27
• 44 y old came with dyspnea on exertion and cough. Has been told
she has emphysema. Attempt of tobacco cessation failed.
• Physical examination reveals crackles
• Her radiographic ,pathology shown
• Which of the following is most likely ?
a. COPD
b. Goodpasture Syndrome with diffuse pulmonary haemorrhage
c. Pulmonary Langerhans histiocytosis
d. DIP

116. Q.

117. Q.

118. Combined Pulmonary Fibrosis and
Emphysema (CPFE)
•Upper lung emphysema and lower lung fibrosis
•Typically male smokers
•Relatively preserved spirometry and lung volumes with
low DLCO
•Increased incidence of pulmonary hypertension –
associated with increased mortality
•“Pulmonary fibrosis” includes UIP, NSIP, RB-ILD, DIP,
etc.

119. DPLD II

120. LearningTargets -II-
To elaborate the diagnosis to other
ILDs including clinical , radiological
findings and management lines.

121. DPLD II
• CTD-associated ILDs
•Diffuse Cystic Lung Diseases
• Others

122. Q 28
Respiratory manifestations in CTD is characterized by
which one of the following?
A. Obstructive lung disease is not seen.
B. Lung biopsy is usually needed.
C. SLE involves the lung more often than other CTDs.
D. Acute exacerbation can occur in patients with CTD-
ILDs.

123. Rheumatic Disease ILD
•RA 20 - 30 %
•PM/DM 20 - 50 %
More common with anti-synthetase antibodies
•Systemic sclerosis 45 % (“clinically significant”)
More common in diffuse disease; topoisomerase-1
antibodies
•SLE 2 - 8 %
Usually in patients with multisystem disease
•MCTD 20 – 60 %
•Sjögren to 25 %
(Castelino et al. Arthritis ResTher 2010)

124. Q 29
45 year old man known case of PM/DM , presented with three
weeks history of dyspnea on exertion ,progressive and
associated with dry cough ,weight loss and loss of appetite He
has a history of Raynaud’s. Physical Examination show ankle
joint swelling, HRCT show bilateral interstitial infiltrate diffuse
predominantly upper lobes with traction bronchiectasis ILD.
What is true about this disease?
a. 2-5 % of PMDM patients will have it
b. UIP pattern is the commonest to be found in HRCT
c. Anti –jo antibodies positive
d. Obstructive ventilatory defect in PFT
e. None of the above

125. AntiSythestase Syndrome
•Subset (16-30%) of patients with PM/DM
• Characterized by relatively acute onset, constitutional
symptoms, Raynaud’s, “mechanic’s hands”, arthritis,
ILD.
•anti-Jo-1 (anti-histidyl–tRNA synthetase)
• Associated with ↑ risk of ILD Usually NSIP > UIP > OP;
sometimes LIP, DAD, etc
•Can be more refractory to treat than other PM/DM-
associated ILD

126. Q 30
• 30 y lady with SLE , Co progressive SOB 3 m , no other respiratory
symptoms . OnWarfarin for previous DVT. Examination is normal
• PFT FEV1 55% ,FVC 58% FEV1FVC 0.78 TLC 68%
RV 100%
DLCO 77% adjusted to alveolar volume 100%
• CXR small lung volume without lung infiltrates or effusion
What is the next to do ?
a. Echo
b. VQ scan
c. Maximum Inspiratory and Expiratory Pressure
d. Bronchoscopy

127. A.
• MIP ,MEP
• To assess muscle weakness ?myositis or phrenic n palsy
• Dx shrinking lung syndrome
• Tx steroid , theophylline , beta agonist and immunosuppression

128. Q 31
• 66 y old lady with Systemic sclerosis , Raynaud's
• Never smoker , work as a secretary
• Examination reveals Spo2 93% , diffuse skin thickening and
telangactasia upper limb digitals
• FVC 60%
• HRCT bilateral basal ground glass opacities
What is the best treatment ?
a. Cyclophosamide
b. Cyclosporine
c. Steroids
d. Azathioprine

129. A.
• B.
• RCT
• 49% improved with cyclophosphamide Vs 26%
Clin Rheumatol. 2006 Mar;25(2):205-12. Epub 2005 Oct 14.
A randomized unblinded trial of cyclophosphamide versus azathioprine in
the treatment of systemic sclerosis.
Nadashkevich O1, Davis P, Fritzler M, Kovalenko W

