Diffuse Parenchymal Lung Disease

Question 1

Normal breakdown of BAL cell count

Answer 1

Macrophages >85%, lymphocytes 10-15%, neutrophils <3%, eosinophils <1%, epithelial cells <5%

Question 2

ILDs that require bronchoscopic diagnosis

Answer 2

Sarcoidosis, HP, Pneumoconioses, COP, DAD, RBILD/DIP, LIP, Eosinophilic pneumonias, Rare ILDs (PLCH, PAP, LAM), Infections and cancers

Question 3

8 types of interstitial pneumonias

Answer 3

UIP (IPF), NSIP, RBILD, DIP, COP, AIP, LIP, IPPFE (idopathic pleuroparenchymal fibroelastosis)

Question 4

Typical UIP pattern on HRCT

Answer 4

Subpleural/basal predominance, reticular abnormality, honeycombing +/- traction bronchiectasis, Absence of features inconsistent with UIP (extensive consolidation, GGOs, mosaic attenuation, nodules, cysts)

Question 5

Definition of acute exacerbation of IPF

Answer 5

Acute, clinically significant respiratory deterioration with new, widespread alveolar abnormalities not explained by another disease (i.e. pneumonia or CHF)

Question 6

2 antifibrotic drugs for IPF

Answer 6

Nintendanib, perfinidone

Question 7

Study that showed improvement in IPF with perfenidone

Answer 7

Ascend study (NEJM 2014)

Question 8

Study that showed improvement in IPF with nintendanib

Answer 8

INPULSIS 1 & 2 (NEJM 2014)

Question 9

Criteria to refer for lung transplant in ILD (4)

Answer 9

UIP or NSIP
FVC < 80% or DLCO < 40%
limiting DOE
oxygen use

Question 10

Criteria to place ILD patient on transplant list (7)

Answer 10

FVC decline > 10% in 6 months
DLCO decline >15% in 6 months
Desat to < 88% or < 250 m on 6MWT
pulmonary HTN
hospitalization for respiratory decline
pneumothorax
Acute exacerbation

Question 11

Treatment of ILD when idiopathic by type

Answer 11

IPF = antifibrotic therapy, NSIP = immunosuppressive therapy, COP = steroids and immunosuppressants, AIP = steroids and supportive, IPPFE = supportive

Question 12

Steroid treatment duration in COP

Answer 12

6-12 months, high recurrence rate

Question 13

ILDs associated with smoking

Answer 13

RBILD, DIP, AEP, PLCH, CPFE

Question 14

PFT pattern in CPFE

Answer 14

Relatively preserved spirometry and lung volumes with low DLCO

Question 15

RA usually causes what type of ILD pattern

Answer 15

UIP

Question 16

Sjogren syndrome causes what ILD pattern

Answer 16

NSIP or LIP

Question 17

PM/DM antibody that is associated with increased risk of ILD

Answer 17

Anti-histidyl-tRNA synthetase (anti-Jo-1)

Question 18

Duration of treatment for CTD induced ILD

Answer 18

3-6 months

Question 19

Stepwise treatment of RA associated ILD

Answer 19

Steroids, MMF/AZA, RTX, Abatacept/tocilizumab, antifibrotic

Question 20

Stepwise treatment of Sjogren associated ILD

Answer 20

Steroids, MMF/AZA, RTX

Question 21

Stepwise treatment of SCL associated ILD

Answer 21

MMF, Tocilizumab, CFM/Nintendanib, RTX, Abatacept/HSCT

Question 22

Stepwise treatment of PM/DM associated ILD

Answer 22

Steroids, MMF/AZA/MTX, Tacrolimus/cyclosporine, RTX/IVIg, CFM

Question 23

Stepwise treatment of SLE associated ILD

Answer 23

Steroids, AZA, MMF, RTX, CFM

Question 24

Criteria for interstitial pneumonia with autoimmune features (IPAF)

Answer 24

Presence of interstitial pneumonia, exclusion of alternative etiologies, does not meet criteria of a defined connective tissue disease, and at least 1 feature from 2/3 categories (clinical, serologic, and morphologic)

Question 25

Disease characterized by noncaseating epithelioid granulomas

Answer 25

Sarcoidosis

Question 26

Race and gender predominant for sarcoid

Answer 26

African-American and scandinavian, women

Question 27

Skin manifestation of sarcoidosis

Answer 27

erythema nodosum

Question 28

Eye manifestation of sarcoidosis

Answer 28

uveitis

Question 29

Mechanism of hypercalcemia in sarcoidosis

Answer 29

macrophages metabolize (25) vitamin D to (1,25) vitamin D

Question 30

Cardiac manifestation of sarcoidosis

Answer 30

conduction blocks, arrhythmias, and myocardial dysfunction

Question 31

Pulmonary manifestations of sarcoidosis

Answer 31

Hilar and mediastinal adenopathy, nodules along bronchovascular bundles in the mid and upper lobes, fibrosis with upward retraction of the hila, can have cavitary perihilar masses

Question 32

Symptoms of Lofgren syndrome

Answer 32

fever, erythema nodosum, arthralgias, bilateral hilar adenopathy (no biopsy needed)

Question 33

BAL diagnostic criteria for sarcoidosis

Answer 33

lymphocytic predominance with CD4/CD8 ratio > 4

Question 34

Stage 0 sarcoidosis characteristics

Answer 34

No lung involvement

Question 35

Stage I sarcoidosis characteristics

Answer 35

bilateral hilar adenopathy without parenchymal involvement

Question 36

Stage II sarcoidosis characteristics

Answer 36

bilateral hilar adenopathy with parenchymal involvement

Question 37

Stage III sarcoidosis characteristics

Answer 37

parenchymal involvement without hilar adenopathy

Question 38

Stage IV sarcoidosis characteristics

Answer 38

progressive fibrosis +/- cavity/cysts

Question 39

Indications for systemic treatment of sarcoidosis

Answer 39

Stage II or III with PFT impairment or progression, cardiac/neurologic/ocular/hypercalcemia involvement

Question 40

Treatment of sarcoidosis w/o cardiac involvement

Answer 40

Prednisone 20-40 mg/d x 4-6 weeks, then taper of 6-12 months

Question 41

Treatment of sarcoidosis with cardiac involvement

Answer 41

Prednisone 40-60 mg/d x 4-6 weeks, then taper of 6-12 months

Question 42

Alternative immunosuppressive agents for sarcoidosis

Answer 42

Methotrexate (preferred), AZA, leflunomide, TNF antagonist

Question 43

Disease characterized by nonnecrotizing granulomas, LIP, and follicular bronchiolitis on histopathology

Answer 43

Granulomatous lymphocytic ILD