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GBA - Y4 > Harrison's 287- Interstitial Lung Diseases > Flashcards

Harrison's 287- Interstitial Lung Diseases Flashcards

1
Q

What Interstitial Lung Diseases cause Alveolar Inflammation & Fibrosis due to Unknown Cause?

A

IPF
AIP
COP
SLE/RA and other CTD

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2
Q

What Interstitial Lung Diseases cause Alveolar Inflammation & Fibrosis due to Known own Cause?

A

Asbestosis
Noxious Gases
Radiation Pneumonitis
Aspirations

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3
Q

What Interstitial Lung Diseases cause Granulomas in the Interstitium & Vessels due to Known Cause?

A

Hypersensitivity Pneumonitis - Organic Dust, Silicosis, Berylliosis

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4
Q

What Interstitial Lung Diseases cause Granulomas in the Interstitium & Vessels due to Unknown Cause?

A

Sarcoidosis
Granulomatosis with Polyangiitis (Wegner’s)
Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss)

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5
Q

What are the non specific symptoms of ILDs?

A

Tachycardia
Basilar Crackles Bilaterally
Cyanosis
Clubbing

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6
Q

What are the laboratory Findings of ILDs?

A

ANA/RF (even in non-CTDs)
LDH↑
ACE ↑
If Vasculitis - ANCA / Anti-GBM

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7
Q

What are the Blood Gases changes of ILDs?

A

While In Rest = Normal

Hypoxia (V/Q Mismatch↑) or Respiratory Alkalosis are possible

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8
Q

What Imaging Method is the most adequate to diagnose ILDs?

A

CT
Reticular Patterns Identified better than X-ray
(Biopsy is a Must for Diagnosis)

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9
Q

Changes in some PFT in ILDs :
TLC
FRC
RV

A

Changes in some PFT in ILDs :
TLC↓
FRC↓
RV↓

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10
Q

Changes in some PFT in ILDs :
FEV1
FVC
FEV1/FVC ratio (Tiffeneau-Pinelli index)

A

Changes in some PFT in ILDs :
FEV1↓
FVC↓
FEV1/FVC ratio ↑ or Normal

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11
Q

Changes in some PFT in ILDs :
DLCO
A-a Gradient

A

Changes in some PFT in ILDs :
DLCO↓
A-a Gradient↑

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12
Q

64 year old man, Dyspnea, Unproductive Cough, Reticular Opacities with Honeycombing. Arterial Hypoxia that worsens with Exertion. Peripheral Subpleural Fibrosis on Biopsy.
What is the most likely diagnosis? what is the treatment? What is the Prognosis?

A

Idiopathic Pulmonary Fibrosis
No effective treatment - Thalidomide for Symptomatic cough relief
Poor Prognosis

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13
Q

28 year old women, Non Smoker, with Dyspnea, Unproductive Cough, Sand-Glass Patten Opacities on subpleural lower lobes in Imaging. ANA Positive. On Biopsy - Uniform Fibrosis Patterns.
What is the most likely diagnosis? what is the treatment? What is the Prognosis?

A

Nonspecific Interstitial Pneumonia
Steroids with Azathioprine or Mycophenolate Mofetil
Good Prognosis

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14
Q

45 year old women, with ARDS like patterns on Labs with Consolidations bilaterally. Prodrome of 7 days with fever, cough, dyspnea and malsie.
What is the most likely diagnosis? what is the treatment? What is the Prognosis?

A

Acute Interstitial Pneumonia
in Many cases - Mechanical Ventilation & Supportive
60% Mortality

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15
Q

56 year old women, subacute pneumonia symptoms.Opacities on periphery & subpleural lower lobes. Granulation tissue on small airways with Inflammatory Alveoli.
What is the most likely diagnosis? what is the treatment? What is the Prognosis?

A

Cryptogenic Organizing Pneumonia

Steroid allow recovery in 2/3 of patients

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16
Q

What possible drugs cause ILDs with Fibrosis?

A 
Bleomycin
Busulfan 
Amiodarone
Methotrexate
Nitrofurantoin
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17
Q

ILD - CTD Etiologies?

A 
RA
SLE
Progressive Systemic Sclerosis
Polymyositis & Dermatomyositis
Sjogren's Syndrome
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18
Q

ILD - secondary to Smoking ?

A

Desquamative Interstitial Pneumonia
Respiratory Bronchitis-Associated ILD
Pulmonary Langerhans cell Histiocytosis

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19
Q

What type of HSN is present in Hypersensitivity Pneumonitis?

A

Mixed Type III/IV Hypersensitivity

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20
Q

Who is common to have Hypersensitivity Pneumonitis?