130. What is the Diagnosis?

131. Diffuse Cystic Lung Disease
• Cyst = a round parenchymal lucency with a well defined
thin-wall (<2 mm), usually contain air • Focal/multifocal
vs diffuse
•Cavity = a lucency within pulmonary consolidation, a
mass, or a nodule. (thick wall)
•Emphysema = focal areas of low attenuation without
visible walls
Fleischner Society, 2008

132. Mechanism of Cyst Formation
•Elastolysis mediated by matrix metalloproteinases
MMPs) – LAM, PLCH
• Destruction of the bronchial wall and progressive
luminal dilatation – PLCH
•Airway narrowing and check valve mechanism – LIP,
amyloidosis
• Hamartoma-like cystic alveolar formation – BHD
• Cavitation of nodule (inflammatory/infectious,
neoplastic)

133. Q 32
Which of the following radiographic features is
least likely to be found in Langerhans’ cell
histiocytosis of the lung?
a.Nodules ranging in size up to 10 mm
b.Bilateral reticulonodular opacities
c.Pneumothorax
d.Pleural effusion
e.Honeycomb lung

135. Langerhans’ cell histiocytosis
(LCH)
d. Pleural effusion
1. Early , Centrilobular nodules (2–10 mm in size) and
2. Reticular and nodular opacities with a predominantly bilateral
symmetric upper- to mid-lung distribution.
3. Late ,Cysts develop and become the dominant imaging finding.
Cysts vary in size but usually are smaller than 1 cm may result
in bullous formation, which then predisposes the patient to
recurrent spontaneous pneumothorax. In advanced LCH,
honeycomb changes can occur.
4. Pleural effusions are rare.
Zaveri et al. 2014

136. Pulmonary Langerhans cell
Histiocytosis
Diagnosis
• BAL: ³5% CD1a cells
• Lung biopsy: bronchoscopic or
VATS
Management
• Stop smoking
• Sometimes, 2-
chlorodeoxyadenosine (2-CdA,
cladribine) •
B-raf inhibitors (e.g.,
vemurafenib)?
• Pulmonary hypertension
• PrognosisVariable, risk of
malignancy

137. Q 33
• 56 y F , smoker
• Secretary
• Progressive SOB for last 2 y
• Childhood asthma with FH of asthma
• Not on any medications
• Chest examination reduced breath
sounds -No skin lesions
• Previous- 1 y - CXR reported as
increase lung volume.
• FVC 79% FEV1 46% DLCO 60
What is the most likely diagnosis?
a.LAM
b.PLCH
c. Birt-Hogg-Dude syndrome
d.LIP

138. LAM
• Proliferation of abnormal
smooth muscle cells (LAM cells
– HMB-45+)
• Sporadic andTSC-related
forms; caused by mutation in
theTSC genes
• Mostly women
• High risk of pneumothorax: 60-
80%
• PFT:Typically obstructive

139. Diagnosis and Management -
LAM
CT chest findings plus any one or more of the following:
• Biopsy of lung or extrapulmonary LAM
• Renal angiomyolipomas
• Chylothorax (seen in 20-40% during course)
•Tuberous sclerosis complex (TSC)
• High serumVEGF-D level, >800 pg/mL Management

140. What is the name of this radiological
findings?

141. PAP
• Most are 20-60 y of age (median ~40)
• Nonspecific presentation: insidious onset DOE, cough - sometimes
asymptomatic
• Fever, fatigue, weight loss, chest pain, haemoptysis
• Inspiratory crackles in 20- 50%
• Serum LDH, surfactant A and D, KL-6 (mucinlike glycoprotein) -
common, nonspecific
• Anti-GM-CSF antibodies detectable in serum & BAL fluid in most
cases of acquired PAP
• PFTs: a restrictive defect, reduced DLCO
• Whole Lung Lavage

142. What is the diagnosis?

143. Radiation-induced Lung Injury
After radiation therapy in patients with lung cancer and mediastinal
lymphoma, radiologic abnormalities occur in 60-90%; 5-15%
symptomatic.
Radiation pneumonitis - symptoms 4-12 weeks after irradiation
Radiation fibrosis - 6-12 months after irradiation
• Imaging: radiographic abnormalities confined to radiation field
with “straight line” effect
• Management: symptomatic pneumonitis, prednisone 40-60 mg/d x
2 wk, then taper over 4-12 wk

144. THANKYOU