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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
Diffuse alveolar damage **Hyaline membranes granulation tissue organizing, fibroproliferative
26
factors possibly contributing to development of IPF
Genetic - MUC5B, TERT/TERC, TOLLIP Short elomere syndrome Cellular senescence Microbiome GERD
27
Effect of antifibrotic for IPF
Reduces rate of FVC decline MAY: improve QOL Reduce rate of hospitalization and AE +/- on prolonging life
28
Antifibrotics for IPF
Pirfenidone Nintedanib
29
Treatment of PH in ILD
Treprostinil
30
Indications for Nintedanib
IPF SSc-ILD Chronic fibrosing ILD with progression
31
Markers of langerhans
CD1a+ (BAL) Langerin+ (CD207) S100 (stain)
32
Mutations in langerhans
BRAF-V600E in 50% pts
33
HRCT in langerhans
Irregular cysts, basilar sparing Nodules +/- cavitation, reticular opacities
34
Langerhans management
Stop smokin g B-raf inhibitors (vemurafenib) Cobimetinib 2-CdA (cladribine) Lung transplant
35
PFTs in combined pulmonary fibrosis and emphysema
Relatively preserved spiro and lung volumes with low DLCO
36
Fibrotic disease pattern in RA
UIP >NSIP
37
Lung dz in sjogrens
ILD- NSIP>LIP>UIP Amyloid airway- xerotrachea Pleuritis PH Lymphoma (MALToma)
38
Antisynthetase syndrome pulm dz
PM/DM- anti-jo-1 and aminoacyl-tRNA synthetase autoab increased risk of ILD
39
Risk factors for ILD in SSc
diffuse SSC +anti-SCL70 ab, neg anti-centromere
40
Tx scleroderma ILD
**Mycophenolate - less toxic than CFM Tocilizumab- stable FVC Cyclophosphamide- stabilize FVC Nintedanib- slower decline FVC
41
Treatment RA ILD
Steroids MMF,AZA
42
tx Sjogren ILD
Steroids MMF, AZA
43
SLE ILD tx
Steroids AZA MMF
44
Clinical features sarcoid
presents 20-60yo wheezing Uncommon: crackles and clubbing
45
Dx sarcoid
Clinical features Presence of noncaseating granulomas via biopsy BAL
46
When do you NOT need biopsy for sarcoid dx?
- Asymptomatic bilateral hilar LAD - Lofgrens syndrome (fever, e nodosum, arthralgias, ,BHL) - Lupus pernio - Heerfordt's (parotid swelling, uveitis, bells palsy)
47
BAL findings for sarcoid
Lymphocytic CD4/CD8 >4
48
When to treat pulm sarcoid?
Stage II or III with mod to severe PFT impairment or progression Also treat if extrapulm including hyperCa
49
Tx for sarcoid
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
Cause and tx of Granulomatous Lymphocytic ILD
CVID, immunodef Necrotizing granulomas, folicular bronchiolitis tx, IVIG, steroids, immunosupress/RTX
51
Cytokine specific to eos
IL-5
52
HMB-45+ associated with what dz?
LAM
53
Dx LAM
CT with diffuse cysts AND LAM on biopsy, renal AML, chylothorax, TSC OR Increased serum VEGF-D
54
Tx LAM
Sirolimus transplant
55
HRCT Langerhans
Irregular cysts Basilar sparing Nodules +/- cavitation reticular opacities
56
Mutation in Birt-Hogg-Dube syndrome
BHD (FLCN) gene (chromosome 17p11.2); tumor suppressor protein, folliculin
57
Clinical presentation Birt-Hogg-Dube
cystic lung dz PTX Skin lesions (fibrofolliculomas) Renal tumors
58
HRCT for Birt-Hogg-Dube
Cysts of varying sizes and shapes, more lower and medial lobes
59
HRCT follicular bronchiolitis
Small nodules, patchy ggo, sometimes cysts tx: underlying dz tx, bronchodilators, steroids, erythromycin
60
Heritable disorders that cause cysts
NF1 Marfans (upper lung bullae)
61
Lab findings for autoimmune PAP
Increased serum LDH Surfactant A and D KL-6 Anti-GM-CSF Ab in serum and BAL
62
PFT autoimmune PAP
Restrictive and reduced DLCO
63
autoimmune PAP finding on BAL
Milky effluent BAL
64
Tx PAP
full lung lavage GMS-CSF SQ or neb RTX Lung txp
65
SLC34A2 gene mutation with calcific micronodular infiltrate on CT
Pulmonary alveolar microlithiasis
66
Cause of Erdheim-chester disease
histiocytic disorder, multi organ Malig of myeloid progenitor cells Somatic BRAF mutation
67
Presentation of Erdheim-chester disease
bone pain, cardiac, CNS, hairy kidney, pleuro-pulm
68
Dx Erdheim-chester disease
Histo: foamy histiocytes (CD1a-), MNG histiocytes (Touton cells), fibrosis
69
management Erdheim-chester disease
BRAF kinase inhibitor (vemurafenib) MEK inhibitor (cobimetinib) Cladribine radiation
70
Chyloptysis is a sign of...
Diffuse pulmonary lymphangiomatosis
71
Diffuse pulmonary lymphangiomatosis HRCT
diffuse interlobular septal thickening, patchy ggo, medist infil. pleural thick.
72
Timing of radiation pneumonitis
4-12 weeks after irradiation
73
Timing of radiation fibrosis
6-12 months after irradiation imaging confined to rad field (straight line effect)
74
Timing of radiation induced organizing pneumonia (BOOP)
1-12 mo after therapy (usually for breast cancer
75
Imaging findings radiation induced organizing pneumonia (BOOP)
patchy alveolar infiltrates OUTSIDE radiation field
76
Etiologies of constrictive bronchiolitis/obliterative/BO
Allograft recipient post-infectious CTD -- RA Inhalation injury Drugs/toxins DIPNECH Cryptogenic
77
HRCT constrictive bronchiolitis
moasicism with patchy air trapping, scattered bronchiectasis CXR with hyperinflation
78
Characteristics of DIPNECH
Middle aged women with respiratory sx and obstruction on PFT Mosaic pattern and small nodules CT Can lead to carcinoid
79
Differential for cavitary lung lesions
Neoplasia - Mets (squamous cell common) - multifocal bronchogenic adenocarcinoma - MALT Infection Systemic disease
80
Infectious causes of cavitary lung lesions
Septic emboli lung abscess Mycobacterium TB NTMB fungal infections
81
Systemic disease and other causes of cavitary lung lesions (not infectious or malignant)
GPA RA nodules Sarcoidosis Langerhans cell histiocytosis (early) pulm juvenile papillomatosis (rare) COP/BOOP (rare)
82
CT features of OP
extensive airspace disease patchy lower lung zone–predominant consolidation ground-glass opacities with a subpleural and/or peribronchovascular distribution
83
Treatment for SSc ILD
MMF
84

ABIM Pulmonary Boards - Jackie (11 decks)

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