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ABIM Pulmonary Boards - Jackie > Diffuse parenchymal lung disease > Flashcards

Diffuse parenchymal lung disease Flashcards

1
Q

Lymphocytic BAL ddx

A

Sarcoid, NSIP, HP, drug induced, CTD, radiation, COP, lymphoproliferative disorder

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2
Q

Eosinophilic predominant BAL ddx

A

Eos PNA, drug-induced, BM transplant, asthma, ABPA, infections, hodgkin

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3
Q

UIP Histology

A

Peripheral patchy fibrosis, Fibroblastic foci, honeycomb, +/- inflammation

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4
Q

UIP HRCT

A

Subpl/basal, reticular, traction bronchiectasis +/- honeycomb

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5
Q

Causes of UIP

A

CTDs/vasculitis, cHP, asbestosis, drugs, genetic disorders

if idiopathic =IPF

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6
Q

NSIP histology

A

Homogenous, chronic intersitital inflam/fibrosis

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7
Q

NSIP HRCT

A

GGOs/reticulations +/- subpleural sparing
Homogenous

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8
Q

NSIP causes

A

**CTDs
drugs, inhalation injury, ICH, infection

idiopathic= iNSIP

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9
Q

OP histology

A

Patchy, granulation tissue plugs

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10
Q

OP HCRT

A

Patchy consolidation/ GGOs

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11
Q

OP causes

A

CTDs/Vasculitis, drugs, infections, XRT, aspiration, eos PNA, HP, DAD

idiopathic= COP

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12
Q

RB-ILD/DIP Histology

A

Pigmented macs, bronchiolocentric or diffuse

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13
Q

RB-ILD/DP HRCT

A

Patchy to diffuse GGOs, vague nodules

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14
Q

Diffuse alveolar damage histology

A

Hyaline membranes
granulation tissue
organizing, fibroproliferative

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15
Q

Diffuse alveolar damage HRCT

A

Diffuse consolidation/ GGOs

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16
Q

Causes of diffuse alveolar damage

A

Acute time course

Infections, toxins, drugs, XRT, AE-ILD (causes of ARDS)

Idiopathic= AIP

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17
Q

LIP histology

A

Lymphoid, plasma cells

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18
Q

Pleuroparenchymal fibroelastosis histology

A

Pleural/subpleural fibroelastosis, septal elastosis

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19
Q

Pleuroparenchymal fibroelastosis HRCT

A

Upper/subpl fibrosis +/- pneumomed/ptx

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20
Q

Causes of Pleuroparenchymal fibroelastosis

A

Chemo, stem cell transplant, recurrent infection, familial PF

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21
Q
A

NSIP
Homogenous, chronic intersitital inflam/fibrosis
diffuse insterstitial thickening

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22
Q
A

OP histo
Patchy, granulation tissue plugs

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23
Q
A

RBILD path

Pigment laden macrophages

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24
Q
A

LIP
lymphoid

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25
Q
A

Diffuse alveolar damage

**Hyaline membranes
granulation tissue
organizing, fibroproliferative

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26
Q

factors possibly contributing to development of IPF

A

Genetic - MUC5B, TERT/TERC, TOLLIP
Short elomere syndrome
Cellular senescence
Microbiome
GERD

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27
Q

Effect of antifibrotic for IPF

A

Reduces rate of FVC decline

MAY: improve QOL
Reduce rate of hospitalization and AE +/- on prolonging life

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28
Q

Antifibrotics for IPF

A

Pirfenidone
Nintedanib

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29
Q

Treatment of PH in ILD

A

Treprostinil

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30
Q

Indications for Nintedanib

A

IPF
SSc-ILD
Chronic fibrosing ILD with progression

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31
Q

Markers of langerhans

A

CD1a+ (BAL)
Langerin+ (CD207)
S100 (stain)

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32
Q

Mutations in langerhans

A

BRAF-V600E in 50% pts

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33
Q

HRCT in langerhans

A

Irregular cysts, basilar sparing
Nodules +/- cavitation, reticular opacities

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34
Q

Langerhans management

A

Stop smokin g
B-raf inhibitors (vemurafenib)
Cobimetinib
2-CdA (cladribine)
Lung transplant

35
Q

PFTs in combined pulmonary fibrosis and emphysema

A

Relatively preserved spiro and lung volumes with low DLCO

36
Q

Fibrotic disease pattern in RA

A

UIP >NSIP

37
Q

Lung dz in sjogrens

A

ILD- NSIP>LIP>UIP
Amyloid
airway- xerotrachea
Pleuritis
PH
Lymphoma (MALToma)

38
Q

Antisynthetase syndrome pulm dz

A

PM/DM- anti-jo-1 and aminoacyl-tRNA synthetase autoab

increased risk of ILD

39
Q

Risk factors for ILD in SSc

A

diffuse SSC
+anti-SCL70 ab, neg anti-centromere

40
Q

Tx scleroderma ILD

A

**Mycophenolate - less toxic than CFM
Tocilizumab- stable FVC
Cyclophosphamide- stabilize FVC
Nintedanib- slower decline FVC

41
Q

Treatment RA ILD

A

Steroids
MMF,AZA

42
Q

tx Sjogren ILD

A

Steroids
MMF, AZA

43
Q

SLE ILD tx

A

Steroids
AZA
MMF

44
Q

Clinical features sarcoid

A

presents 20-60yo
wheezing

Uncommon: crackles and clubbing

45
Q

Dx sarcoid

A

Clinical features
Presence of noncaseating granulomas via biopsy
BAL

46
Q

When do you NOT need biopsy for sarcoid dx?