A

Farmers or Birds Fanciers

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21
Q

Caplan Syndrome

A

Rheumatoid Arthritis + Pneumoconiosis with Intrapulmonary Nodules

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22
Q

What kind of Pneumoconiosis has granulomas?

A

Berylliosis

23
Q

What kind of Pneumoconiosis appears on upper lobes?

A

Asbestosis

24
Q

Why does Silicosis Predisposes for TB infections?

A

Defective Phagocytosis by Macrophages

& Enhanced V/Q Mismatch

25
Q

How would the following change in IPF:
Pneumocytes Type 1
Pneumocytes Type 2
Fibroblasts

A

IPF:
Pneumocytes Type 1↓
Pneumocytes Type 2↑
Fibroblasts↑

26
Q

What are the histological markings of Asbestosis?

A

Asbestosis

Ferruginous Bodies

27
Q

Bilateral Interstitial Opacities on X Ray with BAL Showing more than 50% Lymphocytes

A

Hypersensitivity Pneumonitis

28
Q

Pleural Plaques are common in (Pneumoconiosis type)

A

Asbestosis

29
Q

Asbestosis leads to higher risk of Bronchogenic Carcinoma / Mesothelioma?

A

Asbestosis leads to higher risk of Bronchogenic Carcinoma ! (Although Mesothelioma is associated with Asbestosis )

30
Q

RA related Restrictive Lung Disease is more common in - Men/Women?

31
Q

What ILD is the most common to present with Clubbing?

32
Q

What ILD is the most common in Non-Smoker young Women?

33
Q

What are the HRCT classical Findings in IPF?

A

Honeycombing
Ground Glass
Traction Bronchiectasis

34
Q

What type of Scleroderma is most common with ILD?

A

Systemic Sclerosis with Diffuse Scleroderma

35
Q

What is the other name for Hamman-rich Syndrome? What is the Mortality rate? & Exacerbation Mortality rate?

A

Acute Interstitial Pneumonia
Mortality rate > 50%
Exacerbation Mortality rate > 85%

36
Q

What drugs can slow the decline of IPF patients?

A

Anti-Fibrotic Agents

Pirfenidone & Nintedanib

37
Q

Mesotheliomas will usually stain positive for

A

Cytokeratin

38
Q

Mesotheliomas will may stain positive for

A

Calretinin

39
Q

What are the common cells that allow for Macrophages build up and granuloma in sarcoidosis of lungs? Cytokines relevant for this?

A

Th1, IL2, IFN-gamma

40
Q

What ILD involves Surfactant Debris accumulation in the airways as Lamellar Bodies? What cells are defective?

A

PAP - Pulmonary Alveolar Proteinosis

Alveolar Macrophages defected

41
Q

Birefringent Particles with Densed Whorled Collagenous nodules along with dust laden macrophages - this is common for?

42
Q

The _____ Promoter Region Variant is common in 40% of IPF Patients and Includes Prodromal Imaging findings type of disease.

A

The MUC5B Promoter Region Variant is common in 40% of IPF Patients and Includes Prodromal Imaging findings type of disease.

43
Q

What type of Dermatomyositis/Polymyositis Patients will have in 45% an ILD?

A

Dermatomyositis/Polymyositis with Anti-synthetase

44
Q

What type of Dermatomyositis/Polymyositis Patients will have a rapidly progressing onset of ILD?

A

Dermatomyositis/Polymyositis with Anti-Jo

45
Q

What is the name of the Histopathological Pattern of Hamman Rich Syndrome?

A

Diffuse Alveolar Damage (DAD)

46
Q

What is the name of the Smoking-Related ILD?

Most associated of all

A

Desquamative Interstitial Pneumonia (DIP)

Respiratory Bronchiolitis ILD (RB-ILD)

47
Q

Pulmonary Lymphangioleiomyomatosis -LAM:

We should suspect in a premenopausal woman with on of these 3:

A
  1. Emphysema.
  2. Reccurent Pneumothorax
  3. Chylothorax
48
Q

Proliferation of interstitial lung smooth muscle & Cysts are common histological findings in

A

Pulmonary Lymphangioleiomyomatosis -LAM

49
Q

What medication is used in Pulmonary Lymphangioleiomyomatosis -LAM for quality of life and symptomatic treatment?

A

Sirolimus (Rapamycin)

mTOR inhibition causes less IL2 sensitivity in Lymphocytes

50
Q

In What ILD there is a 67% of Hemoptysis in the diagnostic workup?

A

Diffuse alveolar hemorrhage - DAH

51
Q

What pneumoconiosis will have an eggshell pattern on CXR?

52
Q

What two AD diseases can have a older age onset together with ILDs?

A

Tuberous sclerosis

Neurofibromatosis

53
Q

What kind of Acid-Base disorder we can detect in late ILD?

A

Respiratory Alkalosis

GBA - Y4 (12 decks)

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