ILD

Question 1

Normal number of Macrophages in BAL

Answer 1

> 85%

Question 2

Normal number of Lymphocytes in BAL

Answer 2

10-15%

Question 3

Normal number of Eosinophils in BAL

Answer 3

<1%

Question 4

IPF number of Eosinophils in BAL

Answer 4

<10%

Question 5

IPF number of Lymphocytes in BAL

Answer 5

<30%

Question 6

PL-12 positive autoantibody

Answer 6

Commonly seen in patient with interstitial lung disease without evidence of myositis

Question 7

Positive anti-Jo-1 antibody

Answer 7

Patient have concurrent ILD 90% of the time

Question 8

Myositis-specific autoantibodies

Answer 8

Antisynthetase antibodies (30% of patients with PM and DM)
Anti-Jo-1 and anti-PL12 antibodies may occur in patient with ILD but without overt myositis

Question 9

Anti-Ku antibodies

Answer 9

Associated with overlap syndromes with myositis and scleroderma

Question 10

Anti-MDA-5 autoantibodies

Answer 10

Associated with dermatomyositis without myositis but with progressive ILD

Question 11

Anti-SRP antibodies

Answer 11

Associated with severe necrotizing myopathy

Question 12

Lymphocytic interstitial pneumonia associated with

Answer 12

HIV
Sjogrens
Cystic and interstitial lung disease

Question 13

SEATS upper lobe interstitial fibrosis

Answer 13

Silicosis
Eosinophilic granuloma
Ankylosing spondylitis
TB
Sarcoidosis

Question 14

Monday fever

Answer 14

Byssinosis

Question 15

Byssinosis

Answer 15

Pneumoconniosis from cloth (hemp, flax, cotton)

Question 16

Berryliosis

Answer 16

Like sarcoidosis, chronic noncaseating granulomatous disease

Question 17

Berrylium testing

Answer 17

Beryllium lymphocyte proliferation test (serum)

Question 18

CD4:CD8 normal

Answer 18

2

Question 19

CD4:CD8 low

Answer 19

Hypersensitivity pneumonitis

Question 20

CD4:CD8 high

Answer 20

Sarcoidosis

Question 21

Pulmonary alveolar microlithiasis (PAM) characteristic findings

Answer 21

Widespread intraalveolar accumulation of minute calcium phosphate deposits

Question 22

Pulmonary alveolar microlithiasis (PAM) etiology

Answer 22

Mutation in SLC34A2 gene = dysfunctional type 2 alveolar cells

Question 23

Telomere genes leading to IPF or familial pulmonary fibrosis

Answer 23

TERT, TERC

Question 24

MUC5b

Answer 24

Increased risk for pulmonary fibrosis compared to telomere pathway but improved survival

Question 25

Senescence

Answer 25

Programmed cell death (associated with telomeres)

Question 26

Cause of hypercalcemia in sarcoidosis

Answer 26

1-alpha-hydroxylase activity in the granulomas that activate 25-hydeoxyvitamin d3 increasing guy absorption of calcium

Question 27

Vascular endothelial growth factor D (VEGF-D)

Answer 27

Elevated in LAM

Question 28

LAM presentation

Answer 28

Dyspnea
Pneumothorax
Could have chylous pleural effusion

Question 29

LAM may occur with

Answer 29

Tuberous sclerosis complex
Renal angiolypoma
Chylous effusion

Question 30

LAM treatment

Answer 30

Sirolimus

Question 31

Diffuse cystic lung disease

Answer 31

LAM
Birthings-Hogg-Dube
Lymphocytic interstitial pneumonia
Langerhans

Question 32

Langerhans typical findings

Answer 32

Lung cysts and nodules

Associated with smoking

Question 33

Birt-Hogg-Dube (BHD) typical findings

Answer 33

Skin fibrofolliculomas

Risk for renal adenocarcinoma

Question 34

Birt-Hogg-Dube (BHD) epidemiology

Answer 34

Autosomal dominant

Question 35

Birt-Hogg-Dube (BHD) diagnosis

Answer 35

Folliculin gene testing

Question 36

Nintedanib MOA

Answer 36

Tyrosine kinase inhibitor

Question 37

Langerhan’s cells staining

Answer 37

S-100 protein

CD1a positive

Question 38

Therapy for Langerhan’s

Answer 38

Stop smoking

Cladribine

Question 39

LAM staining

Answer 39

HMB-45 antibody