A
  • Asymptomatic bilateral hilar LAD
  • Lofgrens syndrome (fever, e nodosum, arthralgias, ,BHL)
  • Lupus pernio
  • Heerfordt’s (parotid swelling, uveitis, bells palsy)
47
Q

BAL findings for sarcoid

A

Lymphocytic
CD4/CD8 >4

48
Q

When to treat pulm sarcoid?

A

Stage II or III with mod to severe PFT impairment or progression

Also treat if extrapulm
including hyperCa

49
Q

Tx for sarcoid

A

Pred 20-40mg/d for 4-6 wk and taper 6-12 mo

if stage I-II consider ICS
Cardiac: 40-60mg/d

MTX

50
Q

Cause and tx of Granulomatous Lymphocytic ILD

A

CVID, immunodef

Necrotizing granulomas, folicular bronchiolitis

tx, IVIG, steroids, immunosupress/RTX

51
Q

Cytokine specific to eos

A

IL-5

52
Q

HMB-45+ associated with what dz?

A

LAM

53
Q

Dx LAM

A

CT with diffuse cysts
AND
LAM on biopsy, renal AML, chylothorax, TSC OR Increased serum VEGF-D

54
Q

Tx LAM

A

Sirolimus
transplant

55
Q

HRCT Langerhans

A

Irregular cysts
Basilar sparing
Nodules +/- cavitation
reticular opacities

56
Q

Mutation in Birt-Hogg-Dube syndrome

A

BHD (FLCN) gene (chromosome 17p11.2); tumor suppressor protein, folliculin

57
Q

Clinical presentation Birt-Hogg-Dube

A

cystic lung dz
PTX
Skin lesions (fibrofolliculomas)
Renal tumors

58
Q

HRCT for Birt-Hogg-Dube

A

Cysts of varying sizes and shapes, more lower and medial lobes

59
Q

HRCT follicular bronchiolitis

A

Small nodules, patchy ggo, sometimes cysts

tx: underlying dz tx, bronchodilators, steroids, erythromycin

60
Q

Heritable disorders that cause cysts

A

NF1
Marfans (upper lung bullae)

61
Q

Lab findings for autoimmune PAP

A

Increased serum LDH
Surfactant A and D
KL-6

Anti-GM-CSF Ab in serum and BAL

62
Q

PFT autoimmune PAP

A

Restrictive and reduced DLCO

63
Q

autoimmune PAP finding on BAL

A

Milky effluent BAL

64
Q

Tx PAP

A

full lung lavage
GMS-CSF SQ or neb
RTX
Lung txp

65
Q

SLC34A2 gene mutation with calcific micronodular infiltrate on CT

A

Pulmonary alveolar microlithiasis

66
Q

Cause of Erdheim-chester disease

A

histiocytic disorder, multi organ Malig of myeloid progenitor cells

Somatic BRAF mutation

67
Q

Presentation of Erdheim-chester disease

A

bone pain, cardiac, CNS, hairy kidney, pleuro-pulm

68
Q

Dx Erdheim-chester disease

A

Histo: foamy histiocytes (CD1a-), MNG histiocytes (Touton cells), fibrosis

69
Q

management Erdheim-chester disease

A

BRAF kinase inhibitor (vemurafenib)
MEK inhibitor (cobimetinib)
Cladribine
radiation

70
Q

Chyloptysis is a sign of…

A

Diffuse pulmonary lymphangiomatosis

71
Q

Diffuse pulmonary lymphangiomatosis HRCT

A

diffuse interlobular septal thickening, patchy ggo, medist infil. pleural thick.

72
Q

Timing of radiation pneumonitis

A

4-12 weeks after irradiation

73
Q

Timing of radiation fibrosis

A

6-12 months after irradiation

imaging confined to rad field (straight line effect)

74
Q

Timing of radiation induced organizing pneumonia (BOOP)

A

1-12 mo after therapy (usually for breast cancer

75
Q

Imaging findings radiation induced organizing pneumonia (BOOP)

A

patchy alveolar infiltrates OUTSIDE radiation field

76
Q

Etiologies of constrictive bronchiolitis/obliterative/BO

A

Allograft recipient
post-infectious
CTD – RA
Inhalation injury
Drugs/toxins
DIPNECH
Cryptogenic

77
Q

HRCT constrictive bronchiolitis

A

moasicism with patchy air trapping, scattered bronchiectasis

CXR with hyperinflation

78
Q

Characteristics of DIPNECH

A

Middle aged women with respiratory sx and obstruction on PFT

Mosaic pattern and small nodules CT

Can lead to carcinoid

79
Q

Differential for cavitary lung lesions

A

Neoplasia
- Mets (squamous cell common)
- multifocal bronchogenic adenocarcinoma
- MALT

Infection
Systemic disease

80
Q

Infectious causes of cavitary lung lesions

A

Septic emboli
lung abscess
Mycobacterium TB
NTMB
fungal infections

81
Q

Systemic disease and other causes of cavitary lung lesions (not infectious or malignant)

A

GPA
RA nodules
Sarcoidosis
Langerhans cell histiocytosis (early)
pulm juvenile papillomatosis (rare)
COP/BOOP (rare)

82
Q

CT features of OP

A

extensive airspace disease
patchy lower lung zone–predominant consolidation
ground-glass opacities with a subpleural and/or peribronchovascular distribution

83
Q

Treatment for SSc ILD

A

MMF

84
Q
A

ABIM Pulmonary Boards - Jackie (11 decks)

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