Restrictive Lung Disease

Question 1

Coal Workers Pneumoconiosis is caused by

Answer 1

• Inhalation of coal mine dust
• Coal is inhaled into alveolus and engulfed by macrophages.
• Macrophages release reactive O2 species, triggering release of cytokines (interleukins, tumor necrosis factor) responsible for inflammation and fibrosis.
• This leads to the development of coal macules.
• These enlarge to coal nodules

Question 2

Pneumoconiosis Symptoms

Answer 2

Cough, sputum production, SHOB/DOE

Question 3

What is found on CXR for pneumoconiosis?

Answer 3

Small nodules that predominate the upper lungs

Question 4

Pneumoconiosis treatment

Answer 4

Supportive- O2 supplementation and rehab

Question 5

Silicosis is caused by

Answer 5

prolonged inhalation and retention of silica particles

Question 6

What is the hallmark lesion of silicosis?

Answer 6

• Silicotic nodule
• Small, rounded opacities in upper and mid lungs, 3-5 mm
• 1st involve hilar lymph nodes and may calcify or erode the airway.
• The lung parenchyma is subsequently involved, usually upper lobes.

Question 7

Silicosis symptoms

Answer 7

Tachypnea, fine crackles, SHOB

Question 8

Diagnosis of silicosis

Answer 8

need open lung biopsy for definitive diagnosis

Question 9

Silicosis treatment

Answer 9

avoid exposure, stop smoking, lavage if needed

Question 10

What is increased in silicosis patients?

Answer 10

Increase in TB

Question 11

Asbestosis characteristics

Answer 11

• Interstitial fibrosis and pleural thickening, similar to Silicosis
• Can be seen 15-20 years after lengthy exposure
• Exposure via renovation/destruction of old buildings, ship building

Question 12

Asbestosis symptoms

Answer 12

DOE, Non-productive cough

Question 13

What is found on asbestosis CXR?

Answer 13

Fibrosis, pleural plaques.

*Make sure to get CT to evaluate plaque. The fewer the opacities, the better the prognosis.

Question 14

Asbestosis treatment

Answer 14

• Oxygen if needed and pulmonary rehab
• Must stop smoking.

Risk of lung cancer in nonsmoking asbestosis worker, 5.2 –> risk of lung cancer in smoking asbestosis worker, risk increases 53 fold

Question 15

Risks in asbestosis patients

Answer 15

increased risk of developing Mesothelioma of the pleura, bronchogenic carcinoma, and TB

Question 16

Sarcoidosis definition

Answer 16

• Multisystem granulomatous disorder of unknown etiology

- Can effect: lungs (90%), lymph nodes, eyes, skin, liver, spleen, heart, joints, and nervous system

Question 17

Sarcoidosis is most commonly seen in…

Answer 17

young African American females, 20-40 y/o, and Northern Europeans

Question 18

A sarcoidosis pathology report will read…

Answer 18

“non caseating granulomatous inflammation”

Question 19

Sarcoidosis symptoms

Answer 19

Cough, SHOB, fever, wt loss, sweats, or nothing

Question 20

What will be seen on CXR for sarcoidosis?

Answer 20

• Bilateral hilar adenopathy, interstitial infiltrates or both
• Get a chest CT for closer evaluation

Question 21

Sarcoidosis diagnosis

Answer 21

Bases on pathology from a TBB (transbronchial biopsy) or FNA (fine needle aspiration)

Question 22

Sarcoidosis treatment

Answer 22

• is based on symptoms
• 90% respond to corticosteroids
• 50-60% have spontaneous remissions

Question 23

What baseline labs should you draw for sarcoidosis?

Answer 23

Complete PFTs, BNP, ECHO, 24 hour urine Ca, Bilateral hand Xrays, ACE level, and refer to Ophthalmology
-Follow pt with serial PFTs and CXR or HRCT

Question 24

What joint is affected first in sarcoidosis?

Answer 24

The wrist joint

Question 25

Nonspecific Interstitial Pneumonitis (NSIP) characteristics

Answer 25

• Younger patients, 40-50 y/o

- Onset usually gradual, 6 months to 3 years before dx

Question 26

NSIP symptoms

Answer 26

cough, wt loss, breathlessness

Question 27

NSIP physical exam

Answer 27

diffuse fine basilar crackles

Question 28

What will be seen on CT for NSIP?

Answer 28

• diffuse, symmetrical GG changes w/wo traction bronchiectasis
• confluent and homogenous pattern.
• Honeycombing is rarely seen

Question 29

NSIP treatment

Answer 29

corticosteroids

Question 30

Cryptogenic Organizing Pneumonia (COP) characteristics

Answer 30

• Organizing pneumonias
• Most common in nonsmokers, roughly 50-60 y/o.
• Males = Females
• Short duration, <3 month history of SHOB, dry cough, may have malaise, wt loss, myalgias
• Chest infection that it slow to resolve after several rounds of antibiotics

Question 31

COP physical exam

Answer 31

may be normal or hear crackles

Question 32

What will be seen on imaging studies in COP?

Answer 32

• CXR: patchy consolidations or even a solitary mass

- HRCT: areas of consolidation with air bronchogram, GGO (these areas can migrate spontaneously)

Question 33

Definitive diagnosis of COP

Answer 33

TBB (transbronchial biopsy) or open lung biopsy

Question 34

COP treatment

Answer 34

long steroid taper over months, may need 6-12 months of therapy.
*Follow with CT

Question 35

Idiopathic Pulmonary Fibrosis

Answer 35

• Diffuse lung disease of unknown etiology
• Involves the interstitium of the lung the fibrous framework of the lung
• Onset of 9-24 months
• MC in males
• Mean age is 65

Question 36

Idiopathic Pulmonary Fibrosis exam findings

Answer 36

fine basal and late inspiratory crackles

Question 37

What would you find on imaging for idiopathic pulmonary fibrosis?

Answer 37

CXR: peripheral and basal reticular shadowing

HRCT: bilateral, peripheral, and subpleural reticulation with honeycombing and traction bronchiectasis

Question 38

What is the prognosis for idiopathic pulmonary fibrosis?

Answer 38

• Very poor prognosis
• Stable vs. decline over years vs. decline rapidly
• Median survival is 2.5 to 3.5 years at time of diagnosis
• Prior to 11/2014 there were no treatment options other than supplemental oxygen.
• -2 new medication to help slow down progression –> Ofev and Esbriet